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Telvizian T, May AC, Agyei O, Zeger E. Primary splenic angiosarcoma presenting as thrombocytopenia. BMJ Case Rep 2025; 18:e262536. [PMID: 40345677 DOI: 10.1136/bcr-2024-262536] [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: 05/11/2025] Open
Abstract
Primary splenic angiosarcoma is an extremely rare and aggressive vascular neoplasm associated with high rates of metastasis. A male patient in his 70s presented with thrombocytopenia. Physical examination and blood tests were non-revealing. A large splenic lesion was found on imaging, and splenectomy confirmed the diagnosis of splenic angiosarcoma. A PET scan and brain MRI showed no evidence of metastasis. The patient is now under surveillance with imaging. Primary splenic angiosarcoma presents a significant clinical challenge due to its aggressive nature and nonspecific symptoms, with outcomes typically poor despite treatment efforts. Chemotherapeutic options lack consensus due to the rarity of the disease. Most patients present with metastases, and treatment primarily involves resection, with adjuvant therapies considered based on risk factors. This case underscores the importance of considering rare malignancies in the differential diagnosis of patients presenting with thrombocytopenia and nonspecific symptoms. Early detection and prompt intervention are fundamental in improving outcomes.
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Affiliation(s)
- Talar Telvizian
- Hematology and Oncology, Lankenau Medical Center, Wynnewood, Pennsylvania, USA
| | - Alexander C May
- Hematology and Oncology, Lankenau Medical Center, Wynnewood, Pennsylvania, USA
| | - Obed Agyei
- Hematology and Oncology, Lankenau Medical Center, Wynnewood, Pennsylvania, USA
| | - Erik Zeger
- Hematology and Oncology, Lankenau Medical Center, Wynnewood, Pennsylvania, USA
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2
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Prasad AS, Chua SS, Ramani NS, Shiralkar KG, Shanbhogue KP, Surabhi VR. Stroma-derived neoplasms and pseudoneoplastic lesions of the spleen: a select review of pathologic and CT/MRI findings. Abdom Radiol (NY) 2025; 50:480-495. [PMID: 38937338 DOI: 10.1007/s00261-024-04461-y] [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: 04/07/2024] [Revised: 06/09/2024] [Accepted: 06/15/2024] [Indexed: 06/29/2024]
Abstract
A wide spectrum of benign and malignant primary mesenchymal tumors and tumor-like lesions of the spleen has been recently included under the umbrella term 'stroma-derived' neoplasms and tumor-like lesions. These include dendritic cell neoplasms such as follicular dendritic cell sarcoma, EBV-positive inflammatory follicular dendritic cell sarcoma, and fibroblastic reticular cell tumor; smooth muscle and myofibroblastic lesions such as inflammatory pseudotumor, EBV-associated smooth muscle tumor and undifferentiated pleomorphic sarcoma as well as a diverse spectrum of vascular and vascular-stromal tumors and tumor-like lesions. While some tumor and tumor-like lesions are unique to the spleen, others may also occur in diverse extra-splenic viscera. These tumors and tumor-like lesions demonstrate characteristic histopathology, immunocytochemistry and biological behavior. While cross-sectional imaging studies allow detection, staging and limited characterization of these splenic lesions, histopathological confirmation permits optimal management and surveillance strategies.
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Affiliation(s)
- Aditya S Prasad
- Departments of Radiology, University of Texas MD Anderson Cancer Center, Houston, USA
| | | | - Nisha S Ramani
- Department of Pathology, Michael E DeBakey VA Medical Center, Houston, USA
| | | | | | - Venkateswar R Surabhi
- Departments of Radiology, University of Texas MD Anderson Cancer Center, Houston, USA.
- Department of Abdominal Imaging, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1473, Houston, TX, 77030, USA.
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3
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Wächtershäuser EM, Köhler G, Böhmer V, Marx A, Hellinger A. Primary Liposarcoma of the Spleen: Case Report With Review of the Literature. Int J Surg Pathol 2024:10668969241291880. [PMID: 39533758 DOI: 10.1177/10668969241291880] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2024]
Abstract
We present a 55-year-old woman with a palpable mass in the right upper abdomen. Contrast-enhanced computed tomography showed a highly vascularized and primary resectable tumor of the spleen. We performed an open splenectomy with locoregional lymphadenectomy. Histopathological and immunohistochemical evaluation did not reveal classic markers of angiosarcoma, littoral cell angioma, or follicular dendritic cell sarcoma, but showed MDM2-amplification by FISH in all tumor cells as a marker for liposarcoma. The tumor showed growth limited to the spleen tissue. No MDM2-amplification was detectable in the perisplenic adipose tissue, so that infiltration of the spleen by retroperitoneal liposarcoma could be excluded. In summary, well-differentiated liposarcoma confined to the spleen was diagnosed. To the best of our knowledge, this is the first description of a primary liposarcoma of the spleen reported in the literature. Due to the local recurrence risk of liposarcomas, even after R0 resection, we recommended long-term periodic follow-up.
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Affiliation(s)
| | | | - Verena Böhmer
- Department of Pathology, Klinikum Fulda, Fulda, Germany
| | - Alexander Marx
- Department of Pathology, University of Göttingen, Göttingen, Germany
| | - Achim Hellinger
- Department of General and Visceral Surgery, Klinikum Fulda, Fulda, Germany
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Oh YJ, Lee JE, You SK, Ohm JY, Han HY, Kim JM, Shin KS. Multimodality Imaging Features of Various Splenic Lesions: Clinical and Histopathologic Correlation. JOURNAL OF THE KOREAN SOCIETY OF RADIOLOGY 2024; 85:1099-1125. [PMID: 39660321 PMCID: PMC11625836 DOI: 10.3348/jksr.2024.0004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/03/2024] [Revised: 04/02/2024] [Accepted: 04/24/2024] [Indexed: 12/12/2024]
Abstract
The spleen is occasionally referred to as the 'forgotten organ' because splenic lesions are less common and encountered rarely compared to pathologies of other abdominal solid organs. Therefore, although well-demonstrated using various abdominal imaging modalities, radiologists tend to be less familiar with splenic diseases, making interpretation challenging. This study aimed to review common and uncommon splenic diseases and illustrate the multimodal imaging (including ultrasonography, CT, MRI, and PET/CT) features of these lesions in correlation with their histopathology. Recognizing the radiological findings of various splenic lesions helps narrow down the differential diagnosis and guide appropriate clinical decision-making for radiologists.
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Awashra A, Sawaftah Z, Odah AB, Sawafta A, Khatib A, Dababat H, Yasin A, Khamaysa J, Daraghmeh M. Spontaneous splenic rupture as a primary manifestation of angiosarcoma: a case report. J Surg Case Rep 2024; 2024:rjae633. [PMID: 39380797 PMCID: PMC11460614 DOI: 10.1093/jscr/rjae633] [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: 08/06/2024] [Accepted: 09/24/2024] [Indexed: 10/10/2024] Open
Abstract
Angiosarcoma, a rare endothelial cell tumor, accounts for < 2% of all sarcomas, typically affecting elderly patients. We present the case of a 62-year-old male with a history of hypertension and ischemic heart disease, who initially presented with severe lower back pain. Subsequent imaging revealed a ruptured splenic lesion and multiple liver lesions, leading to a diagnosis of stage 4 angiosarcoma. Post-splenectomy, the patient exhibited pathological vertebral fractures and elevated liver function tests, consistent with metastatic disease. Despite aggressive surgical intervention and supportive care, the patient's condition remained critical. The treatment plan included palliative chemotherapy, spine radiotherapy, and ongoing pain management, with close monitoring of disease progression and response to therapy. This case describes the diagnostic challenges and poor prognosis associated with metastatic angiosarcoma.
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Affiliation(s)
- Ameer Awashra
- Department of Medicine, An Najah National University, Nablus P606, Palestine
| | - Zaid Sawaftah
- Department of Medicine, An Najah National University, Nablus P606, Palestine
| | - Ali Bani Odah
- Department of Medicine, An Najah National University, Nablus P606, Palestine
| | - Ahmed Sawafta
- Department of Medicine, An Najah National University, Nablus P606, Palestine
| | - Amer Khatib
- Department of Surgery, Tubas Turkish Governmental Hospital, Tubas P606, Palestine
| | - Huthayfa Dababat
- Department of Surgery, Tubas Turkish Governmental Hospital, Tubas P606, Palestine
| | - Anas Yasin
- Department of Surgery, Tubas Turkish Governmental Hospital, Tubas P606, Palestine
| | - Jehad Khamaysa
- Department of Radiology, Tubas Turkish Governmental Hospital, Tubas P606, Palestine
| | - Muath Daraghmeh
- Department of Radiology, Patient's Friends Society, Nablus P606, Palestine
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Roels E, Pierrot E, Porsmoguer C, Herrmann A, Fastrès A, Bolen G. Ultrasonographic and CT-scan findings of splenic active chylous effusion and postlymphangiography enhancement in a cat with chylothorax associated with splenic angiosarcoma. Vet Radiol Ultrasound 2024; 65:528-533. [PMID: 38804245 DOI: 10.1111/vru.13387] [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: 10/20/2023] [Revised: 05/07/2024] [Accepted: 05/08/2024] [Indexed: 05/29/2024] Open
Abstract
A 12-year-old domestic shorthair cat was presented with relapsing noncardiogenic chylothorax. CT-lymphangiography of the thorax confirmed bilateral pleural effusion without evidence of an underlying intra-thoracic origin. Abdominal CT-lymphangiography revealed a 2.6 cm diameter splenic mass surrounded by chylous effusion actively collecting during ultrasonographic assessment. Following splenectomy, histopathological analysis revealed that the splenic mass exhibited characteristics indicative of splenic angiosarcoma. This case report highlights the utility of advanced thoracic and abdominal imaging, notably CT-lymphangiography, in the diagnostic evaluation of chylothorax in cats. The identification of a splenic mass encased in chylous effusion should prompt a proactive case management strategy.
