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Kitazono I, Murakami M, Takeda A, Noguchi H, Tasaki T, Kirishima M, Higashi M, Ueda K, Tanimoto A. Pulmonary pleomorphic carcinoma: A rare case showing arterial carcinomatosis mimicking pulmonary artery intimal sarcoma. Pathol Int 2025; 75:114-120. [PMID: 39783178 DOI: 10.1111/pin.13508] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2024] [Revised: 12/11/2024] [Accepted: 12/24/2024] [Indexed: 01/12/2025]
Abstract
A male in his seventies presented with lung cancer in the right lower lobe. The surgically resected specimen revealed a pleomorphic carcinoma featuring an adenocarcinoma component with lepidic, acinar, and papillary patterns, alongside a spindle cell component spreading along the pulmonary artery wall, resembling intimal sarcoma. The spindle tumor cells were positive for keratins, TTF-1, napsin A, and vimentin, but negative for p40, CK14, desmin, alpha-smooth muscle actin, CDK4, and MDM2. This profile indicates that the spindle cells originated from the adenocarcinoma cells via epithelial-mesenchymal transition. The vascular spread of pleomorphic carcinoma, mimicking pulmonary artery intimal sarcoma, represents an extremely rare mode of tumor invasion, which could be termed "arterial carcinomatosis". Immunohistochemical analysis is crucial for distinguishing pleomorphic carcinoma from coexisting intimal sarcoma, as the latter's prognosis is markedly poor.
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Affiliation(s)
- Ikumi Kitazono
- Department of Surgical Pathology, Kagoshima University Hospital, Kagoshima, Japan
| | - Miki Murakami
- Department of Surgical Pathology, Kagoshima University Hospital, Kagoshima, Japan
| | - Aya Takeda
- Department of General Thoracic Surgery, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
| | - Hirotsugu Noguchi
- Department of Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
| | - Takashi Tasaki
- Department of Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
| | - Mari Kirishima
- Department of Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
| | - Michiyo Higashi
- Department of Surgical Pathology, Kagoshima University Hospital, Kagoshima, Japan
| | - Kazuhiro Ueda
- Department of General Thoracic Surgery, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
| | - Akihide Tanimoto
- Department of Surgical Pathology, Kagoshima University Hospital, Kagoshima, Japan
- Department of Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
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Park C, Kim R, Bae JM, Lee T, Song S, Kwak Y, Lee KB, Youk J, Keam B, Kim TM, Kim DW, Kim JI, Choi J, Kim M. Genomic profiling of intimal sarcoma reveals molecular subtypes with distinct tumor microenvironments and therapeutic implications. ESMO Open 2025; 10:104097. [PMID: 39778225 PMCID: PMC11758979 DOI: 10.1016/j.esmoop.2024.104097] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/04/2024] [Revised: 11/14/2024] [Accepted: 12/02/2024] [Indexed: 01/11/2025] Open
Abstract
BACKGROUND Intimal sarcoma is a rare and aggressive soft-tissue sarcoma with limited treatment options. We explored genomic profiles of intimal sarcoma to uncover therapeutic implications. MATERIALS AND METHODS We analyzed tumor tissues from patients with intimal sarcoma who visited the Seoul National University Hospital (SNUH) using whole-exome, whole-transcriptome, and clinical next-generation sequencing (NGS), integrated with intimal sarcoma NGS data from two public cohorts. We examined expression characteristics and tumor-infiltrating lymphocytes (TILs) according to molecular subtypes. RESULTS Our study included 42 samples in total. Thirty-three patients showing copy number variation (CNV) enrichment with frequent CDK4/MDM2 amplifications were classified as the CNV-high (CNV-H) subtype. Five patients showing predominant MLH1 mutations or homozygous deletions were classified as the microsatellite instability-high-like (MSI-H-like) subtype. Hallmark pathways up-regulated in the CNV-H subtype included Wnt β-catenin and Hedgehog signaling. In the MSI-H-like subtype, interferon-γ response, tumor necrosis factor-α signaling via nuclear factor-κB, interferon-α response, inflammatory response, and interleukin-6-Jak-Stat3 signaling were up-regulated. CNV-H subtype samples predominantly showed an immune-desert phenotype, whereas MSI-H-like subtype samples predominantly showed an immune-inflamed phenotype. Two MSI-H-like subtype patients received pembrolizumab and experienced tumor shrinkage. CONCLUSIONS We identified two intimal sarcoma molecular subtypes. Compared with CNV-H, MSI-H-like is enriched in pathways associated with tumor immune responses and TILs. Further efforts and clinical trials to better define these molecular subtypes are warranted to open new avenues for personalized treatment approaches and improve patient outcomes.
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Affiliation(s)
- C Park
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
| | - R Kim
- Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Korea; Genomic Medicine Institute, Medical Research Center, Seoul National University, Seoul, Korea
| | - J M Bae
- Department of Pathology, Seoul National University Hospital, Seoul, Korea
| | | | | | - Y Kwak
- Department of Pathology, Seoul National University Hospital, Seoul, Korea
| | - K B Lee
- Department of Pathology, Seoul National University Hospital, Seoul, Korea
| | - J Youk
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
| | - B Keam
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
| | - T M Kim
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
| | - D-W Kim
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
| | - J-I Kim
- Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea; Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Korea; Genomic Medicine Institute, Medical Research Center, Seoul National University, Seoul, Korea
| | - J Choi
- Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Korea; Genomic Medicine Institute, Medical Research Center, Seoul National University, Seoul, Korea.
| | - M Kim
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
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Atahan C, Güral Z, Yücel S, Ağaoğlu F. Pulmonary artery intimal sarcoma: Case report of a patient managed with multimodality treatment and a comprehensive literature review. Strahlenther Onkol 2024; 200:725-729. [PMID: 38866999 PMCID: PMC11272804 DOI: 10.1007/s00066-024-02250-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2024] [Accepted: 05/20/2024] [Indexed: 06/14/2024]
Abstract
Pulmonary artery intimal sarcoma (PAIS) is a rare and aggressive malignancy originating from the intimal layer of the pulmonary artery with poor prognosis due to its aggressive nature. The management of PAIS poses both diagnostic and therapeutic challenges. It presents with nonspecific symptoms and is often misdiagnosed as pulmonary embolism. While surgical resection is the primary treatment modality, the role of adjuvant chemotherapy and radiotherapy remains uncertain. However, given the high recurrence rate, adjuvant chemotherapy and/or radiotherapy have been utilized in a limited number of case reports. We present the case of a 46-year-old woman who was diagnosed with PAIS and underwent surgical resection followed by adjuvant chemotherapy (ChT) and radiotherapy (RT), demonstrating good tolerance to this multimodal treatment approach.
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Affiliation(s)
- C Atahan
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey
| | - Z Güral
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey
| | - S Yücel
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey
| | - F Ağaoğlu
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey.
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Zhang Z, Zhen Y, Liu J, Liu X, Yang L, Xu M, Wen J, Liu P. Outcomes of pulmonary endarterectomy for patients with pulmonary artery sarcoma. Front Cardiovasc Med 2024; 11:1302372. [PMID: 39015681 PMCID: PMC11250646 DOI: 10.3389/fcvm.2024.1302372] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2024] [Accepted: 05/22/2024] [Indexed: 07/18/2024] Open
Abstract
Objective Pulmonary artery sarcoma (PAS) is an exceedingly rare and insufficiently investigated disease, leading to uncertain in its optimal management. This study aims to present our institutional experience and the outcomes of pulmonary endarterectomy for PAS. Methods We gathered clinical characteristics, intraoperative data, postoperative outcomes, and prognosis information from PAS patients who underwent surgical treatment at our institution between December 2016 and September 2023. Results A total of 20 patients with PAS underwent pulmonary endarterectomy. The median age of the patients was 52 (IQR 45, 57) years, with 12 patients (60%) being female. Intimal sarcoma was confirmed in 19 patients, while the remaining one was diagnosed with large cell neuroendocrine carcinoma. The perioperative mortality rate was three cases (15%). Follow-up was conducted for a median duration of 14 months (range: 1-61). During the follow-up period, 11 patients experienced recurrence or metastasis, and 5 patients succumbed to the disease. The estimated cumulative survival rates at 1 and 2 years for all 20 patients were 66.4% and 55.3%, respectively. Conclusion Pulmonary endarterectomy emerges as a palliative but effective approach for managing PAS, particularly when complemented with postoperative therapies such as chemotherapy and targeted therapy, which collectively contribute to achieving favorable long-term survival outcomes.
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Affiliation(s)
- Zhaohua Zhang
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Yanan Zhen
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Jingwen Liu
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Xiaopeng Liu
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Liang Yang
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Mingyuan Xu
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Jianyan Wen
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Peng Liu
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
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Smith HJ, Al-Zubaidi F, Sitaranjan D, Chiu S, Jenkins D. Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review. J Cardiothorac Surg 2024; 19:288. [PMID: 38745263 PMCID: PMC11092144 DOI: 10.1186/s13019-024-02700-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2023] [Accepted: 03/27/2024] [Indexed: 05/16/2024] Open
Abstract
Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.
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Affiliation(s)
- Harry James Smith
- Royal Papworth Hospital, Papworth Road, Trumpington, Cambridge, CB2 0AY, UK.
| | - Fadi Al-Zubaidi
- Royal Papworth Hospital, Papworth Road, Trumpington, Cambridge, CB2 0AY, UK
| | - Daniel Sitaranjan
- Royal Papworth Hospital, Papworth Road, Trumpington, Cambridge, CB2 0AY, UK
| | - Steven Chiu
- Royal Papworth Hospital, Papworth Road, Trumpington, Cambridge, CB2 0AY, UK
| | - David Jenkins
- Royal Papworth Hospital, Papworth Road, Trumpington, Cambridge, CB2 0AY, UK
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Dörr A, Flörcken A, Bullinger L, Capper D, Deimling AV, Kaul D, Märdian S, Starck C, Horst D, Dragomir MP, Schäfer FM, Jarosch A. Thrombus or tumor? A case report of a rare sarcoma entity: intimal sarcoma of the pulmonary arteries. Mol Biol Rep 2024; 51:568. [PMID: 38656400 PMCID: PMC11043182 DOI: 10.1007/s11033-024-09467-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2024] [Accepted: 03/22/2024] [Indexed: 04/26/2024]
Abstract
BACKGROUND Tumor embolism is a very rare primary manifestation of cancers and the diagnosis is challenging, especially if located in the pulmonary arteries, where it can mimic nonmalignant pulmonary embolism. Intimal sarcoma is one of the least commonly reported primary tumors of vessels with only a few cases reported worldwide. A typical location of this malignancy is the pulmonary artery. Herein, we present a case report of an intimal sarcoma with primary manifestation in the pulmonary arteries. A 53-year-old male initially presented with dyspnea. On imaging, a pulmonary artery embolism was detected and was followed by thrombectomy of the right ventricular outflow tract, main pulmonary artery trunk, and right pulmonary artery after ineffective lysis therapy. Complementary imaging of the chest and abdomen including a PET-CT scan demonstrated no evidence of a primary tumor. Subsequent pathology assessment suggested an intimal sarcoma further confirmed by DNA methylation based molecular analysis. We initiated adjuvant chemotherapy with doxorubicin. Four months after the completion of adjuvant therapy a follow-up scan revealed a local recurrence without distant metastases. DISCUSSION Primary pulmonary artery intimal sarcoma (PAS) is an exceedingly rare entity and pathological diagnosis remains challenging. Therefore, the detection of entity-specific molecular alterations is a supporting argument in the diagnostic spectrum. Complete surgical resection is the prognostically most important treatment for intimal cardiac sarcomas. Despite adjuvant chemotherapy, the prognosis of cardiac sarcomas remains very poor. This case of a PAS highlights the difficulty in establishing a diagnosis and the aggressive natural course of the disease. CONCLUSION In case of atypical presentation of a pulmonary embolism, a tumor originating from the great vessels should be considered. Molecular pathology techniques support in establishing a reliable diagnosis.