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Affiliation(s)
- Elodie Roels
- Department of Clinical Sciences, Fundamental and Applied Research for Animals & Health (FARAH), Faculty of Veterinary Medicine, University of Liege, Liege, Belgium
| | - Emilie Pierrot
- Department of Clinical Sciences, Fundamental and Applied Research for Animals & Health (FARAH), Faculty of Veterinary Medicine, University of Liege, Liege, Belgium
| | - Charles Porsmoguer
- Department of Clinical Sciences, Fundamental and Applied Research for Animals & Health (FARAH), Faculty of Veterinary Medicine, University of Liege, Liege, Belgium
| | - Annika Herrmann
- VPG Histopathology, Veterinary Pathology Group (VPG), Exeter, United Kingdom
| | - Aline Fastrès
- Department of Clinical Sciences, Fundamental and Applied Research for Animals & Health (FARAH), Faculty of Veterinary Medicine, University of Liege, Liege, Belgium
| | - Géraldine Bolen
- Department of Clinical Sciences, Fundamental and Applied Research for Animals & Health (FARAH), Faculty of Veterinary Medicine, University of Liege, Liege, Belgium
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Krähling H, Seidensticker M, Heindel WL, Gerwing M. Diagnostic approach to splenic lesions. ROFO-FORTSCHR RONTG 2024; 196:573-581. [PMID: 37967822 DOI: 10.1055/a-2193-2292] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2023]
Abstract
BACKGROUND Splenic lesions are rare and mostly incidental findings on cross-sectional imaging. Most lesions are of benign nature and can be correctly identified based on imaging characteristics. Further, invasive evaluation is only necessary in cases of splenic lesions with uncertain or potentially malignant etiology. METHOD While in most cases a correct diagnosis can be made from computed tomography (CT), (additional) magnetic resonance imaging (MRI) can aid in the identification of lesions. As these lesions are rare, only a few of the differential diagnoses are regularly diagnosed in the clinical routine. RESULT AND CONCLUSION This review presents the differential diagnoses of splenic lesions, including imaging characteristics and a flowchart to determine the right diagnosis. In conjunction with laboratory results and clinical symptoms, histological workup is necessary only in a few cases, especially in incidental findings. In these cases, image-guided biopsies should be preferred over splenectomy, if possible. KEY POINTS · Splenic lesions are rare and are usually incidental findings on abdominal imaging. · CT imaging and MRI imaging are the diagnostic tools of choice for the further workup of splenic lesions. · Based on their image morphological characteristics, a large number of splenic lesions can be assigned to one entity and do not need histological analysis. CITATION FORMAT · Krähling H, Seidensticker M, Heindel WL et al. Diagnostic approach to splenic lesions. Fortschr Röntgenstr 2024; 196: 573 - 581.
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Affiliation(s)
| | - Max Seidensticker
- Department of Radiology, Ludwig-Maximilians-Universität München, Germany
| | | | - Mirjam Gerwing
- Clinic of Radiology, University of Münster, Münster, Germany
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Shao M, Qi W, Xu R, Luo Z, Liao F, Fan S. Application value of 18F-FDG PET/CT in primary spleen angiosarcoma with liver metastasis: a case report and literature review. Front Oncol 2024; 14:1366560. [PMID: 38854730 PMCID: PMC11157051 DOI: 10.3389/fonc.2024.1366560] [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: 01/06/2024] [Accepted: 05/07/2024] [Indexed: 06/11/2024] Open
Abstract
Background Primary splenic angiosarcoma (PSA) is a rare neoplasm. It is a malignant tumor derived from endothelial cells of the splenic sinuses. PSA has an unknown etiology, a high degree of malignancy, easy early metastasis, atypical clinical symptoms and imaging findings, and difficult early diagnosis. This paper reports the 18F-FDG PET/CT findings of a case of PSA with intrahepatic metastasis; summarizes its clinical, imaging, and pathological data; and reviews the literature. Case description A 64-year-old male patient presented with left lower abdominal distending pain without obvious causes on 13 March 2022. The pain was persistent and dull and worsened after sitting and eating. Blood routine examination results were RBC ↓ 3.33 × 1012/L, WBC ↑ 12.32 × 109/L, and PLT ↓ 40 × 109/L. The tumor markers indicated CA125 ↑ 47.0 U/ml, AFP (-), CEA (-), CA199 (-), and CA724 (-). Non-contrast-enhanced CT scan of the abdomen showed that the spleen was significantly enlarged in volume and irregular in shape and had multiple nodules and clumpy low-density shadows, unclear boundaries, uneven density, and multiple necrotic areas. Enhanced CT showed diffuse uneven mild enhancement of the spleen, and the degree of enhancement increased with time. Multiple nodular low-density shadows were seen in the liver, which were slightly enhanced by the enhanced scan.18F-FDG PET/CT showed multiple nodular and massive lesions in the spleen with multiple necrotic areas. There were multiple nodular lesions in the liver, the level of FDG metabolism increased, the SUVmax of the spleen lesions was 9.0, and the SUVmax of the liver lesions was 5.6. The 18F-FDG PET/CT diagnosis was splenic malignancy with liver metastasis. Finally, after a multidisciplinary discussion, it was decided to perform laparoscopic total splenectomy and portal vein infusion chemotherapy. Pathological examination showed that the tumor cells were round, oval, or fusiform, with obvious atypia, arranged into a cable or anastomosed vascular lumen. The final diagnosis was primary splenic angiosarcoma with massive necrosis. After surgery, the patient received antitumor combined therapy and died 5 months later. Conclusion The incidence of PSA is very low, and its clinical and radiological manifestations lack specificity. 18F-FDG PET/CT imaging has a certain diagnostic value for PSA and significant utility in preoperative staging, guiding biopsy procedures, evaluating postoperative treatment response, and monitoring disease recurrence. PSA should be considered in the presence of a space-occupying lesion within the spleen that exhibits necrotic areas, shows progressive enhancement on contrast-enhanced scans, and demonstrates heterogeneous increases in FDG uptake.
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Affiliation(s)
- Mingyan Shao
- Department of Nuclear Medicine, Jiangxi Provincial People’s Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China
| | - Wanling Qi
- Department of Nuclear Medicine, Jiangxi Provincial People’s Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China
| | - Rong Xu
- Department of Nuclear Medicine, Jiangxi Provincial People’s Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China
| | - Zhehuang Luo
- Department of Nuclear Medicine, Jiangxi Provincial People’s Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China
| | - Fengxiang Liao
- Department of Nuclear Medicine, Jiangxi Provincial People’s Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China
| | - Sisi Fan
- Department of Pathology, Jiangxi Provincial People’s Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China
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9
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Jiang P, Li X, Chen J, Li D, Han Y. Spontaneous splenic rupture as the initial symptom of splenic angiosarcoma: case report and literature review. Front Oncol 2024; 14:1366554. [PMID: 38756665 PMCID: PMC11096474 DOI: 10.3389/fonc.2024.1366554] [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: 01/09/2024] [Accepted: 04/03/2024] [Indexed: 05/18/2024] Open
Abstract
Spontaneous splenic rupture is an extremely rare occurrence, often attributed to tumorous pathologies. Among these, primary splenic angiosarcoma stands as a malignancy arising from the endothelial cells within the spleen. While sporadic cases have been reported globally, there remains a lack of comprehensive consensus on standardized approaches for diagnosis and treatment. We report a case of an 83-year-old male who underwent emergency enhanced CT due to sudden shock, revealing significant intra-abdominal fluid accumulation. Emergency surgery revealed splenic rupture necessitating splenectomy. Histopathological examination confirmed the diagnosis of splenic angiosarcoma. Despite successful surgery, the patient succumbed to severe complications two weeks postoperatively.
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Affiliation(s)
- Peiwu Jiang
- Department of Vascular Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Xiaowen Li
- Department of General Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Jie Chen
- Department of General Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Du Li
- Department of Pathology, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Yehong Han
- Department of General Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
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Wu M, Li Z, Luo L, Zhao W, Luo J. Metastatic splenic angiosarcoma presenting with anemia and bone marrow fibrosis mimicking primary myelofibrosis: A case report and literature review. Mol Clin Oncol 2024; 20:16. [PMID: 38274087 PMCID: PMC10809309 DOI: 10.3892/mco.2024.2714] [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: 06/26/2023] [Accepted: 12/12/2023] [Indexed: 01/27/2024] Open
Abstract
Angiosarcomas, originating from endothelial cells, are infrequent soft tissue sarcomas characterized by a high propensity for metastasis and an unfavorable prognosis. Splenic angiosarcoma, an exceedingly rare and aggressive neoplasm, exhibits variable clinical manifestations. The present case report describes a patient initially exhibiting anemia and bone marrow fibrosis, mimicking primary myelofibrosis, ultimately diagnosed with splenic angiosarcoma. The findings of the present case report underscore the importance of considering splenectomy for histopathological confirmation. Employing a panel of vascular differentiation markers is invaluable for establishing the diagnosis of angiosarcoma.
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Affiliation(s)
- Meiqing Wu
- Department of Hematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
| | - Zhongqing Li
- Department of Hematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
| | - Lin Luo
- Department of Hematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
| | - Weihua Zhao
- Department of Hematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
| | - Jun Luo
- Department of Hematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
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11
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Suzuki T, Henshaw MJ, Yanagi T, Aoshima K. Current understanding of comparative pathology and prospective research approaches for canine hemangiosarcoma. Res Vet Sci 2024; 167:105120. [PMID: 38150941 DOI: 10.1016/j.rvsc.2023.105120] [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: 06/19/2023] [Revised: 12/11/2023] [Accepted: 12/18/2023] [Indexed: 12/29/2023]
Abstract
Hemangiosarcoma (HSA) is a malignant tumor originating from endothelial cells. HSA typically develops in dogs, but is rare in other animals, including humans. Although surgery and chemotherapy are conventional treatments for HSA, neither treatment can significantly improve patient prognosis. To develop novel and effective therapeutics, a deeper understanding of HSA pathogenesis must be acquired. However, the limited research tools for HSA have been unable to make a breakthrough; therefore, it is crucial to widely utilize or establish novel research tools such as patient-derived xenograft models, organoids, and chicken embryo xenograft models. The pathogenesis of the human counterpart of HSA, angiosarcoma (AS), also remains incompletely understood, preventing the extrapolation of findings from humans to dogs, unlike other diseases. In this review, we summarize the clinicopathological and morphological features of HSA, and then we discuss the current understanding of the molecular pathology of HSA. Finally, we highlight promising research tools that may accelerate HSA basic research toward developing novel therapeutics. We also briefly summarize AS to help researchers comprehend HSA from the perspective of comparative pathology.
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Affiliation(s)
- Tamami Suzuki
- Laboratory of Comparative Pathology, Department of Clinical Sciences, Faculty of Veterinary Medicine, Hokkaido University, Sapporo, Hokkaido 060-0818, Japan
| | - Michael James Henshaw
- English Education Section, Faculty of Veterinary Medicine, Hokkaido University, Sapporo, Hokkaido 060-0818, Japan
| | - Teruki Yanagi
- Department of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaido 060-8638, Japan
| | - Keisuke Aoshima
- Laboratory of Comparative Pathology, Department of Clinical Sciences, Faculty of Veterinary Medicine, Hokkaido University, Sapporo, Hokkaido 060-0818, Japan; Cancer Research Unit, One Health Research Center, Hokkaido University, Sapporo, Hokkaido 060-0818, Japan.
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12
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Soon GST, Chen ZE, Wu TT, Torbenson MS, Yasir S. Hepatic Angiosarcomas With Sinusoidal Growth Patterns. Am J Surg Pathol 2023; 47:1045-1051. [PMID: 37357916 DOI: 10.1097/pas.0000000000002082] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/27/2023]
Abstract
Hepatic angiosarcomas are aggressive malignant tumors of the liver with variable morphology. One of the rare morphologies is that of the sinusoidal growth pattern, which is challenging to diagnose because of its subtle imaging and morphologic findings. This retrospective study characterizes the clinical, histologic, and immunohistochemical features of sinusoidal hepatic angiosarcomas. Thirteen cases were included in the study, comprising 12 (92.3%) needle core biopsies and 1 wedge biopsy; one of the needle biopsies also had a subsequent resection specimen available for review. Multiple biopsies were needed to make the diagnosis in 4 cases. At least moderate sinusoidal dilatation was seen in 53.8% of cases. Increased cellularity within the sinusoids was seen at both low-power and high-power magnification (69.2% and 84.6%, respectively). Cytologic atypia ranged from mild to marked. Multinucleated tumor cells were present in most cases (10/13 cases) but were often sparse. Mitotic activity was identified in 5/13 cases. ERG immunostains were more reliable than CD31 and CD34 in identifying the tumor cells. Ki-67 proliferative index ranged from 5% to 30%. p53 immunostains were available in 9 cases and c-MYC in 7 cases; they were positive in 62.5% and 33.3% of cases, respectively and had a mutually exclusive staining pattern. In summary, this rare pattern of hepatic angiosarcoma is challenging to diagnose but has distinctive morphologic findings that can be supplemented with immunostains to establish the diagnosis.
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Affiliation(s)
- Gwyneth S T Soon
- Department of Pathology, National University Hospital, Singapore, Singapore
| | | | - Tsung-Teh Wu
- Division of Anatomic Pathology, Mayo Clinic, Rochester, MN
| | | | - Saba Yasir
- Division of Anatomic Pathology, Mayo Clinic, Rochester, MN
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13
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Slack JC, Putra J, Callahan MJ, Church AJ, Teot LA, Eng W, Perez-Atayde AR. Splenic Lymphatic Malformation With Papillary Endothelial Proliferation: A Rare Histologic Variant or a Unique Entity? Am J Surg Pathol 2023; 47:915-925. [PMID: 37334821 DOI: 10.1097/pas.0000000000002070] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/21/2023]
Abstract
Lymphatic malformations (LMs) are congenital anomalies of the lymphatic system due to abnormalities that occur during the development of the lymphovascular system. Also known as lymphangiomas, they are usually multifocal, affect multiple organ systems, and are seen in a variety of developmental or overgrowth syndromes. Splenic lymphangiomas are uncommon and usually occur in the context of multiorgan lymphangiomatosis. Within the spleen, 7 prior cases have been reported of LMs with unusual papillary endothelial proliferations (PEPs), which can mimic more aggressive splenic lymphovascular tumors. It is not currently known if splenic LM-PEP represents a unique entity, or is simply an unusual, site-specific, morphologic variant of LM. To address this question, we conducted a retrospective, single-institutional review of this rare entity and systematically evaluated its clinical, histologic, radiologic, electron microscopical, and molecular features. In all 3 splenic LM-PEPs, the clinical course was benign, imaging demonstrated subcapsular lesions with characteristic "spoke-and-wheel" appearance, histology showed distinctive PEPs within lymphatic microcysts, immunohistochemistry confirmed a lymphatic endothelial phenotype and electron microscopy demonstrated lesional endothelial cells, rich in mitochondria and intermediate filaments with prominent cytoplasmic lumina and vacuoles and lacking Weibel-Palade granules. Occasional lymphothelial cells were situated within the cytoplasm of another lesional cell, appearing to be engulfed. Next-generation sequencing identified a PIK3CA mutation in 1 patient, while in 2 others no molecular alterations were identified. We conclude with a summary of all prior published cases and discuss key diagnostic elements that distinguish this benign entity from its more aggressive mimickers.
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Affiliation(s)
| | | | | | | | | | - Whitney Eng
- Department of Pediatric Hematology/Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, MA
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14
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Deshpande A, Munoz J, Kelemen K, Dabak V, Hanbali A, Kurzrock R. Images in Immunotherapy and Precision Oncology: Angiosarcoma of the Spleen and Liver. JOURNAL OF IMMUNOTHERAPY AND PRECISION ONCOLOGY 2023; 6:56-58. [PMID: 36751660 PMCID: PMC9888520 DOI: 10.36401/jipo-22-22] [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: 08/01/2022] [Revised: 10/26/2022] [Accepted: 11/02/2022] [Indexed: 06/18/2023]
Abstract
Primary splenic or hepatic angiosarcomas are ultra-rare and aggressive malignancies associated with poor prognosis. The mainstay treatments are surgical resection and chemotherapy. We report a case of angiosarcoma in a 50-year-old woman who presented with bruising, fatigue, ecchymosis, and hepatosplenomegaly. She was treated with the multi-kinase inhibitor sunitinib for 4 weeks before developing a splenic hemorrhage and succumbing. Recent studies have demonstrated the clinical benefit of immunotherapies in angiosarcomas. Additionally, sequencing techniques have showcased the diverse molecular aberrations involved in angiosarcomas, which offer opportunities for precision-matched targeted therapies such as inhibitors of the VEGF/VEGFR axis and PI3K/Akt/mTor pathway.
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Affiliation(s)
| | - Javier Munoz
- Department of Hematology, Mayo Clinic Arizona, Phoenix, AZ, USA
| | - Katalin Kelemen
- Division of Hematopathology, Department of Laboratory Medicine and Pathology, Mayo Clinic Arizona, Phoenix, AZ, USA
| | - Vrushali Dabak
- Department of Hematology and Oncology, Henry Ford Health System, Detroit, MI, USA
| | - Amr Hanbali
- King Faisal Specialist Hospital, Riyadh, Saudi Arabia
| | - Razelle Kurzrock
- Medical College of Wisconsin, Milwaukee, WI, USA
- Worldwide Innovative Networking in Personalized Cancer Medicine (WIN) Consortium, Paris, France
- University of Nebraska, Omaha, NE, USA
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15
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Damouny M, Mansour S, Khuri S. Primary Angiosarcoma of the Spleen: An Aggressive Neoplasm. World J Oncol 2022; 13:337-342. [PMID: 36660212 PMCID: PMC9822683 DOI: 10.14740/wjon1542] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2022] [Accepted: 11/23/2022] [Indexed: 12/25/2022] Open
Abstract
Primary tumors of the spleen are rare, with an incidence rate of about 0.1%. These tumors could be benign, usually asymptomatic, or malignant which are usually symptomatic with abdominal pain being the most common symptom. Lymphoid neoplasms are the most common primary splenic tumors. Primary angiosarcoma is one of the extremely rare malignant vascular neoplasms of the spleen, which carries a dismal prognosis. It constitutes almost 7.4% of all primary malignant splenic neoplasms and is well known as an aggressive tumor with high local recurrence and distant metastasis rates. Overall survival is up to 12 months following diagnosis, regardless of management strategy. Due to the broad differential diagnosis of splenic tumors, this tumor is often forgotten, and is very challenging to diagnose early. Less than 300 cases of primary splenic angiosarcoma have been reported in the English literature. The main issue of this article is to review the current English literature to figure out the characteristic demographic features, clinical presentation, imaging findings and management of such tumors, in order to increase awareness of the treating physicians to improve diagnosis, management, as well as overall survival.
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Affiliation(s)
- Mira Damouny
- General Surgery Department, Rambam Medical Center, Haifa, Israel
| | - Subhi Mansour
- General Surgery Department, Rambam Medical Center, Haifa, Israel
| | - Safi Khuri
- General Surgery Department, Rambam Medical Center, Haifa, Israel
- HPB and Surgical Oncology Unit, Rambam Medical Center, Haifa, Israel
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16
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Luo H, Wang T, Xiao L, Wang C, Yi H. Multiple disciplinary team management of rare primary splenic malignancy: Two case reports. World J Clin Cases 2022; 10:10535-10542. [PMID: 36312480 PMCID: PMC9602245 DOI: 10.12998/wjcc.v10.i29.10535] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2021] [Revised: 11/27/2021] [Accepted: 09/01/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Malignant splenic tumors are rare but fatal, presenting a challenge in diagnosis and management involving hematology, oncology, and general surgery. By contrast, diagnosing and treating other common malignant tumors (such as lung and gastrointestinal cancer) offers multiple strategies for chemotherapy, radiotherapy, targeted therapy, and immunotherapy with the prospect of a cure. With various specialists involved in clinical multiple disciplinary team (MDT) discussion, personal bias can be minimized. It can also ignite important discussion which can benefit not only one patient but many patients.