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Affiliation(s)
- A Dörr
- Department of Hematology, Oncology, and Tumor Immunology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin and Berlin Institute of Health, Berlin, Germany.
| | - A Flörcken
- Department of Hematology, Oncology, and Tumor Immunology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin and Berlin Institute of Health, Berlin, Germany
- German Cancer Consortium (DKTK), Partner site Berlin, German Cancer Research Center (DKFZ), Heidelberg, Germany
| | - L Bullinger
- Department of Hematology, Oncology, and Tumor Immunology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin and Berlin Institute of Health, Berlin, Germany
- German Cancer Consortium (DKTK), Partner site Berlin, German Cancer Research Center (DKFZ), Heidelberg, Germany
| | - D Capper
- German Cancer Consortium (DKTK), Partner site Berlin, German Cancer Research Center (DKFZ), Heidelberg, Germany
- Department of Neuropathology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin and Berlin Institute of Health, Berlin, Germany
| | - A Von Deimling
- Department of Neuropathology, and CCU Neuropathology, University Hospital Heidelberg, DKFZ, Heidelberg, Germany
| | - D Kaul
- German Cancer Consortium (DKTK), Partner site Berlin, German Cancer Research Center (DKFZ), Heidelberg, Germany
- Department of Radiation Oncology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt and Universität zu Berlin and Berlin Institute of Health, Berlin, Germany
| | - S Märdian
- Center for Musculoskeletal Surgery, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin and Berlin Institute of Health, Berlin, Germany
| | - C Starck
- Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany
| | - D Horst
- German Cancer Consortium (DKTK), Partner site Berlin, German Cancer Research Center (DKFZ), Heidelberg, Germany
- Department of Pathology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany
| | - M P Dragomir
- German Cancer Consortium (DKTK), Partner site Berlin, German Cancer Research Center (DKFZ), Heidelberg, Germany
- Department of Pathology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany
- Berlin Institute of Health (BIH), Berlin, Germany
| | - F M Schäfer
- Institute for Radiology, Charité - Universitätsmedizin Berlin, Universität zu Berlin, Corporate member of Freie Universität Berlin and Humboldt, Berlin, Germany
| | - A Jarosch
- Department of Pathology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany
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Wang Y, Rong C, Liu J, Liu X, Zhang W. Pulmonary arterial sarcoma: A case report. Medicine (Baltimore) 2024; 103:e37194. [PMID: 38394547 PMCID: PMC11309704 DOI: 10.1097/md.0000000000037194] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2023] [Accepted: 01/18/2024] [Indexed: 02/25/2024] Open
Abstract
RATIONALE Pulmonary artery sarcoma (PAS) is a rare malignant tumor primarily originating from the pulmonary artery's intima or subintima. Approximately one-third of cases are classified as undifferentiated type. Its clinical manifestations lack specificity, dyspnea is the main symptom but can also present with chest pain, cough, hemoptysis, and other discomforts, making it prone to misdiagnosis as pulmonary embolism (PE). PATIENT CONCERNS A 50-year-old woman was admitted to the hospital with "dyspnea for more than 3 months, aggravated for 2 days," and computed tomography pulmonary angiography suggesting "bilateral multiple pulmonary embolisms." DIAGNOSES The patient was initially misdiagnosed as PE, and was later definitively diagnosed as undifferentiated pleomorphic sarcoma of the pulmonary artery by pathologic biopsy. INTERVENTIONS AND OUTCOMES The patient was initially treated with anticoagulant therapy, but her dyspnea was not relieved. After that, she underwent positron emission computed tomography (PET-CT) and other investigations, which suggested the possibility of PAS, and then she underwent pulmonary endarterectomy to remove the lesion, which relieved her symptoms and was advised to seek further medical attention from the Department of Oncology and Department of Radiotherapy. LESSONS PAS can be easily misdiagnosed as PE. If a diagnosis of PE is made, but anticoagulation or even thrombolytic therapy proves ineffective, and there is no presence of PE causative factors such as deep vein thrombosis in the lower extremities, or D-dimer levels are not high, one should be cautious and consider the possibility of PAS.
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Affiliation(s)
- Yin Wang
- Department of Cardiology, First Hospital of Jilin University, Changchun, China
| | - Chunyan Rong
- Department of Cardiology, First Hospital of Jilin University, Changchun, China
| | - Jingwei Liu
- Department of Cardiology, First Hospital of Jilin University, Changchun, China
| | - Xuhan Liu
- Department of Cardiology, First Hospital of Jilin University, Changchun, China
| | - Weihua Zhang
- Department of Cardiology, First Hospital of Jilin University, Changchun, China
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Ampatzidou F, Drosos O, Madesis A, Drossos G. Pulmonary artery sarcoma presenting as chronic thromboembolic pulmonary hypertension (CTEPH). KARDIOCHIRURGIA I TORAKOCHIRURGIA POLSKA = POLISH JOURNAL OF CARDIO-THORACIC SURGERY 2023; 20:129-131. [PMID: 37564958 PMCID: PMC10410642 DOI: 10.5114/kitp.2023.129538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/27/2022] [Accepted: 03/17/2023] [Indexed: 08/12/2023]
Affiliation(s)
- Fotini Ampatzidou
- Department of Cardiac Surgery, ICU, General Hospital “G. Papanikolaou”, Thessaloniki, Greece
| | | | - Athanasios Madesis
- Department of Cardiac Surgery, ICU, General Hospital “G. Papanikolaou”, Thessaloniki, Greece
| | - George Drossos
- Department of Cardiac Surgery, ICU, General Hospital “G. Papanikolaou”, Thessaloniki, Greece
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Mahdi A, Mahdi M, Ters P. A Journey From Cardiology to Oncology Reveals a Rare Case of Primary Intimal Sarcoma in a Patient With Dyspnea: A Case Report. Cureus 2023; 15:e38439. [PMID: 37273385 PMCID: PMC10234616 DOI: 10.7759/cureus.38439] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/02/2023] [Indexed: 06/06/2023] Open
Abstract
Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that arises from the intimal layer of the pulmonary artery. It typically presents with nonspecific symptoms such as dyspnea, chest pain, and hemoptysis, making early diagnosis challenging. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful in identifying the tumor's location and extent. A definitive diagnosis is established by biopsy, either via surgical resection or percutaneous needle biopsy. However, diagnosis can be difficult due to the rarity of the disease and the need for specialized expertise in interpreting pathology specimens. Treatment of primary intimal sarcoma of the pulmonary artery involves surgical resection, followed by adjuvant chemotherapy and radiation therapy. Despite aggressive treatment, the prognosis remains poor, with a median survival of approximately two years. However, early detection and aggressive multimodal therapy can improve outcomes. We hereby report a rare case of primary intimal sarcoma of the pulmonary artery and discuss its pathophysiology, presentation, diagnostic approach, and treatment options.
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Affiliation(s)
- Ahmad Mahdi
- Internal Medicine, University of Kansas School of Medicine, Wichita, USA
| | - Mahmoud Mahdi
- Internal Medicine, University of Kansas School of Medicine, Wichita, USA
| | - Patrick Ters
- Cardiology, University of Kansas School of Medicine, Wichita, USA
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10
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A 53-Year-Old With Progressive Dyspnea. Chest 2023; 163:e83-e89. [PMID: 36759123 DOI: 10.1016/j.chest.2022.08.2222] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2022] [Revised: 07/27/2022] [Accepted: 08/03/2022] [Indexed: 02/10/2023] Open
Abstract
CASE PRESENTATION A 53-year-old woman with a history of pulmonary embolism treated with rivaroxaban came to the ED after 4 days of acutely worsening dyspnea and chest pressure. On arrival, her temperature was 36.7 °C; heart rate, 71 beats/min; BP, 98/59 mm Hg; respiratory rate, 22 breaths/min; and Spo2 95% on room air. Her WBC count was elevated at 15,770/μL; hemoglobin, 13.3 g/dL; platelets, 280,000/μL; INR (international normalized ratio), elevated at 1.66; and partial thromboplastin time, elevated at 18.8 s. Serum chemistry results were unremarkable, and pro-brain natriuretic peptide was slightly elevated at 530 pg/mL (normal, < 300 pg/mL).
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11
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Zhao M, Nie P, Guo Y, Chen H. Pulmonary artery intimal sarcoma: A rare cause of filling defects in pulmonary arteries. Am J Med Sci 2022; 364:655-660. [PMID: 35588894 DOI: 10.1016/j.amjms.2022.05.009] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2021] [Revised: 10/07/2021] [Accepted: 05/11/2022] [Indexed: 01/25/2023]
Abstract
Pulmonary artery intimal sarcomas are very rare and arise from primitive pluripotent mesenchymal cells. They are often misdiagnosed as pulmonary thromboembolism, leading to futile anticoagulation treatment and delayed diagnosis. We present a case of a patient who showed nonspecific pulmonary symptoms and characteristic imaging manifestation. Progressive symptoms and additional imaging led to the suspicion of a pulmonary artery intimal sarcoma, which was finally confirmed by pathological biopsy. This case serves as a reminder to consider pulmonary artery intimal sarcomas in the differential diagnosis of patients with dyspnea and filling defects on computed tomography pulmonary angiography or contrast-enhanced computed tomography.
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Affiliation(s)
- Mimi Zhao
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003
| | - Pei Nie
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003
| | - Yonghua Guo
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003
| | - Haisong Chen
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003.