CASE SUMMARY Here, we report on the MDT diagnosis and management of the malignant splenic tumors littoral cell angiosarcoma and histiocytic sarcoma. Although only two cases of rare primary splenic malignancy are presented, MDT is a novel means of rare disease treatment.
CONCLUSION To benefit patients, imaging analysis, safe operation, precise pathology examination, and individualized therapeutic treatment strategies are required. The involvement of various specialists in a clinical MDT discussion minimizes personal bias and can create important ideas to benefit all patients.
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Affiliation(s)
- Hao Luo
- Department of General Surgery Center, General Hospital of Western Theater, Chengdu 610083, Sichuan Province, China
| | - Tao Wang
- Department of General Surgery Center, General Hospital of Western Theater, Chengdu 610083, Sichuan Province, China
| | - Le Xiao
- Department of General Surgery Center, General Hospital of Western Theater, Chengdu 610083, Sichuan Province, China
| | - Chao Wang
- Department of Pathology, General Hospital of Western Theater, Chengdu 610083, Sichuan Province, China
| | - Hai Yi
- Department of Hematology, General Hospital of Western Theater, Chengdu 610083, Sichuan Province, China
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17
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Refractory splenic bleeding from splenic angiosarcoma: A case report and literature review. Int J Surg Case Rep 2022; 99:107708. [PMID: 36261945 PMCID: PMC9568872 DOI: 10.1016/j.ijscr.2022.107708] [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: 08/27/2022] [Revised: 09/10/2022] [Accepted: 09/24/2022] [Indexed: 11/26/2022] Open
Abstract
Introduction and importance Atraumatic splenic rupture (ASR) is a rare state that accounts for only <1 % of splenic ruptures. One of the causes of ASR is splenic neoplasm such as angiosarcoma. The treatment strategy for ASR is still unclear given the small number of cases reported in detail. Case presentation A 75-year-old woman presenting with abdominal pain with shock was referred to our hospital. Emergency computed tomography revealed splenic rupture, and hemodynamic stabilization was obtained by emergent vascular embolization. Rebleeding occurred 27 days after the initial treatment, and splenectomy was performed. Pathologically, ASR was diagnosed as caused by splenic angiosarcoma. Clinical discussion ASR is a very rare disease. The etiology of ASR has been reported as neoplastic, infectious, and so on. The treatment for ASR should be decided considering the etiology of ASR, hemodynamic stability, volume of blood transfusion, patient status, severity of the splenic injury, and volume of intraperitoneal bleeding. Conclusions We experienced a very rare case of ASR, in which diagnosis was challenging and the timing of surgery was difficult to determine. When splenic rupture has an atraumatic cause, splenectomy should be considered because of the possibility of malignancy.
The treatment strategy for traumatic splenic rupture is conservative therapy if possible, but that for atraumatic splenic ruptures has not been elucidated. In the case of conservative treatment for atraumatic splenic rupture, pathological examination cannot be performed, which may leave the patient untouched if the disease is malignant and at risk of rebleeding. Given the challenging diagnostics of angiosarcoma, splenectomy should be considered early in cases of atraumatic splenic rupture.
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18
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Chen D, Tang M, Lv S, Wang H, Du W, Zhao X, Lin L, Zhu Y, Wang G, Zhu H, Zhao K. Prognostic usefulness of clinical features and pretreatment 18F-FDG PET/CT metabolic parameters in patients with angiosarcoma. Quant Imaging Med Surg 2022; 12:2792-2804. [PMID: 35502366 PMCID: PMC9014154 DOI: 10.21037/qims-21-563] [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: 05/26/2021] [Accepted: 01/14/2022] [Indexed: 05/01/2024]
Abstract
BACKGROUND To investigate the prognostic value of clinical features and metabolic parameters in pretreatment 18F-2-fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography/X-ray computed tomography (PET/CT) scans of patients with angiosarcoma, a rare neoplasm that has not been well characterized. METHODS In this retrospective study, 19 patients with a histopathologically confirmed diagnosis of angiosarcoma who had undergone pretreatment 18F-FDG PET/CT scans were enrolled. We recorded the age at presentation, sex, underlying diseases, sites of primary tumors, Karnofsky Performance Status (KPS) score, Eastern Cooperative Oncology Group (ECOG) score, time from onset to diagnosis, laboratory examinations, sites and sizes of primary tumors, treatment modalities, histologic features and American Joint Committee on Cancer (AJCC) stage, maximum standardized uptake value (SUVmax), average SUV (SUVavg), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) of primary tumors and the whole body. Univariate and multivariate survival analyses for overall survival were performed according to the metabolic parameters and other clinical variables. RESULTS Patients ranged in age from 27 to 79 years (median: 59 years) with different angiosarcoma types covering all tumor grades and subtypes. Seven (7/19) patients had anemia of varying degrees of severity. Lymph node metastases (n=10) and/or distant metastases (n=11) of angiosarcoma were common. Bone or bone marrow (10/19) and lung (8/19) were the most common distant metastatic organs. Patients with bone metastases, low hemoglobin levels and high ferritin levels had significantly poorer overall survival than those with non-bone metastases, normal hemoglobin levels and normal ferritin levels by the log-rank test, with P values of 0.027, 0.030 and 0.015, respectively. Patients with multiple organ metastases had significantly poorer overall survival than those with single organ metastasis (log-rank P=0.008). In multivariate survival analysis, only whole-body metabolic tumor volume using SUVmax cut-off value of 2.5 (wMTV2.5) was a significant independent prognostic factor. For wMTV2.5, 870.3 cm3 was the best cut-off point to discriminate between a good and poor prognosis (log-rank P=0.01). CONCLUSIONS The systemic 18F-FDG PET/CT with high sensitivity and specificity has significant advantages in the evaluation of angiosarcoma, particularly in detecting occult metastases. Bone metastases on 18F-FDG PET/CT, low hemoglobin levels and high ferritin levels were all associated with a poorer prognosis. MTV2.5 of the whole body is a significant independent metabolic prognostic factor for overall survival in patients with angiosarcoma.
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Affiliation(s)
- Donghe Chen
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Mengmeng Tang
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Sha Lv
- State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, Hangzhou, China
| | - Huatao Wang
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Wendi Du
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Xin Zhao
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Lili Lin
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Yunqi Zhu
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Guangfa Wang
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Huanyan Zhu
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Kui Zhao
- PET Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
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19
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Kim N, Auerbach A, Manning MA. Algorithmic Approach to the Splenic Lesion Based on Radiologic-Pathologic Correlation. Radiographics 2022; 42:683-701. [PMID: 35302864 DOI: 10.1148/rg.210071] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Splenic lesions are commonly discovered incidentally at imaging, without clinical signs or symptoms that may aid in diagnosis. As such, the differential diagnosis and subsequent management are based primarily on imaging characteristics. Much has been written about the myriad pathologic conditions that can occur in the spleen; however, there is little guidance on the approach to an incidental splenic mass. Applying an approach frequently used in imaging to the splenic mass-based on the number and consistency of lesions and refined by supplementary imaging features-allows formulation of a useful differential diagnosis. Solitary cystic masses include true cysts, pseudocysts, and parasitic cysts. When multiple cystic lesions are present, the differential diagnosis expands to include infectious lesions (abscess or microabscesses) and lymphangioma (a benign cystic neoplasm). Hemangioma is the most common solitary solid mass, although other vascular lesions (hamartoma, sclerosing angiomatoid nodular transformation) and nonvascular lesions (inflammatory pseudotumor, lymphoma) manifest as solitary and solid. When multiple solid masses are present, diffuse inflammatory disease (sarcoidosis), littoral cell angioma, and lymphoma should be considered. Malignancies, such as angiosarcoma or metastasis, can manifest as solitary or multiple and solid or cystic masses but are typically associated with symptoms or widespread primary malignancy. Careful assessment of the multimodality imaging characteristics of splenic lesions based on this approach aids the radiologist faced with the incidental splenic lesion. Online supplemental material is available for this article. Work of the U.S. Government published under an exclusive license with the RSNA.
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Affiliation(s)
- Nancy Kim
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
| | - Aaron Auerbach
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
| | - Maria A Manning
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
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20
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Xu W, Wang K, Gu W, Nie X, Zhang H, Tang C, Lin L, Liang J. Case Report: Complete Remission With Anti-PD-1 and Anti-VEGF Combined Therapy of a Patient With Metastatic Primary Splenic Angiosarcoma. Front Oncol 2022; 12:809068. [PMID: 35311098 PMCID: PMC8928100 DOI: 10.3389/fonc.2022.809068] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2021] [Accepted: 02/01/2022] [Indexed: 01/04/2023] Open
Abstract
Primary splenic angiosarcoma (PSA) is a rare malignancy with poor prognosis. At present, little study is available on immunotherapy in PSA. Here, we report a case of a patient with metastatic PSA who was treated with programmed death-1 (PD-1) inhibitors and vascular endothelial growth factor (VEGF) tyrosine kinase inhibitors combined therapy and achieved complete response (CR). The patient was a 57−year−old woman with three liver metastases. She was treated with seven cycles of toripalimab plus anlotinib. Programmed death-ligand 1 (PD-L1) immunohistochemistry and next-generation sequencing was performed, and the PD-L1 tumor proportion score was 75%. Finally, she achieved CR after six cycles of the combined therapy regimen. No serious adverse events were detected. To the best of our knowledge, this is the first clinical evidence that anti-PD-1 plus anti-VEGF therapy might be a promising option for patients with metastatic PSA. However, more clinical trials are needed to verify this conclusion.
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Affiliation(s)
- Weiran Xu
- Department of Oncology, Peking University International Hospital, Beijing, China
| | - Kai Wang
- Department of Laboratory Medicine, Beijing Haidian Hospital, Beijing, China
| | - Wenguang Gu
- Department of Medicine, Geneplus-Beijing, Beijing, China
| | - Xinxin Nie
- Department of Medical Affairs, Shanghai Junshi Biosciences Co., Ltd., Beijing, China
| | - Hao Zhang
- Department of Medical Affairs, Shanghai Junshi Biosciences Co., Ltd., Beijing, China
| | - Chuanhao Tang
- Department of Oncology, Peking University International Hospital, Beijing, China
| | - Li Lin
- Department of Oncology, Peking University International Hospital, Beijing, China
| | - Jun Liang
- Department of Oncology, Peking University International Hospital, Beijing, China
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21
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Lei Y, Huang Q, Li X, Zheng X, Liu M. Characteristics and survival outcomes of primary splenic cancers: A SEER population-based study. Medicine (Baltimore) 2022; 101:e28539. [PMID: 35060511 PMCID: PMC8772628 DOI: 10.1097/md.0000000000028539] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/29/2021] [Accepted: 12/20/2021] [Indexed: 02/05/2023] Open
Abstract
Primary splenic cancers represent a small number of cancer cases and studies on its clinicopathological features and outcomes are limited. Splenic lymphomas and primary splenic angiosarcoma (PSA) are the 2 most common histological types of splenic cancers. This population-based study aimed to investigate the clinical characteristics and survival outcomes of patients with splenic lymphomas or PSA.Patients diagnosed with splenic lymphomas or PSA between 2000 and 2015 were identified from the Surveillance Epidemiology and End Results database of the National Cancer Institutes. Overall survival (OS) and cancer-specific survival (CSS) rates were calculated using the Kaplan-Meier method. A Cox proportional hazard models were used to identify independent predictors of cancer-specific mortality.A total of 700 patients with splenic lymphoma and 48 patients with PSA were included in this study. The median age of patients with splenic lymphoma was 65 years and 57 years for patients with PSA. For patients with splenic lymphoma, the most prevalent histological subtypes were splenic marginal zone lymphoma and diffuse large B-cell lymphoma. A total of 52.6% of the cases had stage IV disease based on the Ann Arbor staging system. Five-year OS and CSS were 76.9% and 83.4%, respectively. Multivariate analysis revealed that independent predictors of splenic lymphoma CSS included race, stage, chemotherapy, and histological subtype. However, a much shorter OS time was seen in the PSA cohort which had a 5-year OS of 11.8%, a median OS of 10.0 months and the 5-year CSS of 12.4%. Chemotherapy was correlated with better outcomes in patients with PSA. However, the survival benefits of surgery for splenic cancer were not statistically significant in our study.The current study is the largest cohort of primary splenic cancer presented in literature based on the Surveillance Epidemiology and End Results database and our large series describe the characteristics and survival outcomes of such rare diseases which may provide reliable information for further studies and clinicians.
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22
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Bhaludin BN, Thway K, Adejolu M, Renn A, Kelly-Morland C, Fisher C, Jones RL, Messiou C, Moskovic E. Imaging features of primary sites and metastatic patterns of angiosarcoma. Insights Imaging 2021; 12:189. [PMID: 34921641 PMCID: PMC8684573 DOI: 10.1186/s13244-021-01129-9] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2021] [Accepted: 11/14/2021] [Indexed: 12/14/2022] Open
Abstract
Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease. CT plays an important role in the evaluation of metastatic disease. Given the wide range of imaging findings, correlation with clinical findings, specific risk factors and patterns of metastatic disease can help narrow the differential diagnosis. The final diagnosis should be confirmed with histopathology and immunohistochemistry in combination with clinical and imaging findings in a multidisciplinary setting with specialist sarcoma expertise. The purpose of this review is to describe the clinical and imaging features of primary sites and metastatic patterns of angiosarcomas utilising CT and MRI.
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23
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Juin Hsien BL, Shelat VG. Spleen angiosarcoma: a world review. Expert Rev Gastroenterol Hepatol 2021; 15:1115-1141. [PMID: 34160346 DOI: 10.1080/17474124.2021.1945920] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2021] [Accepted: 06/17/2021] [Indexed: 02/08/2023]
Abstract
Introduction: Spleen angiosarcoma (SA) is a rare malignant neoplasm that arises from the splenic vascular endothelium, with only around 300 cases reported to date. Due to a limited number of reported cases, there is a paucity of data and a lack of understanding of its presentation, diagnosis, and management. In this study, we aim to provide a comprehensive review of SA.Areas covered: On 27 February 2021, a literature search was done in PubMed and Embase database. The search yielded 122 articles involving 205 patients. The focus was on patient demographics, risk factors, clinical presentations, investigation results, preliminary diagnoses, therapies provided, and patient outcomes. These factors were analyzed to identify possible risk factors, diagnostic modalities, and therapeutic principles that were not mentioned before.Expert opinion: The clinical presentation or investigation results of patients with SA are often nonspecific. Hence, they may not be sufficient to clinch the diagnosis of SA if used alone. The authors recommend a triple assessment of clinical examination, imaging findings, and pathology to diagnose SA with high accuracy. Splenectomy should be the mainstay of management, with chemotherapy and radiotherapy considered as adjuncts, especially in the presence of metastases.
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24
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Takehara M, Miyamoto H, Fujino Y, Tomonari T, Taniguchi T, Kitamura S, Okamoto K, Sogabe M, Sato Y, Muguruma N, Bando Y, Takayama T. Long-Term Survival due to Chemotherapy including Paclitaxel in a Patient with Metastatic Primary Splenic Angiosarcoma. Case Rep Gastroenterol 2021; 15:910-918. [PMID: 34720843 PMCID: PMC8543300 DOI: 10.1159/000519211] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2021] [Accepted: 08/20/2021] [Indexed: 11/19/2022] Open
Abstract
A primary splenic angiosarcoma is a rare type of soft tissue sarcoma and is associated with an extremely poor prognosis. In this study, we describe the case of a patient who was diagnosed with metastatic primary splenic angiosarcoma and survived for about 2 years. A 62-year-old female was referred to us for the treatment of splenic angiosarcoma with disseminated intravascular coagulation (DIC) and multiple liver and bone metastases. Paclitaxel therapy resulted in recovery from DIC and enabled her to continue sequential treatment through to sixth-line chemotherapy. We reviewed all splenic angiosarcoma case reports which were described as stage IV to date and compared with our case. From these data, we found that the median overall survival was 105 days, and the prognosis of splenic angiosarcoma of stage IV was worse than conventional case series. Splenectomy was performed in more patients than chemotherapy as a treatment. Moreover, various chemotherapeutic regimens were used. These data suggest that administering chemotherapy including paclitaxel to patients with splenic angiosarcoma might improve their prognosis.
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Affiliation(s)
- Masanori Takehara
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Hiroshi Miyamoto
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Yasuteru Fujino
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Tetsu Tomonari
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Tatsuya Taniguchi
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Shinji Kitamura
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Koichi Okamoto
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Masahiro Sogabe
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Yasushi Sato
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Naoki Muguruma
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Yoshimi Bando
- Department of Pathology, Tokushima University Hospital, Tokushima, Japan
| | - Tetsuji Takayama
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
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25
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Wheelwright M, Spartz EJ, Skubitz K, Yousaf H, Murugan P, Harmon JV. Primary angiosarcoma of the spleen, a rare indication for splenectomy: a case report. Int J Surg Case Rep 2021; 82:105929. [PMID: 33957408 PMCID: PMC8113851 DOI: 10.1016/j.ijscr.2021.105929] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2021] [Revised: 04/22/2021] [Accepted: 04/22/2021] [Indexed: 11/29/2022] Open
Abstract
INTRODUCTION AND IMPORTANCE Primary angiosarcoma of the spleen is a rare condition with a nonspecific clinical presentation and is associated with a poor prognosis. We describe two patients with primary splenic angiosarcoma successfully treated with splenectomy and adjuvant chemotherapy. CASE PRESENTATIONS Case 1: A 50-year-old female presented with fatigue and left-sided rib, shoulder, and abdominal pain. A CT scan demonstrated a large splenic mass, and biopsy was diagnostic of angiosarcoma. An open en bloc resection of the spleen was performed, and pathologic examination confirmed high-grade angiosarcoma; the surgical margins were negative. The patient received pegylated liposomal doxorubicin (PLD) and ifosfamide; she demonstrated no evidence of recurrence with four years of follow-up. Case 2: A 70-year-old male presented with acute back pain. A CT scan demonstrated a splenic mass; biopsy was diagnostic of angiosarcoma. The patient underwent open splenectomy, and pathology revealed high-grade angiosarcoma; the surgical margins were positive. The patient received PLD and ifosfamide but presented three years later with metastatic tumor to the spine. The patient had a favorable tumor response to pembrolizumab. The patient's tumor burden remains stable at 5 years following splenectomy. CLINICAL DISCUSSION Angiosarcoma of the spleen is a rare clinical entity and is often challenging to diagnose early. Moratality is high, especially in the case of metastasis or spontaneous rupture. CONCLUSION Due to the rare nature of this tumor, optimal treatment is not known. Here, we show excellent response in two patients to surgery combined with adjuvant therapy.