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Oliveira MF, Sá I, Gonçalves F, Santos M, Reis A. A Not So Innocent Murmur. Heart Lung Circ 2022; 31:e147-e148. [PMID: 35927192 DOI: 10.1016/j.hlc.2022.04.055] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2021] [Revised: 06/11/2021] [Accepted: 04/04/2022] [Indexed: 01/27/2023]
Affiliation(s)
- Marta Fontes Oliveira
- Cardiology Department, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal.
| | - Isabel Sá
- Cardiology Department, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
| | - Fabienne Gonçalves
- Pulmonary Vascular Disease Unit, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
| | - Mário Santos
- Cardiology Department, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal; Pulmonary Vascular Disease Unit, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
| | - Abílio Reis
- Pulmonary Vascular Disease Unit, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
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13
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Pott J, Gräfin Zu Reventlow P, Simon M, Steurer S, Harbaum L, Henes FO, Klose H. [Intimal pulmonary sarcoma: A case report of a rare differential diagnosis of chronic thromboembolic pulmonary hypertension]. Pneumologie 2022; 76:633-638. [PMID: 36104018 DOI: 10.1055/a-1883-8890] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/14/2022]
Abstract
Pulmonary angiosarcoma is a rare and malignant disease of the blood vessels. Initially, it can be misdiagnosed as chronic thromboembolic hypertension (CTEPH). In CTEPH, there is increased pressure and resistance of the pulmonary arteries following persistent obstruction of pulmonary circulation from (recurrent) thromboembolism despite adequate anticoagulative treatment.A 76-year-old patient was referred to our centre for pulmonary hypertension after a central, left-sided, subacute pulmonary thromboembolism had been observed 7 months earlier. It was treated with apixaban, but the patient described persistent dyspnoea and cough. We observed severely reduced diffusion capacity, ineffective ventilation during cardiopulmonary exercise testing and right heart strain on echocardiograph, signs that are in agreement with suspected CTEPH. Computer tomography of the chest showed a persistent, size-constant obliteration of the left main pulmonary artery, and ventilation perfusion scan confirmed complete interruption of perfusion. We suspected malignancy; PET-CT scan confirmed metabolically active lesions. Histopathological examination of a sample obtained from the lesion by endobronchial ultrasound-guided needle aspiration showed a sarcomatous tumour with amplification of the MDM2-gene. We diagnosed an intimal angiosarcoma of the left pulmonary artery and referred the patient to pneumectomy.Angiosarcoma of the pulmonary arteries is a rare differential diagnosis of persistent thrombotic lesion and suspected CTEPH. In 2015 there were less than 300 cases described.Pulmonary angiosarcoma should be considered if: lesion occupies the entire lumen of pulmonary arteries with dilatation, contrast enhancement and infiltration of the wall in radiological examination, FDG-PET CT reveals metabolically active lesions, no pulmonary thromboembolism was documented in the anamnesis, increase in size is seen despite anticoagulation, patient presents with B symptoms.Diagnosis confirmed by biopsy, resection of tumour and removal of metastases is the therapeutic standard. Median survival remains poor. Further research is needed for improved diagnosis and treatment.
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Affiliation(s)
- Julian Pott
- Abteilung für Pneumologie, II. Medizinische Klinik und Poliklinik, Zentrum für Onkologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
| | - Paulina Gräfin Zu Reventlow
- Zentrum für Radiologie und Endoskopie, Klinik und Poliklinik für Diagnostische und Interventionelle Radiologie und Nuklearmedizin, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
| | - Marcel Simon
- Abteilung für Pneumologie, II. Medizinische Klinik und Poliklinik, Zentrum für Onkologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
| | - Stefan Steurer
- Institut für Pathologie mit den Sektionen Molekularpathologie und Zytopathologie, Zentrum für Diagnostik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
| | - Lars Harbaum
- Abteilung für Pneumologie, II. Medizinische Klinik und Poliklinik, Zentrum für Onkologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
| | - Frank Oliver Henes
- Zentrum für Radiologie und Endoskopie, Klinik und Poliklinik für Diagnostische und Interventionelle Radiologie und Nuklearmedizin, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
| | - Hans Klose
- Abteilung für Pneumologie, II. Medizinische Klinik und Poliklinik, Zentrum für Onkologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany
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14
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Cortopassi IO, Gosangi B, Asch D, Bader AS, Gange CP, Rubinowitz AN. Diseases of the pulmonary arteries: imaging appearances and pearls. Clin Imaging 2022; 91:111-125. [DOI: 10.1016/j.clinimag.2022.08.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2022] [Revised: 08/16/2022] [Accepted: 08/19/2022] [Indexed: 11/03/2022]
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15
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Hieu NL, Tu VN, Hoan L, Hai HB, Luu DT, Cuong NN, Tra My TT, Minh TN, Manh PT. A case report of primary pulmonary artery intimal sarcoma. Radiol Case Rep 2022; 17:1986-1990. [PMID: 35432686 PMCID: PMC9010888 DOI: 10.1016/j.radcr.2022.03.052] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2022] [Revised: 03/13/2022] [Accepted: 03/13/2022] [Indexed: 12/02/2022] Open
Abstract
Primary pulmonary artery sarcoma is a rare tumor that mimics pulmonary embolism. Patients may present with cough, dyspnea, chest pain, and weight loss. The diagnosis is challenging. Herein, we report a case of 29-year-old female patient who had presented with dyspnea, fatigue for 2 weeks. Computed tomography pulmonary angiography scan suggests pulmonary embolism. We decided to perform surgical embolectomy. The histopathological results, however demonstrated primary pulmonary artery intimal sarcoma. The patient died 1-month post-surgery because of respiratory and circulatory failure.
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16
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Keita P, Janko M, Peterman NJ, Toledo S, Pelletier M. Pulmonary Artery Root Replacement for Leiomyosarcoma: A Viable Thoracic Oncovascular Operation. Cureus 2022; 14:e24734. [PMID: 35673312 PMCID: PMC9165929 DOI: 10.7759/cureus.24734] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/03/2022] [Indexed: 11/05/2022] Open
Abstract
Leiomyosarcoma of the pulmonary artery is a rare but potentially fatal disease. Due to its rarity, the treatment algorithm is not well-established. While there may be a role for both chemotherapy and radiotherapy, surgical management is the most definitive method. Unfortunately, when the disease process is advanced, surgery may not be curative. However, it may still be a palliative treatment option. In this case report, we present a patient who suffered from respiratory symptoms that were initially attributed to pulmonary embolism (PE). However, upon the diagnosis of pulmonary artery leiomyosarcoma (PAL), surgery intervention was undertaken and resulted in an improved quality of life for the patient.
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Affiliation(s)
- Papus Keita
- Surgery, Carle Foundation Hospital, Urbana, USA
| | - Matthew Janko
- Cardiac Surgery, University Hospitals Cleveland Medical Center, Cleveland, USA
| | | | | | - Marc Pelletier
- Cardiac Surgery, University Hospitals Cleveland Medical Center, Cleveland, USA
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17
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Tuft C, Maheepala K, Raguparan A, Naeem A, Lodh S, Lindstrom S. Pulmonary artery sarcoma: An important mimic of pulmonary embolism-Case reports and literature review. Respirol Case Rep 2022; 10:e0897. [PMID: 35028154 PMCID: PMC8743422 DOI: 10.1002/rcr2.897] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2021] [Revised: 11/27/2021] [Accepted: 12/06/2021] [Indexed: 11/10/2022] Open
Abstract
Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low d-dimer, troponin T or NT-proBNP, as well as characteristic findings on CT pulmonary angiography such as the 'wall eclipsing sign' and an non-dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2-year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.
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Affiliation(s)
- Colin Tuft
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Krishan Maheepala
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Ajantha Raguparan
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Anas Naeem
- Intensive Care UnitSt George HospitalSydneyNew South WalesAustralia
| | - Suhrid Lodh
- Radiology DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Steven Lindstrom
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
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18
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Chuang TM, Hsiao HH, Tsai KB. Primary intimal sarcoma with chondrosarcoma differentiation of the pulmonary artery. JOURNAL OF CANCER RESEARCH AND PRACTICE 2022. [DOI: 10.4103/jcrp.jcrp_37_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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19
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Rackauskas M, Beaver T. Commentary: Are we always ready for a challenging surprise? JTCVS Tech 2021; 10:320-321. [PMID: 34977747 PMCID: PMC8691815 DOI: 10.1016/j.xjtc.2021.09.046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2021] [Revised: 09/19/2021] [Accepted: 09/24/2021] [Indexed: 11/22/2022] Open
Affiliation(s)
- Mindaugas Rackauskas
- Division of Thoracic Surgery, Department of Surgery, University of Florida College of Medicine, Gainesville, Fla
| | - Thomas Beaver
- Division of Cardiac Surgery, Department of Surgery, University of Florida College of Medicine, Gainesville, Fla
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20
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Giant Pulmonary Artery Thrombotic Material, Due to Chronic Thromboembolic Pulmonary Hypertension, Mimics Pulmonary Artery Sarcoma. MEDICINA-LITHUANIA 2021; 57:medicina57090992. [PMID: 34577914 PMCID: PMC8465601 DOI: 10.3390/medicina57090992] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/13/2021] [Revised: 09/07/2021] [Accepted: 09/16/2021] [Indexed: 11/16/2022]
Abstract
In this article, we present the case of a 38-year-old female who suffered from serious respiratory distress. After an extensive pulmonary artery imaging diagnostic work-up (CTPA, MRA and PET), we were unable to differentiate between chronic thromboembolic pulmonary hypertension (CTEPH) vs. pulmonary artery sarcoma (PAS) due to extensive filling defects and extraluminal findings. Although surgery was postponed for nine months due to the COVID-19 pandemic, CTEPH diagnosis, due to a high-thrombus burden, was finally confirmed after pulmonary endarterectomy (PEA). Conclusively, imaging findings of rare cases of CTEPH might mimic PAS and the surgical removal of the lesion are both needed for a final diagnosis. What is Already Known about This Topic? Pulmonary artery sarcoma (PAS) is a rare but aggressive malignancy, which originates from the intimal layer of the pulmonary artery (PA); Chronic thromboembolic pulmonary hypertension (CTEPH) is based on chronic, organized flow-limiting thrombi inside PA circulation and subsequent pulmonary hypertension. What Does This Study Contribute? Since radiological findings of CTEPH cases might rarely mimic PAS, pulmonary artery endarterectomy and subsequent histopathologic study are needed for a final diagnosis.
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21
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de Perrot M, Gopalan D, Jenkins D, Lang IM, Fadel E, Delcroix M, Benza R, Heresi GA, Kanwar M, Granton JT, McInnis M, Klok FA, Kerr KM, Pepke-Zaba J, Toshner M, Bykova A, Armini AMD, Robbins IM, Madani M, McGiffin D, Wiedenroth CB, Mafeld S, Opitz I, Mercier O, Uber PA, Frantz RP, Auger WR. Evaluation and management of patients with chronic thromboembolic pulmonary hypertension - consensus statement from the ISHLT. J Heart Lung Transplant 2021; 40:1301-1326. [PMID: 34420851 DOI: 10.1016/j.healun.2021.07.020] [Citation(s) in RCA: 44] [Impact Index Per Article: 11.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2021] [Accepted: 07/22/2021] [Indexed: 02/08/2023] Open
Abstract
ISHLT members have recognized the importance of a consensus statement on the evaluation and management of patients with chronic thromboembolic pulmonary hypertension. The creation of this document required multiple steps, including the engagement of the ISHLT councils, approval by the Standards and Guidelines Committee, identification and selection of experts in the field, and the development of 6 working groups. Each working group provided a separate section based on an extensive literature search. These sections were then coalesced into a single document that was circulated to all members of the working groups. Key points were summarized at the end of each section. Due to the limited number of comparative trials in this field, the document was written as a literature review with expert opinion rather than based on level of evidence.
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Affiliation(s)
- Marc de Perrot
- Division of Thoracic Surgery, Toronto General Hospital, Toronto, Ontario, Canada.
| | - Deepa Gopalan
- Department of Radiology, Imperial College Healthcare NHS Trust, London & Cambridge University Hospital, Cambridge, UK
| | - David Jenkins
- National Pulmonary Endarterectomy Service, Department of Cardiothoracic Surgery, Papworth Hospital, Cambridge, UK
| | - Irene M Lang
- Department of Cardiology, Pulmonary Hypertension Unit, Medical University of Vienna, Vienna, Austria
| | - Elie Fadel
- Department of Thoracic and Vascular Surgery and Heart Lung Transplantation, Marie-Lannelongue Hospital, Paris Saclay University, Le Plessis-Robinson, France
| | - Marion Delcroix
- Clinical Department of Respiratory Diseases, Pulmonary Hypertension Centre, UZ Leuven, Leuven, Belgium; Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department of Chronic Diseases and Metabolism (CHROMETA), KU, Leuven, Belgium
| | - Raymond Benza
- Division of Cardiovascular Medicine, The Ohio State University, Columbus, Ohio
| | - Gustavo A Heresi
- Department of Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, Ohio
| | - Manreet Kanwar
- Cardiovascular Institute, Allegheny Health Network, Pittsburgh, Pennsylvania
| | - John T Granton
- Division of Respirology, University Health Network, Toronto, Ontario, Canada
| | - Micheal McInnis
- Joint Department of Medical Imaging, University Health Network, Toronto, Ontario, Canada
| | - Frederikus A Klok
- Department of Medicine, Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands
| | - Kim M Kerr
- University of California San Diego Medical Health, Division of Pulmonary Critical Care and Sleep Medicine, San Diego, California
| | - Joanna Pepke-Zaba
- Pulmonary Vascular Disease Unit, Royal Papworth Hospital NHS foundation Trust, Cambridge, Cambridgeshire, UK
| | - Mark Toshner
- Pulmonary Vascular Disease Unit, Royal Papworth Hospital NHS foundation Trust, Cambridge, Cambridgeshire, UK; Heart Lung Research Institute, University of Cambridge, Cambridge, UK
| | - Anastasia Bykova
- Division of Thoracic Surgery, Toronto General Hospital, Toronto, Ontario, Canada
| | - Andrea M D' Armini
- Unit of Cardiac Surgery, Intrathoracic-Trasplantation and Pulmonary Hypertension, University of Pavia, Foundation I.R.C.C.S. Policlinico San Matteo, Pavia, Italy
| | - Ivan M Robbins
- Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee
| | - Michael Madani
- Department of Cardiovascular and Thoracic Surgery, University of California San Diego, La Jolla, California
| | - David McGiffin
- Department of Cardiothoracic Surgery, The Alfred Hospital and Monash University, Melbourne, VIC, Australia
| | - Christoph B Wiedenroth
- Department of Thoracic Surgery, Campus Kerckhoff of the University of Giessen, Kerckhoff Heart and Thorax Center, Bad Nauheim, Germany
| | - Sebastian Mafeld
- Division of Vascular and Interventional Radiology, Joint Department of Medical Imaging, University Health Network, Toronto, Ontario, Canada
| | - Isabelle Opitz
- Department of Thoracic Surgery, University Hospital Zurich, Zurich, Switzerland
| | - Olaf Mercier
- Department of Thoracic and Vascular Surgery and Heart Lung Transplantation, Marie-Lannelongue Hospital, Paris Saclay University, Le Plessis-Robinson, France
| | - Patricia A Uber
- Pauley Heart Center, Virginia Commonwealth University Health System, Richmond, Virginia
| | - Robert P Frantz
- Department of Cardiovascular Disease, Mayo Clinic College of Medicine, Rochester, Minnesota
| | - William R Auger
- Pulmonary Hypertension and CTEPH Research Program, Temple Heart and Vascular Institute, Temple University, Lewis Katz School of Medicine, Philadelphia, Pennsylvania
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22
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Li J, Liu L, Song LX, Zhang YH, Liu Y, Gu S, Wang JF, Huang Q, Ma ZH, Guo XJ, Yang MF, Jiang W, Li F, Yang YH. Clinical Features and Outcomes of Pulmonary Artery Sarcoma. Heart Lung Circ 2021; 31:230-238. [PMID: 34417115 DOI: 10.1016/j.hlc.2021.06.448] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2020] [Revised: 05/28/2021] [Accepted: 06/08/2021] [Indexed: 01/27/2023]
Abstract
OBJECTIVES A retrospective cohort study was designed to describe the clinical features and outcomes of pulmonary artery sarcoma (PAS). METHODS Twenty-two (22) consecutive patients diagnosed with PAS by pathological examination were enrolled and followed up until they died or until January 2020. The medical records were retrospectively reviewed to evaluate the clinical characteristics, image findings, and outcomes. RESULTS 1) Twenty-one (21, 95.5%) patients were firstly misdiagnosed. Dyspnoea was the most common presenting symptom (19 of 22, 86.4%). 2) Filling defects in the right pulmonary artery were seen in 17 patients (77.3%) with computed tomography pulmonary angiography or magnetic resonance pulmonary angiography. Among those patients, 14 underwent positron emission tomography-computed tomography detection and 13 (92.9%) were found to have increased uptake value in the pulmonary artery. 3) The median survival (from diagnosis to death or January 2020) of the total series was 11.6 months (range, 0.7-68.5 months). The estimated cumulative survival rates at 1, 2, and 3 years were 52.6%, 32.8%, and 19.7%, respectively. Patients who received surgery and/or chemo-radiotherapy treatment had a better survival rate compared with patients without treatment (the estimated cumulative survival rates at 1, 2, and 3 years were 60.3%, 39.1%, and 29.3%, respectively, vs 33.3%, 16.6%, and 0, accordingly) and better survival time (median survival 17.02 vs 3.16 months, respectively) (p=0.025). CONCLUSIONS Pulmonary artery sarcoma is easily misdiagnosed, as the symptoms and routine image detection are nonspecific. Positron emission tomography-computed tomography may be helpful in diagnosis. Surgery and/or chemo-radiotherapy offer a chance for better outcomes.
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Affiliation(s)
- Jifeng Li
- Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China. https://twitter.com/JifengLi5
| | - Lin Liu
- Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China
| | - Ling-Xie Song
- Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Yu-Hui Zhang
- Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China
| | - Yan Liu
- Department of Cardiac Surgery, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Song Gu
- Department of Cardiac Surgery, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Jian-Feng Wang
- Department of Interventional Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Qiang Huang
- Department of Interventional Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Zhan-Hong Ma
- Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Xiao-Juan Guo
- Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Min-Fu Yang
- Department of Nuclear Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Wei Jiang
- Department of Echocardiography, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Feng Li
- Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China
| | - Yuan-Hua Yang
- Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, P. R. China; Department of Respiratory Disease, Capital Medical University, Beijing, P. R. China.
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23
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Qin J, Ng CS, He P, Lin X, Lin X, Hou P. Pulmonary artery intimal sarcoma - A primeval or rediscovered tumor? A report of 14 new cases with literature review. Pathol Res Pract 2021; 224:153548. [PMID: 34280751 DOI: 10.1016/j.prp.2021.153548] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/26/2021] [Revised: 07/06/2021] [Accepted: 07/08/2021] [Indexed: 11/28/2022]
Abstract
Pulmonary artery sarcomas (PAS) are rare with many being undifferentiated pleomorphic or spindle cell (UPSC) sarcomas with variable atypia. The term pulmonary artery intimal sarcoma (PAIS) was rarely coined in the early literature and sometimes used for luminal sarcomas. With the advent of immunohistochemistry and molecular genetics, many of these UPSC sarcomas were found to frequently overpress MDM2 and/or CDK4 and PDGFRA with genetic alterations in 12q12-15 and 4q12, where the MDM2, CDK4 and PDGFRA genes are located. These recent developments enabled refinement in diagnosis of PAIS. We diagnosed 14 cases of PAIS (6 males and 8 females, mean age 44 years) in 2015 - 2020 in our institution. Six were initially misdiagnosed as thromboembolism and the remaining pulmonary artery tumors. The tumors were pulmonary artery intraluminal polypoid masses with histology of spindle cell sarcomas exhibiting immunohistochemical positivity for MDM2 (100%) and CDK4 (79%) with MDM2 gene amplification (100%). Ten surgically treated patients fared better than four other biopsy only and not surgically treated patients, who died of disease within 5-11 months. PAIS needs to be differentiated from other spindle cell tumors and those exhibiting MDM2 gene amplification, especially dedifferentiated liposarcoma. The use of biopsy to provide diagnostic material poses a sampling error problem and correlation with clinical, radiologic, histologic, immunophenotypic and genotypic features are essential for accurate diagnosis and early surgical intervention of PAIS.
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Affiliation(s)
- Jilong Qin
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Chi Sing Ng
- Department of Pathology, St. Teresa's Hospital, 327 Prince Edward Road, Kowloon, Hong Kong, China.
| | - Ping He
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Xiaodong Lin
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Xina Lin
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Peng Hou
- PET-CT Center, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
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24
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Raboso Moreno B, Aguilar-Rivilla E, Castillejos-Rodríguez L, Aramburu González JA, Matesanz López C. ¿Y si no fuese un tromboembolismo pulmonar? OPEN RESPIRATORY ARCHIVES 2021. [PMID: 37496765 PMCID: PMC10369546 DOI: 10.1016/j.opresp.2021.100110] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022] Open
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25
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Pulmonary artery sarcoma: A differential diagnosis of persistent pulmonary embolism. Respir Med Res 2021; 80:100842. [PMID: 34171553 DOI: 10.1016/j.resmer.2021.100842] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2021] [Revised: 04/30/2021] [Accepted: 05/29/2021] [Indexed: 11/21/2022]
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26
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Primary pulmonary artery sarcoma versus pulmonary thromboembolism: a multimodal imaging comparison. J Thromb Thrombolysis 2021; 52:1129-1132. [PMID: 33963485 PMCID: PMC8104460 DOI: 10.1007/s11239-021-02464-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/26/2021] [Indexed: 10/27/2022]
Abstract
Primary pulmonary artery sarcoma (PPAS) is a rare malignancy that is commonly mistaken for pulmonary embolism due to similarities in clinical presentation and radiographic findings. Distinct radiographic findings to help differentiate between the two diseases are highlighted in the case presented. (1) Several nuances in various imaging modalities have been identified to help distinguish pulmonary artery sarcoma from pulmonary thromboembolic disease. (2) The wall eclipsing sign is considered pathognomonic for pulmonary artery sarcoma. (3) Positron emission tomography/computed tomography may help reduce time between diagnosis and treatment, which may ultimately prolong survival. (4) Providers should be well versed on the subtle differences on imaging to prevent future delays in diagnosis and treatment.