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Affiliation(s)
- Matthew Wheelwright
- Department of Surgery, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America.
| | - Ellen J Spartz
- University of Minnesota Medical School, Minneapolis, MN 55414, United States of America
| | - Keith Skubitz
- Department of Medicine, Division of Hematology, Oncology, and Transplantation, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America
| | - Hira Yousaf
- Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America
| | - Paari Murugan
- Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America
| | - James V Harmon
- Department of Surgery, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America
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26
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Sözel H, Yılmaz F. Primary Splenic Angiosarcoma with Liver Metastasis Caused by Malign Transformation of Hemangioma: A Case Report and Literature Review. J Gastrointest Cancer 2020; 52:1086-1089. [DOI: 10.1007/s12029-020-00550-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/05/2020] [Indexed: 10/23/2022]
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27
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Zhao S, Zhu L, Tong F, Tinzin L, Huang F, Zhou Y. Unexpected death due to spontaneous splenic rupture: A rare case in splenic angiosarcoma. Leg Med (Tokyo) 2020; 47:101785. [PMID: 32977197 DOI: 10.1016/j.legalmed.2020.101785] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2019] [Revised: 08/28/2020] [Accepted: 09/01/2020] [Indexed: 12/27/2022]
Abstract
Splenic angiosarcoma (SA) is a rare disease that can cause spontaneous splenic rupture leading to unexpected death. The rare incidence and non-specific clinical presentations made the early correct diagnosis of SA impossible in clinical practice. Even with medical intervention, 80% of patients died within 6 months after diagnosis. Here, we report a man who had complained of abdominal distension for 2 weeks and succumbed to the disease nine hours after admission. Diagnosis of hepatic and splenic angiosarcomas was based on post-mortem histopathological examination and immunohistochemistry, and the cause of death was hemorrhagic shock caused by spontaneous splenic rupture secondary to SA. This present case was the first forensic autopsy of spontaneous splenic rupture secondary to SA, which can highlight the diagnosis of rare diseases in forensic practice, and forensic pathologists should bear in mind these rare diseases even in routine practice.
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Affiliation(s)
- Shuquan Zhao
- Department of Forensic Medicine, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan 430030, PR China
| | - Longlong Zhu
- Department of Forensic Medicine, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan 430030, PR China
| | - Fang Tong
- Department of Forensic Medicine, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan 430030, PR China
| | - Lopsong Tinzin
- Department of Forensic Medicine, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan 430030, PR China
| | - Fang Huang
- Department of Forensic Medicine, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan 430030, PR China
| | - Yiwu Zhou
- Department of Forensic Medicine, Tongji Medical College, Huazhong University of Science and Technology, No. 13 Hangkong Road, Wuhan 430030, PR China.
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Farah BL, Madeline Chee YM, Ching SS, Tan CS. Primary splenic leiomyosarcoma as an exceptionally rare cause of ruptured splenomegaly – A case report and review of primary splenic sarcomas. HUMAN PATHOLOGY: CASE REPORTS 2020. [DOI: 10.1016/j.ehpc.2020.200452] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
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Bergas A, Escrihuela-Vidal F, Fernández Calvo D, Capdevila Pons O, Corbella X. Relapsing Haemothorax as an Unusual Presentation of Primary Angiosarcoma of the Spleen. Eur J Case Rep Intern Med 2020; 7:001944. [PMID: 33457353 DOI: 10.12890/2020_001944] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2020] [Accepted: 08/25/2020] [Indexed: 01/25/2023] Open
Abstract
Spontaneous haemothorax (SH) is a rare subcategory of haemothorax that involves the accumulation of blood within the pleural space in the absence of trauma. SH especially occurs in middle-aged or elderly patients, but data are usually limited to case reports and case series. Coagulopathy, aneurysm or aortic dissection, Rendu-Osler-Weber syndrome and malignancy have to be considered among the causes of SH. Aim We describe a case of primary angiosarcoma of the spleen presenting as relapsing haemothorax. Results An 81-year-old woman was referred to our hospital because of a 2-month history of relapsing haemothorax after the performance of urgent splenectomy due to active bleeding from large spleen cysts. No evidence of neoplasm was seen after pathological examination of the spleen. On admission, left haemothorax and 2 new cystic masses in the thoracic wall were documented, both in close relation to the scars of previous surgery and chest tubes. After excision of 1 mass, histological examination revealed angiosarcoma, and a final diagnosis of primary angiosarcoma of the spleen with postsurgical metastatic dissemination to the thorax and pleura was made. Conclusion Primary angiosarcoma should be included in the differential diagnosis of haemorrhagic spleen cysts. Clinical diagnosis and management usually requires splenectomy, but it should be carefully planned in order to avoid local metastatic dissemination or haematogenous spread of the tumour. LEARNING POINTS The differential diagnosis of spleen cysts must include malignant conditions.Despite its absolute rarity, angiosarcoma is considered the most common primary non-haematolymphoid splenic malignancy and has a poor prognosis.Splenectomy is usually required for a prompt diagnosis of primary angiosarcoma of the spleen, but it needs to be performed with extreme caution to avoid metastatic dissemination.
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Affiliation(s)
- Alba Bergas
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute-IDIBELL, L'Hospitalet de Llobregat, Spain
| | - Francesc Escrihuela-Vidal
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute-IDIBELL, L'Hospitalet de Llobregat, Spain
| | - Davinia Fernández Calvo
- Department of Pathology, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute-IDIBELL, L'Hospitalet de Llobregat, Spain
| | - Olga Capdevila Pons
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute-IDIBELL, L'Hospitalet de Llobregat, Spain
| | - Xavier Corbella
- Department of Internal Medicine, Hospital Universitari de Bellvitge, Bellvitge Biomedical Research Institute-IDIBELL, L'Hospitalet de Llobregat, Spain.,School of Medicine, Universitat Internacional de Catalunya, Barcelona, Spain
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Chatterjee S. Benign Lesions of the Spleen—Are They Really So Benign? A Report of Two Cases. Indian J Surg 2020. [DOI: 10.1007/s12262-020-02125-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
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31
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Rodríguez JMM, Morandi F, Cavicchio P, Poli A, Verin R. Morphological and Immunohistochemical Description of a Splenic Haemangioma in a Captive European Wolf ( Canis lupus lupus) and a Review of the Current Literature. Vet Sci 2020; 7:vetsci7030102. [PMID: 32756451 PMCID: PMC7558623 DOI: 10.3390/vetsci7030102] [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: 06/08/2020] [Revised: 07/17/2020] [Accepted: 07/22/2020] [Indexed: 12/19/2022] Open
Abstract
Neoplastic diseases are rarely described in wild carnivores; only a few reports have been published on this topic. Here, we describe the histological and immunohistochemical features of a haemangioma in the spleen of a grey wolf (Canis lupus lupus) and we compare the results with the dog (Canis lupus familiaris). Additionally, we list the different publications found in the literature with neoplastic lesions in wolves. Our results show similar immunohistochemical features to dogs, in which neoplastic cells express Vimentin, von Willebrand factor, alpha smooth muscle actin antibody, vascular endothelial growth factor C and low vascular endothelial growth factor receptor 3. Toluidine blue special stain shows moderated increased numbers of mast cells infiltrating the tumor, a feature observed in benign vascular tumors in domestic dogs, but not in the malignant counterparts. To our knowledge, this is the first article describing the gross, histological and immunohistochemical features of a splenic haemangioma in a wolf.
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Affiliation(s)
- Josep Maria Monné Rodríguez
- Institute of Veterinary Pathology, Vetsuisse Faculty, University of Zurich, Winterthurerstrasse 268, CH-8057 Zürich, Switzerland;
| | - Federico Morandi
- National Park “Monti Sibillini” P.zza del Forno, 1 62039 Visso, Italy;
| | - Paolo Cavicchio
- Pistoia Zoological Garden, Via Pieve a Celle Nuova, 160/A, 51100 Pistoia, Italy;
| | - Alessandro Poli
- Department of Veterinary Science, University of Pisa, 56124 Viale delle Piagge, 2 56124 Pisa, Italy
- Correspondence:
| | - Ranieri Verin
- Department of Comparative Biomedicine and Food Science—University of Padova AGRIPOLIS—Viale dell’ Università 16, 35020 Legnaro, Italy;
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Abstract
Primary splenic angiosarcoma is a rare type of cancer that has not been well-illustrated due to infrequency and variability in patient presentation. Limited systemic therapy regimens for splenic angiosarcoma make early detection preferable, as management focuses on monitoring for recurrence and metastatic spread or preventing hemorrhagic complications of tumor burden such as splenic rupture. This cancer, in particular, is aggressive, and metastasis is common. Here, we discuss a 68-year-old female who presented with a splenic laceration caused by an underlying primary splenic angiosarcoma. The purpose of this article is to describe a patient who presents with noteworthy clinical features and a rare complication of splenic angiosarcoma to aid in the treatment and diagnosis of future patients.
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Affiliation(s)
- Brooke E Kania
- Internal Medicine, United Hospital Center, Bridgeport, USA
| | - Sugam Vasani
- General Surgery, United Hospital Center, Bridgeport, USA
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33
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Sangiorgio VFI, Arber DA. Vascular neoplasms and non-neoplastic vascular lesions of the spleen. Semin Diagn Pathol 2020; 38:154-158. [PMID: 32674844 DOI: 10.1053/j.semdp.2020.07.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/14/2020] [Revised: 06/26/2020] [Accepted: 07/02/2020] [Indexed: 02/02/2023]
Abstract
Vascular neoplasms are among the most common conditions affecting the spleen. The majority of these are idiopathic, benign in nature and asymptomatic and therefore treated with a conservative management. Only rare cases cause splenomegaly and/or chronic consumption coagulopathies, thus requiring splenectomy. Among these, the most common is splenic hemangioma, followed by littoral cell angioma and lymphangioma. Peliosis is a peculiar tumor-like non-neoplastic vascular lesion that diffusely affects the spleen and frequently presents with concomitant hepatic involvement. As a distinctive feature, peliosis can occur as a secondary manifestation of infections, malignancies and in individuals using certain drugs. On the opposite spectrum of clinical behavior lies splenic angiosarcoma, a vascular endothelial malignancy with aggressive presentation and poor prognosis. In some cases the endothelial nature of this neoplasm may not be evident on routine histologic examination and immunohistochemistry is used to disclose such phenotype. The term hemangioendothelioma is rarely used to describe borderline vascular neoplasms which appear more aggressive than conventional hemangiomas, but that do not entirely fulfill the diagnostic criteria for angiosarcoma. Some of these neoplasms coexpress endothelial and histiocytic markers and therefore have been proposed as the borderline counterpart of littoral cell angioma. The existence of hemangioendothelioma as a diagnostic entity per se is debated and this diagnosis should be rendered with caution. The current review aims at highlighting the main histologic features of vascular neoplasms and non-neoplastic vascular lesions of the spleen.