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27
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Transient Response of Olaparib on Pulmonary Artery Sarcoma Harboring Multiple Homologous Recombinant Repair Gene Alterations. J Pers Med 2021; 11:jpm11050357. [PMID: 33946955 PMCID: PMC8146095 DOI: 10.3390/jpm11050357] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/08/2021] [Revised: 04/24/2021] [Accepted: 04/27/2021] [Indexed: 12/16/2022] Open
Abstract
Primary pulmonary artery sarcoma (PPAS) is a rare malignancy arising from mesenchymal pulmonary artery cells and mimics pulmonary embolism. Palliative chemotherapy such as anthracycline- or ifosfamide-based regimens and targeted therapy are the only options. However, the evidence of clinically beneficial systemic treatment is scarce. Here, we report a case of disseminated PPAS achieving clinical tumor response to olaparib based on comprehensive genetic profiling (CGP) showing genetic alterations involving DNA repair pathway. This provides supportive evidence that olaparib could be a promising therapeutic agent for patients with disseminated PPAS harboring actionable haploinsufficiency of DNA damage repair (DDR).
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Allen A, Smith SC, Pillappa R, Boikos S, Medalion B, Grizzard J, Cassano A, Harris T. Intimal sarcoma of the pulmonary artery treated with neoadjuvant radiation prior to pulmonary artery resection and reconstruction. Respir Med Case Rep 2021; 33:101414. [PMID: 34401262 PMCID: PMC8348529 DOI: 10.1016/j.rmcr.2021.101414] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2021] [Revised: 03/30/2021] [Accepted: 04/07/2021] [Indexed: 11/28/2022] Open
Abstract
Intimal sarcoma (IS) is a rare malignancy arising in the great vessels or heart, most commonly in the pulmonary artery, primarily treated with surgical intervention. We report a case of IS of the pulmonary artery diagnosed after an endarterectomy to remove a suspected pulmonary embolism. The tumor could not be entirely resected and showed interval growth at post-operative follow up. Neoadjuvant radiotherapy was then delivered to improve resectability. Imaging confirmed decreased tumor size, and a surgical resection with pulmonary artery reconstruction and right upper lobectomy was then successfully performed. Adjuvant gemcitabine and docetaxel was later initiated. Four months post-operatively, the patient is alive without disease recurrence. While prior reports in the literature document use of adjuvant chemotherapy and radiotherapy for treatment of IS of the pulmonary artery, no prior experience has documented utility of neoadjuvant radiotherapy for improvement of resectability. Our experience suggests that neoadjuvant radiation should be considered to improve resectability in cases of borderline resectable IS of the pulmonary artery.
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Affiliation(s)
- Alexander Allen
- Department of Radiation Oncology, Virginia Commonwealth University School of Medicine, 401 College Street, Richmond, VA, 23298-0037, USA
| | - Steven C Smith
- Department of Pathology, Division of Anatomic Pathology, Virginia Commonwealth University School of Medicine, 1200 East Marshall Street, Gateway Building, 6th Floor, Room 205, Box 980662, Richmond, VA, 23298-0662, USA
| | - Raghavendra Pillappa
- Department of Pathology, Division of Anatomic Pathology, Virginia Commonwealth University School of Medicine, 1200 East Marshall Street, Gateway Building, 6th Floor, Room 204, Box 980662, Richmond, VA, 23298-0662, USA
| | - Sosipatros Boikos
- Department of Internal Medicine, Division of Hematology, Oncology, and Palliative Care, Virginia Commonwealth University School of Medicine, 1201, East Marshall Street, 11th Floor, Suite 11-206, Box 980070, Richmond, VA, 23298-0230, USA
| | - Benjamin Medalion
- Department of Surgery, Division of Cardiothoracic Surgery, Virginia Commonwealth University School of Medicine, 1200 East Broad Street, 7th Floor, Box 980645, Richmond, VA, 23298-0068, USA
| | - John Grizzard
- Department of Radiology, Virginia Commonwealth University School of Medicine, 1200 East Broad Street, North Wing, Room 2-013, Box 980470, Richmond, VA, 23298-0470, USA
| | - Anthony Cassano
- Department of Surgery, Division of Cardiothoracic Surgery, Virginia Commonwealth University School of Medicine, 1200 East Broad Street, 7th Floor, Box 980645, Richmond, VA, 23298-0068, USA
| | - Timothy Harris
- Department of Radiation Oncology, Virginia Commonwealth University School of Medicine, 401 College Street, Box 980058, Richmond, VA, 23298-0037, USA
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29
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Vanni F, Scheggi V, Marchionni N, Stefàno PL. A new-onset pulmonary artery stenosis in a young man: case report. EUROPEAN HEART JOURNAL-CASE REPORTS 2021; 5:ytab118. [PMID: 33937621 PMCID: PMC8068665 DOI: 10.1093/ehjcr/ytab118] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/03/2020] [Revised: 11/30/2020] [Accepted: 03/04/2021] [Indexed: 11/14/2022]
Abstract
Background Poorly differentiated and undifferentiated sarcomas are the most common primary tumours of the pulmonary arteries. They usually affect large-calibre vessels and present with predominantly intraluminal growth. Dyspnoea, cough, chest pain, and haemoptysis are the most common presenting symptoms. Clinical and imaging manifestations can mimic pulmonary embolisms and correct diagnosis may require multimodal imaging. The overall prognosis is poor; however, early diagnosis and complete surgical resection seem to improve the prognosis. Case summary A 31-year-old male was admitted to our department after a pre-syncopal episode associated with dyspnoea of recent onset. Echocardiography showed a mass with irregular borders attached to the pulmonary artery trunk, almost obliterating its lumen and determining a flow acceleration with a peak velocity and gradient, respectively, of 3.8 m/s and 60 mmHg. At cardiac magnetic resonance imaging and positron emission tomography-computed tomography scan, the mass had inhomogeneous contrast impregnation and an intense 18-fluorodeoxyglucose uptake, both findings are highly suggestive of an angiosarcoma of the pulmonary artery. Biopsy specimens were taken through bronchoscopy but the material was insufficient for diagnosis. The patient decided to continue treatment in another hospital, where he died a few months later. Discussion The presence of a unique mass involving the main trunk of the pulmonary artery or proximal branches associated with rapidly progressive dyspnoea in a patient at low risk for pulmonary embolism should raise the suspicion of primary sarcoma of the pulmonary artery. There are no guidelines for the treatment. Surgery and neo/adjuvant chemotherapy are reported in literature but burdened by bias and concerning a small number of cases.
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Affiliation(s)
- Francesco Vanni
- Division of Cardiovascular and Perioperative Medicine, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
| | - Valentina Scheggi
- Division of Cardiovascular and Perioperative Medicine, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
| | - Niccolò Marchionni
- Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Division of General Cardiology, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
| | - Pier Luigi Stefàno
- Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Division of Cardiac Surgery, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
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30
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Nguyen ET, Hague C, Manos D, Memauri B, Souza C, Taylor J, Dennie C. Canadian Society of Thoracic Radiology/Canadian Association of Radiologists Best Practice Guidance for Investigation of Acute Pulmonary Embolism, Part 2: Technical Issues and Interpretation Pitfalls. Can Assoc Radiol J 2021; 73:214-227. [PMID: 33781102 DOI: 10.1177/08465371211000739] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
The investigation of acute pulmonary embolism is a common task for radiologists in Canada. Technical image quality and reporting quality must be excellent; pulmonary embolism is a life-threatening disease that should not be missed but overdiagnosis and unnecessary treatment should be avoided. The most frequently performed imaging investigation, computed tomography pulmonary angiogram (CTPA), can be limited by poor pulmonary arterial opacification, technical artifacts and interpretative errors. Image quality can be affected by patient factors (such as body habitus, motion artifact and cardiac output), intravenous (IV) contrast protocols (including the timing, rate and volume of IV contrast administration) and common physics artifacts (including beam hardening). Mimics of acute pulmonary embolism can be seen in normal anatomic structures, disease in non-vascular structures and pulmonary artery filling defects not related to acute pulmonary emboli. Understanding these pitfalls can help mitigate error, improve diagnostic quality and optimize patient outcomes. Dual energy computed tomography holds promise to improve imaging diagnosis, particularly in clinical scenarios where routine CTPA may be problematic, including patients with impaired renal function and patients with altered cardiac anatomy.
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Affiliation(s)
- Elsie T Nguyen
- Joint Department of Medical Imaging, 33540Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada
| | - Cameron Hague
- Department of Radiology, 12358University of British Columbia, Vancouver, Canada
| | - Daria Manos
- Department of Diagnostic Radiology, 3688Dalhousie University, Halifax, Nova Scotia, Canada
| | - Brett Memauri
- Cardiothoracic Sciences Division, St. Boniface General Hospital, 12359University of Manitoba, Winnipeg, Manitoba, Canada
| | - Carolina Souza
- Department of Medical Imaging, 10055The Ottawa Hospital, University of Ottawa, Ottawa, Ontario, Canada
| | - Jana Taylor
- 54473McGill University Health Centre, Montreal, Quebec, Canada
| | - Carole Dennie
- Department of Medical Imaging, 10055The Ottawa Hospital, University of Ottawa, Ottawa, Ontario, Canada
- 27337Ottawa Hospital Research Institute, University of Ottawa, Ottawa, Ontario, Canada
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31
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Ropp AM, Burke AP, Kligerman SJ, Leb JS, Frazier AA. Intimal Sarcoma of the Great Vessels. Radiographics 2021; 41:361-379. [PMID: 33646906 DOI: 10.1148/rg.2021200184] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Intimal sarcomas of the pulmonary artery and aorta are rare entities with a poor prognosis. In many instances, pulmonary artery sarcomas are misinterpreted as acute or chronic pulmonary thromboembolism, whereas aortic intimal sarcomas are often misdiagnosed as protuberant atherosclerotic disease or intimal thrombus. Discernment of intimal sarcomas from these and other common benign entities is essential for the timely initiation of aggressive therapy. The most useful imaging modalities for assessment of a suspected intimal sarcoma include CT angiography, fluorine 18-fluorodeoxyglucose PET, and MRI. The authors discuss the clinical features, current treatment options, characteristic imaging findings, and underlying pathologic features of intimal sarcomas. The authors emphasize imaging discernment of intimal sarcomas and how their differential diagnosis is informed by knowledge of radiologic-pathologic correlation. The most reliable distinguishing imaging features are also emphasized to improve accurate and timely diagnosis. Online supplemental material is available for this article. ©RSNA, 2021.