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Affiliation(s)
| | - Daniel A Arber
- Department of Pathology, University of Chicago, Chicago, IL, USA.
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Dubova EA, Lishchuk SV, Pavlov KA, Katunina OR, Borbat AM, Miromanova EA. [Primary splenic angiosarcoma]. Arkh Patol 2019; 81:78-81. [PMID: 31851197 DOI: 10.17116/patol20198106178] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Primary splenic angiosarcoma is a rare and high-grade vascular tumor having a significant metastatic potential. The authors give the data available in the literature and describe their own case of primary splenic angiosarcoma in a 42-year-old male patient with multiple distant metastases in the lymph nodes and skin. The paper shows some difficulties in the morphologic diagnosis of this tumor.
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Affiliation(s)
- E A Dubova
- State Research Center A.I. Burnazyan Federal Medical Biophysical Center, Federal Biomedical Agency of Russia, Moscow, Russia
| | - S V Lishchuk
- State Research Center A.I. Burnazyan Federal Medical Biophysical Center, Federal Biomedical Agency of Russia, Moscow, Russia
| | - K A Pavlov
- State Research Center A.I. Burnazyan Federal Medical Biophysical Center, Federal Biomedical Agency of Russia, Moscow, Russia
| | - O R Katunina
- State Research Center A.I. Burnazyan Federal Medical Biophysical Center, Federal Biomedical Agency of Russia, Moscow, Russia
| | - A M Borbat
- State Research Center A.I. Burnazyan Federal Medical Biophysical Center, Federal Biomedical Agency of Russia, Moscow, Russia
| | - E A Miromanova
- State Research Center A.I. Burnazyan Federal Medical Biophysical Center, Federal Biomedical Agency of Russia, Moscow, Russia
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35
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Plantinga P, Rahman S, Rizkalla K, Shepherd JG, Phua CW. Splenic Angiosarcoma with Bone Marrow Involvement Initially Diagnosed as Systemic Mastocytosis: A Case Report. Cureus 2019; 11:e5804. [PMID: 31720194 PMCID: PMC6823090 DOI: 10.7759/cureus.5804] [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] [Indexed: 01/31/2023] Open
Abstract
We describe the case of a 67-year-old female patient presenting with constitutional symptoms and rapid decline. Two bone marrow core biopsies were performed, with spindled cells identified and thought to represent marrow involvement by systemic mastocytosis on the first biopsy. A diagnosis of metastatic vascular malignancy with sarcomatoid features was favored on the second core biopsy. The patient rapidly deteriorated and passed away. The post-mortem examination revealed a splenic angiosarcoma with metastasis to the liver and bone marrow. Splenic angiosarcoma is a rare, aggressive entity, with bone marrow metastasis even more uncommon. This report perceives this as a diagnostic consideration on bone marrow biopsies with spindled cells and explores the diagnostic dilemma and overlapping features of systemic mastocytosis and angiosarcoma.
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Affiliation(s)
- Paul Plantinga
- Pathology and Laboratory Medicine, Schulich School of Medicine & Dentistry, Western University, London, CAN
| | - Sadaf Rahman
- Medicine, Schulich School of Medicine & Dentistry, Western University, London, CAN
| | - Kamilia Rizkalla
- Pathology and Laboratory Medicine, London Health Sciences Centre, London, CAN
| | - Jessica G Shepherd
- Pathology and Laboratory Medicine, London Health Science Centre, London, CAN
| | - Chai W Phua
- Hematology, London Health Sciences Centre, London, CAN
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Fiorentino MD, Monteiro JMC, de Siqueira REB, Kim EIM, Curi AP, Ferrreira CR, Nardo M, de Campos FPF. Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum. AUTOPSY AND CASE REPORTS 2019; 9:e2019100. [PMID: 31372360 PMCID: PMC6629266 DOI: 10.4322/acr.2019.100] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2019] [Accepted: 06/06/2019] [Indexed: 12/16/2022] Open
Abstract
Primary splenic angiosarcoma (PSA) is a rare neoplasm of vascular origin associated with aggressive behavior and poor prognosis. The clinical presentation is usually non-specific and is mostly characterized by a wasting disease with anemia and splenomegaly, mimicking a wide range of entities. The authors present the case of an 80-year-old woman with cardiovascular comorbidities with a 6-month history of weight loss, fatigue, weakness, pallor, and abdominal pain. The physical examination showed massive splenomegaly and pallor. After a thorough evaluation that ruled out lymphoproliferative diseases, the working diagnosis was a myelodysplastic disorder. A few days after discharge, she returned to the emergency room with severe abdominal pain, worsening fatigue, and a remarkable pallor. Point-of-care ultrasound showed free intraperitoneal fluid. Spleen rupture was confirmed by abdominal computed tomography (CT) scan, and an emergency laparotomy with splenectomy was performed. The postoperative period was uneventful, and the patient recovered in a few days. The histopathology confirmed the diagnosis of PSA and the patient was referred to an oncological center. Two months later staging CT demonstrated liver and peritoneal metastases, and despite the chemotherapy she died 6 months after the diagnosis.
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Affiliation(s)
- Matheus Dalben Fiorentino
- Universidade de São Paulo (USP), School of Medicine, Internal Medicine Department. São Paulo, SP, Brazil
| | | | | | - Elizabeth Im Myung Kim
- Universidade de São Paulo (USP), Hospital Universitário, Internal Medicine Department. São Paulo, SP, Brazil
| | - Ana Paula Curi
- Universidade de São Paulo (USP), Hospital Universitário, Internal Medicine Department. São Paulo, SP, Brazil
| | - Cristiane Rubia Ferrreira
- Universidade de São Paulo (USP), Hospital Universitário, Internal Medicine Department. São Paulo, SP, Brazil.,Hospital AC Camargo, Unidade Antonio Prudente, Department of Pathology. São Paulo, SP, Brazil
| | - Mirella Nardo
- Instituto do Câncer do Estado de São Paulo, Oncology Department. São Paulo, SP, Brazil
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Immunohistochemical Analysis of Endothelial Cells in Vascular Transformation of Lymph Node Sinuses: Vascular or Lymphatic Differentiation? Appl Immunohistochem Mol Morphol 2019; 27:482-489. [DOI: 10.1097/pai.0000000000000661] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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38
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Cao L, Hong J, Wang Y, Yu J, Ma R, Li J, Wu J, Zheng S. A primary splenic angiosarcoma hepatic metastasis after splenectomy and its genomic alteration profile. Medicine (Baltimore) 2019; 98:e16245. [PMID: 31305404 PMCID: PMC6641855 DOI: 10.1097/md.0000000000016245] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2019] [Revised: 05/03/2019] [Accepted: 06/07/2019] [Indexed: 02/05/2023] Open
Abstract
RATIONALE Primary splenic angiosarcoma (PSA) is a rare mesenchymal malignancy of the splenic vascular origin often with a dismal prognosis. Genomic profile may provide evidence for the solution of therapy. PATIENT CONCERNS We reported a case of a 51-year-old woman with splenectomy 4 years ago and the postoperative histopathology diagnosis revealed "splenic hemangioma" with spontaneous rupture. Two years after the operation, the patient's rechecked abdominal computed tomography (CT) showed multiple hepatic occupations. DIAGNOSES Pathological test suggested PSA hepatic metastasis. INTERVENTIONS The patient was treated with trans-catheter arterial chemoembolization (TACE) and a pathological diagnosis of PSA was highly suspected in the hepatic biopsy. Four somatic alterations, phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA), Fos proto-oncogene, AP-1 transcription factor subunit (FOS), MCL1 apoptosis regulator (MCL1), and phosphoinositide-3-kinase regulatory subunit 1 (PIK3R1) were detected in the tumor tissue using a Next generation sequencing (NGS) technology. The results prompted that the patient may get clinical benefit from using some agents for targeted therapy, Everolimus, Temsirolimus, or Copanlisib. OUTCOMES The patient refused targeted therapy. As a result, the patient passed away within 51 months after splenectomy. LESSONS PSA is an aggressive disease that often presented with a high propensity for metastasis and rupture hemorrhage. Some of these mutations were first discovered in PSA and these findings added new contents to the genomic mutation profile of PSA.
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Affiliation(s)
- Linping Cao
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
| | - Jiawei Hong
- Key Lab of Combined Multi-Organ Transplantation, Ministry of Public Health
| | - Yacong Wang
- Department of Gerontology, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Jun Yu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
| | | | - Jia Li
- OrigiMed, Shanghai, China
| | - Jian Wu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
| | - Shusen Zheng
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
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39
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Ishii S, Omori S, Uesugi N, Tsuyukubo T, Ito A, Kikuchi D, Onoda M, Takata R, Sugai T, Obara W. A case of angiosarcomas which occurred in an adrenal gland and spleen synchronously. Int Cancer Conf J 2019; 7:134-136. [PMID: 31149532 PMCID: PMC6498291 DOI: 10.1007/s13691-018-0337-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2018] [Accepted: 07/04/2018] [Indexed: 11/05/2022] Open
Abstract
We report a case of a 56-year-old woman who simultaneously presented adrenal and spleen tumors. Computed tomography imaging revealed a 7-cm enhancing adrenal and 2-cm solitary spleen masses. The patient simultaneously underwent left adrenalectomy and splenectomy. The pathological findings revealed the presence of synchronous adrenal and spleen angiosarcomas. Remarkably, she is disease-free since postoperative 18 months.