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Affiliation(s)
- Alan M Ropp
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Allen P Burke
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Seth J Kligerman
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Jay S Leb
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Aletta A Frazier
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
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32
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Gnanalingam C, Wright M, Ahmed Y. Pulmonary artery sarcoma masquerading as a pulmonary embolism. BMJ Case Rep 2021; 14:e232676. [PMID: 33637489 PMCID: PMC7919568 DOI: 10.1136/bcr-2019-232676] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/10/2020] [Indexed: 11/04/2022] Open
Abstract
A 52-year-old woman was referred to the respiratory team with worsening shortness of breath for the last 3 months, which had deteriorated significantly over the last 2-3 weeks. She underwent a CT pulmonary angiogram, which was reported locally as showing a large pulmonary embolism. Given the chronic history and appearance of the thrombus, the patient was referred to a specialist pulmonary vascular disease multidisciplinary team and underwent a PET-CT scan with the diagnosis being felt to be more consistent with a pulmonary artery sarcoma. Within 4 weeks, she underwent a pulmonary endarterectomy which confirmed the presence of an extensive mass. She underwent bilateral endarterectomy and pulmonary artery valve replacement, with subsequent improvement of her breathlessness back to premorbid baseline. Postoperative histology has confirmed a pulmonary artery angiosarcoma. Alternate imaging modalities and early referral to a specialist unit allowed as early a diagnosis as possible with good symptomatic benefit.
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Affiliation(s)
| | - Matthew Wright
- Respiratory Medicine, Southend Hospital, Westcliff-on-Sea, Essex, UK
| | - Yasser Ahmed
- Respiratory Medicine, Southend Hospital, Westcliff-on-Sea, Essex, UK
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33
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Song W, Deng L, Zhu J, Zheng S, Wang H, Song Y, Liu S. Surgical treatment of pulmonary artery sarcoma: a report of 17 cases. Pulm Circ 2021; 11:2045894020986394. [PMID: 33532061 PMCID: PMC7829461 DOI: 10.1177/2045894020986394] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2020] [Accepted: 12/14/2020] [Indexed: 11/21/2022] Open
Abstract
Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis
is often delayed, and optimal treatment remains unclear. The aim of this study
is to report our experience in the surgical management of this disease. Between
2000 and 2018, 17 patients underwent operations for PAS at our center. The
medical records were retrospectively reviewed to evaluate the clinical
characteristics, operative findings, the postoperative outcomes, and the
long-term results. The mean age at operation was 46.0 ± 12.4 years (range, 26–79
years), and eight (47.1%) patients were male. Six patients underwent tumor
resection alone, whereas the other 11 patients received pulmonary endarterectomy
(PEA). There were two perioperative deaths. Follow-up was completed for all
patients with a mean duration of 23.5 ± 17.6 months (1–52 months). For all 17
patients, the median postoperative survival was 36 months, and estimated
cumulative survival rates at 1, 2, 3, and 4 years were 60.0%, 51.4%, 42.9%, and
21.4%, respectively. The mean survival was 37.0 months after PEA and 14.6 months
after tumor resection only (p = 0.046). Patients who had no
pulmonary hypertension (PH) postoperatively were associated with improved median
survival (48 vs. 5 months, p = 0.023). In conclusion, PAS is
often mistaken for chronic pulmonary thromboembolism. The prognosis of this very
infrequent disease remains poor. Early detection is essential for prompt and
best surgical approach, superior to tumor resection alone, and PEA surgery with
PH relieved can provide better chance of survival.
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Affiliation(s)
- Wu Song
- Department of Cardiac Surgery, FuWai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Long Deng
- Department of Cardiac Surgery, FuWai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Jiade Zhu
- Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China
| | - Shanshan Zheng
- Department of Cardiac Surgery, FuWai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Haiping Wang
- Department of Radiology, FuWai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Yunhu Song
- Department of Cardiac Surgery, FuWai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Sheng Liu
- Department of Cardiac Surgery, FuWai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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34
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Calin E, Goudreault M, Demers P. Surgical management of a primary pulmonary artery sarcoma: A case report. J Card Surg 2020; 36:363-366. [PMID: 33225465 DOI: 10.1111/jocs.15202] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2020] [Revised: 11/02/2020] [Accepted: 11/04/2020] [Indexed: 11/28/2022]
Abstract
Pulmonary artery (PA) sarcoma is a rare tumor with an overall poor prognosis. It can often be misdiagnosed due to its nonspecific presentation and its similitude to pulmonary embolism on imaging. We, herein, describe the case of a 60-year-old male with a primary PA sarcoma for which a surgical approach was undertaken. Due to the low incidence of PA and therefore the absence of clear guidelines on the management of this disease, we also present a succinct literature review on the subject.
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Affiliation(s)
- Eliza Calin
- Department of Cardiovascular Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada
| | - Marianne Goudreault
- Department of Cardiovascular Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada
| | - Philippe Demers
- Department of Cardiovascular Surgery, Montreal Heart Institute, University of Montreal, Montreal, Quebec, Canada
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35
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Wang J, Li TL, Zheng S. A primary pulmonary artery sarcoma associated with multiple lesions. World J Emerg Med 2020; 11:270-273. [PMID: 33014227 DOI: 10.5847/wjem.j.1920-8642.2020.04.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Affiliation(s)
- Jing Wang
- Department of Geriatric, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China
| | - Tian-Lang Li
- Department of Geriatric, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China
| | - Shu Zheng
- Institution of Tumor, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China
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36
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Pulmonary Artery Intimal Sarcoma: A Diagnostic Challenge Using a Multimodal Approach. Case Rep Med 2020; 2020:6795272. [PMID: 33082786 PMCID: PMC7556087 DOI: 10.1155/2020/6795272] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2020] [Accepted: 09/23/2020] [Indexed: 12/05/2022] Open
Abstract
Pulmonary artery intimal sarcoma (PAIS) is a rare tumor without clear syndromic presentation other than nonspecific symptoms of cough, dyspnea, and weight loss. This diagnosis is difficult due to challenging radiographic interpretations of multiple imaging modalities. We present a case of a 60-year-old male, who presented to his pulmonologist and underwent a CT chest with IV contrast that initially suggested primary lung carcinoma. CT angiogram showed significant vascular filling defects suspicious of an intravascular mass, rather than vascular invasion by lung lesions. The PET/CT scans further suggested a malignant process, but indistinguishable between an extravascular or intravascular etiology. Taking these results together, they suggested an intravascular malignancy, prompting a tissue biopsy, which ultimately led to a diagnosis of PAIS with metastases. Establishing a definitive diagnosis is essential as treatment and prognosis are different for sarcoma compared to carcinoma. There is no standard treatment to date, and management often includes a multidisciplinary approach involving surgery, radiation, chemotherapy, and targeted therapy. PAIS is a rare entity that cannot be diagnosed clinically and needs a multimodality approach for its diagnosis.
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37
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Han Y, Zhen Y, Liu X, Zheng X, Zhang J, Zhai Z, Duan J, Zhang Y, Liu P. Surgical treatment of primary pulmonary artery sarcoma. Gen Thorac Cardiovasc Surg 2020; 69:638-645. [PMID: 32918676 PMCID: PMC7981312 DOI: 10.1007/s11748-020-01476-2] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2020] [Accepted: 08/27/2020] [Indexed: 10/29/2022]
Abstract
OBJECTIVE Primary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS. METHODS From September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data. RESULTS All of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7-28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve. CONCLUSIONS PAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.
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Affiliation(s)
- Yongxin Han
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, 100029, China
| | - Yanan Zhen
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing, 100029, China
| | - Xiaopeng Liu
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing, 100029, China
| | - Xia Zheng
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing, 100029, China
| | - Jianbin Zhang
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing, 100029, China
| | - Zhenguo Zhai
- Respiratory Department, China-Japan Friendship Hospital, Beijing, China
| | - Jun Duan
- Surgical Intensive Care Unit, China-Japan Friendship Hospital, Beijing, China
| | - Yajun Zhang
- Surgical Anesthesia Department, China-Japan Friendship Hospital, Beijing, China
| | - Peng Liu
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, 100029, China. .,Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing, 100029, China.
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38
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Cervilla-Muñoz E, Galeano-Valle F, Del-Toro-Cervera J, Calleja-Cartón E, Demelo-Rodríguez P. Differential diagnosis and treatment approach to pulmonary artery sarcoma: a case report and literature review. ERJ Open Res 2020; 6:00124-2020. [PMID: 32743004 PMCID: PMC7383050 DOI: 10.1183/23120541.00124-2020] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/08/2020] [Accepted: 05/05/2020] [Indexed: 11/05/2022] Open
Abstract
Pulmonary artery sarcoma (PAS) is a rare tumour that originates from the intimal layer of the pulmonary trunk and pulmonary arteries. Diagnosis of PAS is challenging, since it can be difficult to distinguish from a pulmonary embolism. The evidence regarding diagnostic techniques, management of the disease and prognosis is limited to a small series of cases and case reports [1, 2]. Pulmonary artery sarcoma is an aggressive tumour that originates from the intimal layer of the pulmonary trunk and pulmonary arteries. Clinical presentation may resemble a pulmonary embolism. Prompt diagnosis is necessary to improve survival.https://bit.ly/2xZwENm
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Affiliation(s)
- Eva Cervilla-Muñoz
- Venous Thromboembolism Unit, Internal Medicine. General University Hospital Gregorio Marañón, Madrid, Spain.,Dept of Medicine, School of Medicine, Universidad Complutense, Madrid, Spain
| | - Francisco Galeano-Valle
- Venous Thromboembolism Unit, Internal Medicine. General University Hospital Gregorio Marañón, Madrid, Spain.,Dept of Medicine, School of Medicine, Universidad Complutense, Madrid, Spain.,Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Jorge Del-Toro-Cervera
- Venous Thromboembolism Unit, Internal Medicine. General University Hospital Gregorio Marañón, Madrid, Spain.,Dept of Medicine, School of Medicine, Universidad Complutense, Madrid, Spain.,Gregorio Marañón Health Research Institute, Madrid, Spain
| | - Enrique Calleja-Cartón
- Vascular Radiology, Hospital General Universitario Gregorio Marañón Gregorio Marañón, Madrid, Spain
| | - Pablo Demelo-Rodríguez
- Venous Thromboembolism Unit, Internal Medicine. General University Hospital Gregorio Marañón, Madrid, Spain.,Dept of Medicine, School of Medicine, Universidad Complutense, Madrid, Spain.,Gregorio Marañón Health Research Institute, Madrid, Spain
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Ziatabar S, Rabii K, Oron MN, Shah V, Altschul E, Ishikawa O, Gabra NI, Mahmoud OH, Mina BA. A 44-Year-Old Man With Dyspnea and a Pulmonary Artery Filling Defect. Chest 2020; 156:e103-e106. [PMID: 31699232 DOI: 10.1016/j.chest.2019.06.026] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2019] [Revised: 06/02/2019] [Accepted: 06/24/2019] [Indexed: 12/01/2022] Open
Abstract
CASE PRESENTATION A 44-year-old man with a history of coronary artery disease, type 2 diabetes mellitus, and OSA reported progressively worsening dyspnea on exertion over a 6-week period. Outpatient CT angiogram revealed a pulmonary artery filling defect. He was sent to the ED where he was started on a heparin drip for unprovoked pulmonary embolism (PE). Echocardiogram revealed normal cardiac function without evidence of right heart strain. Lower extremity ultrasound was negative for DVT. He improved symptomatically, and no risk factors for PE were identified. He was discharged on apixaban. Five weeks later, the patient returned to the ED with hemoptysis. He reported compliance with anticoagulation and improvement of his dyspnea on exertion. History remained negative for recent travel, trauma, surgery, clotting disorders, thromboembolic disease, and alcohol or drug use. He had a 60 pack-year cigarette smoking history and quit 3 months prior.
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Affiliation(s)
- Sally Ziatabar
- Department of Internal Medicine, Lenox Hill Hospital, New York, NY.
| | - Kevin Rabii
- Department of Internal Medicine, Lenox Hill Hospital, New York, NY
| | - Maly N Oron
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Lenox Hill Hospital, New York, NY
| | - Varun Shah
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Lenox Hill Hospital, New York, NY
| | - Erica Altschul
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Lenox Hill Hospital, New York, NY
| | - Oki Ishikawa
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Lenox Hill Hospital, New York, NY
| | - Nader Ishak Gabra
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Lenox Hill Hospital, New York, NY
| | - Omar H Mahmoud
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Lenox Hill Hospital, New York, NY
| | - Bushra A Mina
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Lenox Hill Hospital, New York, NY
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40
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Kim YY, Wynn TT, Reith JD, Slayton WB, Lagmay J, Fort J, Rajderkar DA. Primary pulmonary artery sarcoma in the pediatric patient: Review of literature and a case report. Radiol Case Rep 2020; 15:1110-1114. [PMID: 32477441 PMCID: PMC7248585 DOI: 10.1016/j.radcr.2020.05.016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2020] [Revised: 05/07/2020] [Accepted: 05/08/2020] [Indexed: 11/27/2022] Open
Abstract
Primary pulmonary artery sarcoma (PAS) is extremely rare in children. Nevertheless, distinguishing primary PAS from pulmonary embolism is critical to a child's survival. Primary PAS is commonly misdiagnosed as a pulmonary embolism due to similar presenting symptoms and radiographic findings. However, compared to adults, pulmonary embolism is rare in children, especially in patients who do not have predisposing factors or hypercoagulable state. We present a child with primary PAS which mimicked pulmonary embolism on presentation but eventually was resected and is doing well 5 years after resection. In the absence of predisposing factors or hypercoagulable state, solid tumors such as primary PAS should be considered when assessing a pediatric patient with presumed pulmonary embolism.
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Affiliation(s)
- Yong Yean Kim
- National Cancer Institute, Pediatric Oncology Branch, 37 Convent Dr, Bethesda, MD 20892, USA
| | - Tung Thanh Wynn
- University of Florida, Department of Pediatric Hematology/Oncology, Gainesville, FL 32610, USA
| | - John David Reith
- Cleveland Clinic, Department of Pathology, Cleveland, OH 44195, USA
| | - William B Slayton
- University of Florida, Department of Pediatric Hematology/Oncology, Gainesville, FL 32610, USA
| | - Joanne Lagmay
- University of Florida, Department of Pediatric Hematology/Oncology, Gainesville, FL 32610, USA
| | - John Fort
- University of Florida, Department of Pediatric Hematology/Oncology, Gainesville, FL 32610, USA
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41
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Lu P, Yin BB. Misdiagnosis of primary intimal sarcoma of the pulmonary artery as chronic pulmonary embolism: A case report. World J Clin Cases 2020; 8:986-994. [PMID: 32190637 PMCID: PMC7062609 DOI: 10.12998/wjcc.v8.i5.986] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2019] [Revised: 01/16/2020] [Accepted: 02/03/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery, which has a low incidence rate and is easily misdiagnosed as pulmonary embolism. There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.
CASE SUMMARY This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017. The clinical characteristics, diagnosis, treatment and outcome of the patient were retrospectively analyzed. The patient was a Chinese Han male aged 44 years. He had three consecutive episodes of syncope, and was thus admitted to a local hospital. Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk, left pulmonary artery, mediastinum and pericardium, which were consistent with recurrence after tumor resection. He underwent surgery, and was pathologically diagnosed with intimal sarcoma of the pulmonary artery. He relapsed 3 mo after surgery, and apatinib was administered. His condition was stable after 4 mo, with tolerable and controllable adverse reactions. He subsequently died 19 mo after surgery.
CONCLUSION Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations. The diagnosis of this disease depends on histopathology and immunohistochemistry, and has a poor clinical prognosis. Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery, and targeted therapy may provide new insights for the development of effective treatment methods.
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Affiliation(s)
- Ping Lu
- Department of Cardiac Surgery, The First Affiliated Hospital of Shandong First Medical University, Jinan 250014, Shandong Province, China
| | - Bei-Bei Yin
- Department of Oncology, The First Affiliated Hospital of Shandong First Medical University, Jinan 250014, Shandong Province, China
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42
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A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery. Crit Rev Oncol Hematol 2020; 147:102889. [PMID: 32035299 DOI: 10.1016/j.critrevonc.2020.102889] [Citation(s) in RCA: 27] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2018] [Revised: 01/20/2020] [Accepted: 01/28/2020] [Indexed: 02/06/2023] Open
Abstract
Only a few hundred cases of intimal sarcomas of pulmonary artery (ISPA) were reported on the literature. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of ISPA. Treatment strategies rely initially on an early aggressive surgery aiming for complete surgical resection with clear margins while no clear recommendations guiding the choice for additional drug therapy or radiotherapy exist. In this article, we perform an extensive review of the literature on ISPA with details on the clinical presentation, diagnosis and management strategies. An additional goal of this paper is to make practicing oncologists aware of this rare entity with clear idea on the initial management.
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43
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Narechania S, Renapurkar R, Heresi GA. Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review. Pulm Circ 2020; 10:2045894019882620. [PMID: 32257112 PMCID: PMC7103595 DOI: 10.1177/2045894019882620] [Citation(s) in RCA: 31] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/22/2019] [Accepted: 09/21/2019] [Indexed: 12/20/2022] Open
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by mechanical obstruction of large pulmonary arteries secondary to one or more episodes of pulmonary embolism. Ventilation perfusion scan is the recommended initial screening test for this condition and typically shows multiple large mismatched perfusion defects. However, not all patients with an abnormal ventilation perfusion scan have CTEPH since there are other conditions that be associated with a positive ventilation perfusion scan. These conditions include in situ thrombosis, pulmonary artery sarcoma, fibrosing mediastinitis, pulmonary vasculitis and sarcoidosis, among others. Although these conditions cannot be distinguished from CTEPH using a ventilation perfusion scan, they have certain characteristic radiological features that can be demonstrated on other imaging techniques such as computed tomography scan and can help in differentiation of these conditions. In this review, we have summarized some key clinical and radiological features that can help differentiate CTEPH from the CTEPH mimics.
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Affiliation(s)
| | - Rahul Renapurkar
- Department of Diagnostic Radiology,
Cleveland
Clinic, Cleveland, OH, USA
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44
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Frezza AM, Assi T, Lo Vullo S, Ben-Ami E, Dufresne A, Yonemori K, Noguchi E, Siontis B, Ferraro R, Teterycz P, Duffaud F, Ravi V, Vincenzi B, Gelderblom H, Pantaleo MA, Baldi GG, Desar I, Fedenko A, Maki RG, Jones RL, Benjamin RS, Blay JY, Kawai A, Gounder M, Gronchi A, Le Cesne A, Mir O, Czarnecka AM, Schuetze S, Wagner AJ, Adam J, Barisella M, Sbaraglia M, Hornick JL, Meurgey A, Mariani L, Casali PG, Thornton K, Stacchiotti S. Systemic treatments in MDM2 positive intimal sarcoma: A multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network. Cancer 2019; 126:98-104. [PMID: 31536651 DOI: 10.1002/cncr.32508] [Citation(s) in RCA: 25] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2019] [Revised: 06/22/2019] [Accepted: 07/08/2019] [Indexed: 11/09/2022]
Abstract
BACKGROUND Intimal sarcoma (InS) is an exceedingly rare neoplasm with an unfavorable prognosis, for which new potentially active treatments are under development. We report on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in patients with InS. METHODS Seventeen sarcoma reference centers in Europe, the United States, and Japan contributed data to this retrospective analysis. Patients with MDM2-positive InS who were treated with anthracycline-based regimens, gemcitabine-based regimens, or pazopanib between October 2001 and January 2018 were selected. Local pathological review was performed to confirm diagnosis. Response was assessed by RECIST1.1. Recurrence-free survival (RFS), progression-free survival (PFS) and overall survival were computed by Kaplan-Meier method. RESULTS Seventy-two patients were included (66 anthracycline-based regimens; 26 gemcitabine-based regimens; 12 pazopanib). In the anthracycline-based group, 24 (36%) patients were treated for localized disease, and 42 (64%) patients were treated for advanced disease. The real-world overall response rate (rwORR) was 38%. For patients with localized disease, the median RFS was 14.6 months. For patients with advanced disease, the median PFS was 7.7 months. No anthracycline-related cardiac toxicity was reported in patients with cardiac InS (n = 26). For gemcitabine and pazopanib, the rwORR was 8%, and the median PFS was 3.2 and 3.7 months, respectively. CONCLUSION This retrospective series shows the activity of anthracycline-based regimens in InS. Of note, anthracyclines were used in patients with cardiac InS with no significant cardiac toxicity. The prognosis in patients with InS remains poor, and new active drugs and treatment strategies are needed.
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Affiliation(s)
- Anna Maria Frezza
- Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy
| | - Tarek Assi
- Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France
| | - Salvatore Lo Vullo
- Unit of Clinical Epidemiology and Trial Organization, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy
| | - Eytan Ben-Ami
- Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts
| | - Armelle Dufresne
- Department of Medical Oncology, Centre Léon Bérard & Université Claude Bernard Lyon I, Lyon, France
| | - Kan Yonemori
- Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Emi Noguchi
- Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Brittany Siontis
- Department of Medicine, University of Michigan, Ann Arbor, Michigan
| | - Richard Ferraro
- Department of Medicine, Sarcoma Medical Oncology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York
| | - Pawel Teterycz
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Institute-Oncology Center, Warsaw, Poland
| | - Florence Duffaud
- Department of Medical Oncology, La Timone University Hospital, Aix-Marseille Université, Marseille, France
| | - Vinod Ravi
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Bruno Vincenzi
- Department of Medical Oncology, Università Campus Bio-Medico di Roma, Rome, Italy
| | - Hans Gelderblom
- Department of Medical Oncology, Leiden University Medical Center, Leiden, Netherlands
| | - Maria A Pantaleo
- Department of Specialized, Experimental and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
| | - Giacomo G Baldi
- Department of Medical Oncology, Nuovo Ospedale "S. Stefano,", Prato, Italy
| | - Ingrid Desar
- Department of Medical Oncology, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Alexander Fedenko
- Department of Medical Oncology, N. N. Blokhin Russian Cancer Research, Moscow, Russian Federation
| | - Robert G Maki
- Medical Oncology, Northwell Cancer Institute and Cold Spring Harbor Laboratory, Long Island, New York
| | - Robin L Jones
- Sarcoma Unit, Royal Marsden NHS Foundation Trust/Institute of Cancer Research, London, United Kingdom
| | - Robert S Benjamin
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Jean Yves Blay
- Department of Medical Oncology, Centre Léon Bérard & Université Claude Bernard Lyon I, Lyon, France
| | - Akira Kawai
- Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Mrinal Gounder
- Department of Medicine, Sarcoma Medical Oncology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York
| | - Alessandro Gronchi
- Sarcoma Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
| | - Axel Le Cesne
- Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France
| | - Olivier Mir
- Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France
| | - Anna M Czarnecka
- Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Institute-Oncology Center, Warsaw, Poland
| | - Scott Schuetze
- Department of Medicine, University of Michigan, Ann Arbor, Michigan
| | - Andrew J Wagner
- Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts
| | - Julien Adam
- Department of Pathology, Gustave Roussy Cancer Campus, Villejuif, France
| | - Marta Barisella
- Department of Diagnostic Pathology and Laboratory Medicine, IRCCS Fondazione Istituto Nazionale dei Tumori, Milan, Italy
| | | | - Jason L Hornick
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts
| | | | - Luigi Mariani
- Unit of Clinical Epidemiology and Trial Organization, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy
| | - Paolo G Casali
- Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy.,Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy
| | - Katherine Thornton
- Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts
| | - Silvia Stacchiotti
- Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy
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45
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Wu Y, Huang J, Wang Q, Zhang M, Luo Y, Wang X, Zhu X, Liu H. Whole-exome sequencing insights into pulmonary artery sarcoma mimicking pulmonary embolism: a case report and review. Onco Targets Ther 2019; 12:6227-6235. [PMID: 31496726 PMCID: PMC6689559 DOI: 10.2147/ott.s212416] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2019] [Accepted: 07/17/2019] [Indexed: 01/18/2023] Open
Abstract
Pulmonary artery sarcoma (PAS) is a rare and aggressive mesenchymal tumor that often mimics thromboembolic disease. Due to its rare and fatal nature, patients are often underdiagnosed or misdiagnosed. There is still no consensus regarding the diagnosis and treatment of PAS. We present a case of a 63 year old male misdiagnosed with pulmonary thromboembolism who received anticoagulant therapy. 18FDG positron emission tomography (PET) integrated with computed tomography (PET/CT) and subsequent surgery led to the final diagnosis of PAS. Whole exome sequencing of the tissue identified the genetic alterations profile of PAS: copy number variation (CNV) of KIT and mutations of TP53, PIK3CA, IL7R and ATR. Treated with chemotherapy followed by anlotinib, the patient's survival time was 8 months after firm diagnosis. To our knowledge, anlotinib used as a treatment for PAS has not been reported.
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Affiliation(s)
- Ying Wu
- Department of Respiratory Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, People's Republic of China
| | - Jing Huang
- Department of Respiratory Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, People's Republic of China
| | - Qin Wang
- Department of Respiratory Medicine, Jinling Hospital, Nanjing University School of Medicine, Nanjing 210002, People's Republic of China
| | - Mei Zhang
- Department of Respiratory Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, People's Republic of China
| | - Yimin Luo
- Department of Respiratory Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, People's Republic of China
| | - Xihua Wang
- Department of Respiratory Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, People's Republic of China
| | - Xiaoli Zhu
- Department of Respiratory Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, People's Republic of China
| | - Hongbing Liu
- Department of Respiratory Medicine, Jinling Hospital, Nanjing University School of Medicine, Nanjing 210002, People's Republic of China
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46
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Feng Y, Xu T, Wang C, Yu D, Shi K, Wang J. Pulmonary artery sarcoma mimicking pneumonia: case report and literature review. INTERNATIONAL JOURNAL OF CLINICAL AND EXPERIMENTAL PATHOLOGY 2019; 12:3620-3624. [PMID: 31934212 PMCID: PMC6949820] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 06/28/2019] [Accepted: 08/26/2019] [Indexed: 06/10/2023]
Abstract
Pulmonary artery sarcoma is a highly malignant and very rare tumor with a high rate of misdiagnosis. We present a case of a patient who complained of recurring fever for six months. He was diagnosed with pneumonia in a tertiary hospital in Nanjing and treated for more than one month, and was admitted with an initial diagnosis of pneumonia to our hospital. Pulmonary CT Angiography (CTPA) demonstrated pulmonary embolism. Until positron emission tomography-computed tomography (PET-CT) was performed, the patient was suspected to have pulmonary artery sarcoma (PAS). According to the histopathology from total left lung resection, he was eventually diagnosed with PAS and recovered very well. The prognosis of PAS depends very closely on early surgical treatment, and therefore physicians must be aware of PAS and grasp the best treatment opportunity.
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Affiliation(s)
- Yuejuan Feng
- Department of Respiratory Diseases, The Affiliated Hospital of Hangzhou Normal University Hangzhou, Zhejiang, China
| | - Ting Xu
- Department of Respiratory Diseases, The Affiliated Hospital of Hangzhou Normal University Hangzhou, Zhejiang, China
| | - Cancan Wang
- Department of Respiratory Diseases, The Affiliated Hospital of Hangzhou Normal University Hangzhou, Zhejiang, China
| | - Dongwei Yu
- Department of Respiratory Diseases, The Affiliated Hospital of Hangzhou Normal University Hangzhou, Zhejiang, China
| | - Kai Shi
- Department of Respiratory Diseases, The Affiliated Hospital of Hangzhou Normal University Hangzhou, Zhejiang, China
| | - Jianfeng Wang
- Department of Respiratory Diseases, The Affiliated Hospital of Hangzhou Normal University Hangzhou, Zhejiang, China
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47
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Ro A, Mori S, Sugiura H, Furukawa T, Asakura K, Kimura S, Kageyama N, Chiba S, Mukai T. An autopsy case of pulmonary artery intimal sarcoma: detailed observation of tumor and its related lesions in pulmonary arteries. Cardiovasc Pathol 2019; 43:107143. [PMID: 31437715 DOI: 10.1016/j.carpath.2019.07.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/19/2019] [Revised: 07/03/2019] [Accepted: 07/12/2019] [Indexed: 11/16/2022] Open
Abstract
We report an autopsy-proven case of a 33-year-old man who died of intimal sarcoma of the pulmonary artery. A large mass (5×4 cm) occluded the main and bilateral pulmonary arteries. Tumor cell morphology was consistent with that of undifferentiated pleomorphic sarcoma. Comprehensive histological observation of 18 pulmonary arteries from proximal to distal revealed continuous extension of the tumor from the main to the subsegmental arteries along the intima, forming an arteriosclerosis-like intimal thickening. Distal small arteries were also affected by eccentric intimal thickening or recanalization. Lung parenchyma was not involved, although there were two wedge-shaped small pulmonary infarctions caused by tumorous obstruction of the associated arteries. Histological results indicated that the intimal sarcoma in the pulmonary artery, which appeared occlusive with growth limited to the proximal artery, had in fact already spread more peripherally than expected. Both the proximal lesions and the distal small arteries were affected by peripheral tumor emboli or by pulmonary hypertension induced by the proximal tumor. However, as seen in this case, most of the occlusive tumor was located locally and intraluminally, in the proximal artery, and removing the proximal tumor by pulmonary endarterectomy was considered effective for symptomatic improvement.
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Affiliation(s)
- Ayako Ro
- Department of Legal Medicine, St. Marianna University School of Medicine, Kanagawa, 216-8511, Japan; Tokyo Medical Examiner's Office, Tokyo,112-0012, Japan.
| | | | - Hiroaki Sugiura
- Department of Radiology, National Defense Medical College, Saitama, 359-8513, Japan
| | - Toshiyuki Furukawa
- Syncope Unit, Toyoko Hospital, St. Marianna University, Kanagawa, 211-0063, Japan
| | | | - Satoko Kimura
- Tokyo Medical Examiner's Office, Tokyo,112-0012, Japan
| | - Norimasa Kageyama
- Department of Legal Medicine, St. Marianna University School of Medicine, Kanagawa, 216-8511, Japan; Tokyo Medical Examiner's Office, Tokyo,112-0012, Japan
| | - Shoetsu Chiba
- Department of Legal Medicine, St. Marianna University School of Medicine, Kanagawa, 216-8511, Japan
| | - Toshiji Mukai
- Department of Legal Medicine, St. Marianna University School of Medicine, Kanagawa, 216-8511, Japan
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48
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Direct Connection: A Man with Lung Nodules and Filling Defects in the Pulmonary Arterial Tree. Ann Am Thorac Soc 2019; 14:1844-1848. [PMID: 29192821 DOI: 10.1513/annalsats.201706-511cc] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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49
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Chiola I, Belgioia L, Vaccara EML, Gusinu M, Corvò R. Reirradiation of pulmonary artery intimal sarcoma: A case report. Clin Case Rep 2019; 7:1342-1346. [PMID: 31360482 PMCID: PMC6637327 DOI: 10.1002/ccr3.2216] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2019] [Revised: 04/02/2019] [Accepted: 05/05/2019] [Indexed: 11/23/2022] Open
Abstract
We report here the case of a patient with 4 years long-term survival after treatment with surgery, chemotherapy, and radiotherapy with good local control. This case highlights the possible role of radiation therapy in this tumor.
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Affiliation(s)
- Ilaria Chiola
- Health Science Department (DISSAL)University of GenoaGenoaItaly
| | - Liliana Belgioia
- Health Science Department (DISSAL)University of GenoaGenoaItaly
- Radiation Oncology DepartmentIRCCS San Martino HospitalGenoaItaly
| | | | - Marco Gusinu
- Medical Physics DepartmentIRCCS San Martino HospitalGenoaItaly
| | - Renzo Corvò
- Health Science Department (DISSAL)University of GenoaGenoaItaly
- Radiation Oncology DepartmentIRCCS San Martino HospitalGenoaItaly
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50
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Pulmonary artery sarcoma: Case report and review of the literature. Respir Med Case Rep 2019; 27:100857. [PMID: 31193694 PMCID: PMC6538954 DOI: 10.1016/j.rmcr.2019.100857] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2019] [Revised: 05/07/2019] [Accepted: 05/13/2019] [Indexed: 11/29/2022] Open
Abstract
Pulmonary artery sarcoma is a rare malignancy with poor prognosis which can be misdiagnosed as pulmonary thromboembolism. We present a case of a middle age woman who initially diagnosed with presumptive pulmonary embolism that was later found to have pulmonary artery sarcoma. Symptoms, pathology, imaging characteristics and available treatments are discussed.
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