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Affiliation(s)
- Shuhei Ishii
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - So Omori
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Noriyuki Uesugi
- 2Department of Molecular Diagnostic Pathology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Takashi Tsuyukubo
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Ayato Ito
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Daichi Kikuchi
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Mitsutaka Onoda
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Ryo Takata
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Tamotsu Sugai
- 2Department of Molecular Diagnostic Pathology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
| | - Wataru Obara
- 1Department of Urology, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, Iwate 020-8505 Japan
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Borch WR, Aguilera NS, Brissette MD, O'Malley DP, Auerbach A. Practical Applications in Immunohistochemistry: An Immunophenotypic Approach to the Spleen. Arch Pathol Lab Med 2019; 143:1093-1105. [PMID: 30917045 DOI: 10.5858/arpa.2018-0211-cp] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
CONTEXT.— Even though immunohistochemistry is routinely used by pathologists, evaluation of immunohistochemistry in splenic lesions remains difficult for many. Classification of benign and splenic lesions often requires a combination of hematoxylin-eosin evaluation, immunophenotyping, and sometimes molecular testing. Immunohistochemical staining is essential in evaluating many splenic lesions, and requires an understanding of the normal compartments of the spleen. OBJECTIVE.— To address different immunohistochemical features used for identification and subclassification of different lesions of the spleen, as well as in the normal compartments of the spleen. DATA SOURCES.— The information outlined in this review article is based on our experiences with a variety of spleen cases, on the current World Health Organization classification of hematopoietic and lymphoid tumors, and on a review of English-language articles published during 2018. CONCLUSIONS.— Features for phenotyping normal spleen as well as a variety of splenic lesions, including littoral cell angioma and splenic marginal zone lymphoma, are discussed. Suggested immunopanels are provided to assist in the diagnosis of different lesions of the spleen.
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Affiliation(s)
- William R Borch
- From the Department of Pathology, Walter Reed National Military Medical Center, Bethesda Maryland (Dr Borch); the Department of Hematopathology, University of Virginia, Charlottesville (Dr Aguilera); the Department of Hematopathology, Joint Pathology Center, Silver Spring, Maryland (Drs Brissette and Auerbach); and the Department of Hematopathology, NeoGenomics, Aliso Viejo, California (Dr O'Malley)
| | - Nadine S Aguilera
- From the Department of Pathology, Walter Reed National Military Medical Center, Bethesda Maryland (Dr Borch); the Department of Hematopathology, University of Virginia, Charlottesville (Dr Aguilera); the Department of Hematopathology, Joint Pathology Center, Silver Spring, Maryland (Drs Brissette and Auerbach); and the Department of Hematopathology, NeoGenomics, Aliso Viejo, California (Dr O'Malley)
| | - Mark D Brissette
- From the Department of Pathology, Walter Reed National Military Medical Center, Bethesda Maryland (Dr Borch); the Department of Hematopathology, University of Virginia, Charlottesville (Dr Aguilera); the Department of Hematopathology, Joint Pathology Center, Silver Spring, Maryland (Drs Brissette and Auerbach); and the Department of Hematopathology, NeoGenomics, Aliso Viejo, California (Dr O'Malley)
| | - Dennis P O'Malley
- From the Department of Pathology, Walter Reed National Military Medical Center, Bethesda Maryland (Dr Borch); the Department of Hematopathology, University of Virginia, Charlottesville (Dr Aguilera); the Department of Hematopathology, Joint Pathology Center, Silver Spring, Maryland (Drs Brissette and Auerbach); and the Department of Hematopathology, NeoGenomics, Aliso Viejo, California (Dr O'Malley)
| | - Aaron Auerbach
- From the Department of Pathology, Walter Reed National Military Medical Center, Bethesda Maryland (Dr Borch); the Department of Hematopathology, University of Virginia, Charlottesville (Dr Aguilera); the Department of Hematopathology, Joint Pathology Center, Silver Spring, Maryland (Drs Brissette and Auerbach); and the Department of Hematopathology, NeoGenomics, Aliso Viejo, California (Dr O'Malley)
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Selove W, Picarsic J, Swerdlow SH. Langerin staining identifies most littoral cell angiomas but not most other splenic angiomatous lesions. Hum Pathol 2018; 83:43-49. [PMID: 30130631 DOI: 10.1016/j.humpath.2018.08.012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/24/2018] [Accepted: 08/10/2018] [Indexed: 12/26/2022]
Abstract
Although littoral cell angiomas (LCAs) are phenotypically well characterized, the antibodies used to support the diagnosis identify many other cells in the normal spleen, and some may be found in other angiomatous lesions. Based on a langerin/CD207+ LCA index case, langerin and other selected immunohistochemical staining was performed on 10 LCAs, 20 other splenic angiomatous lesions, and 7 reactive lymph nodes to further investigate the role of langerin as a diagnostic tool. Ninety percent (9/10) of LCAs were langerin positive, whereas only 1 (5%) of 20 other splenic vascular lesions was partially positive (P < .00001). All LCAs were CD1a-, CD68+, CD34-, and CD8-; 20% were S100+, 70% CD21+, and 90% cyclin D1+. Ultrastructural studies of one LCA did not show Birbeck-type granules in definite lining cells. Sinus lining cells in 7 of 7 reactive lymph nodes showed partial langerin positivity, and 4 of 4 showed partial cyclin D1 positivity. In conclusion, langerin staining is an easily interpreted and highly sensitive and specific (sensitivity [0.90], specificity [0.95]) ancillary study to help distinguish LCA from other vascular tumors of the spleen. Whether this represents cross-reactivity or true CD207 expression is uncertain, as other immunohistochemical and ultrastructural studies do not support a Langerhans cell origin. The cyclin D1 staining seen in most LCA would be consistent with their expression of other selected vascular and histiocytic markers. The similar staining pattern in some lymph node sinus lining cells suggests a possible similar cell of origin, although LCA of lymph nodes is not described.
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Affiliation(s)
- William Selove
- Division of Hematopathology, UPMC Presbyterian, Pittsburgh, PA 15213, USA.
| | - Jennifer Picarsic
- Department of Pathology, University of Pittsburgh School of Medicine, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA 15224, USA.
| | - Steven H Swerdlow
- University of Pittsburgh School of Medicine, UPMC Presbyterian, Pittsburgh, PA 15213, USA.
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42
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Adjuvant Radiochemotherapy with a 23-Month Overall Survival Time in a Patient after a Surgery due to Splenic Hemangiosarcoma Rupture: A Case Report with the Literature Review. Case Rep Oncol Med 2018; 2018:8672407. [PMID: 29607237 PMCID: PMC5828302 DOI: 10.1155/2018/8672407] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2017] [Revised: 11/28/2017] [Accepted: 01/18/2018] [Indexed: 11/17/2022] Open
Abstract
Spleen sarcoma is one of the most rare soft tissue malignancies. The annual incidence is 0.14–0.25/1,000,000 and the average age of diagnosis is 50 to 73 years. The incidence of this cancer has been increasing. Treatment of choice is surgical splenectomy, which rarely gives good results due to the aggressive course of the disease as well as the high potential for metastasis. Overall survival in primary spleen sarcomas as described by various authors is between 4 and 14 months. 80% of patients after spleen rupture do not survive 6 months. We report the case of a 42-year-old male diagnosed with spleen angiosarcoma. The patient underwent surgery in an emergency mode because of rapid rupture of the organ. Due to positive surgical margins, he underwent adjuvant radiochemotherapy followed by chemotherapy. Overall survival time was relatively long (23 months). The international guidelines provide information based on limited data. The role of postoperative radiotherapy in angiosarcomas remains controversial. Postoperative radiotherapy may increase local disease control, especially after nonradical operation, but this does not translate into improvement in overall survival time of these patients. The case shows that adjuvant radiotherapy as part of cancer treatment strategy may prolong the overall survival.
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Primary Spleen Angiosarcoma With Concomitant Hepatic Hemangiomas on 18F-FDG PET/CT. Clin Nucl Med 2018; 43:222-223. [DOI: 10.1097/rlu.0000000000001982] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
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Chen X, Li H, Wang F, Liu H. Early detection and integral resection are keys to extend survival in patients suffered from primary angiosarcoma of the spleen: A care-compliant case report and literature review. Medicine (Baltimore) 2018; 97:e9718. [PMID: 29384853 PMCID: PMC5805425 DOI: 10.1097/md.0000000000009718] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
RATIONALE Primary angiosarcoma of the spleen (PAS) is a very rare malignant neoplasm that originates from endothelial cells of the splenic blood vessels. Without typical clinical presentations and specific radiological features, PAS is very difficult to be early identified and 1-year mortality is extremely high. Late detection and spleen rupture are considered as the most important risk factors for early metastasis. PATIENT CONCERNS Without any obvious symptom, a 35-year-old woman was admitted with splenic neoplasm that was accidentally discovered through a routine physical examination. DIAGNOSES The patient was first diagnosed as lymphoma by laboratory tests and imaging studies, but changed to PAS by histological examinations after the surgery. INTERVENTIONS After careful preoperational assessment, a laparoscopic-assisted splenectomy was scrutinously performed and the entire spleen was removed without any rupture. OUTCOMES The postoperative followed-up was uneventful until 3 years later, when she sought medical attention due to persisting back pain. Bone metastasis was consequently identified and the symptom was quickly alleviated after radiation therapy. However, intra-abdominal metastases leading to intestinal obstruction occurred 4.5 years after surgery. Following short palliative treatment, the patient passed away 4 years and 9 months after the operation due to multiple organ failure. LESSONS PAS is an uncommon and aggressive splenic disease. Once suspected, PAS require prompt and precise surgical procedures to remove the tumor origin. Laparoscopic-assisted splenectomy was technically feasible and therapeutically harmless for PAS treatment compared with open surgery as long as the spleen was removed intact. However, more evaluation of this option will be needed due to limited experience by now. Early discovery, precautious plan, meticulous operation, close follow-up, and comprehensive treatment may significantly prolong the living period of this fatal disease.
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Özcan B, Çevener M, Kargı AO, Dikici H, Yıldız A, Özdoğan M, Gürkan A. Primary splenic angiosarcoma diagnosed after splenectomy for spontaneous rupture. Turk J Surg 2018; 34:68-70. [PMID: 29756113 DOI: 10.5152/turkjsurg.2017.3207] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2015] [Accepted: 07/19/2015] [Indexed: 11/22/2022]
Abstract
Primary splenic angiosarcoma is a rare abnormality and has a bad prognosis. It has unknown pathogenesis. This abnormality is usually presented by splenic rupture. Surgery is the most promising treatm Surgery is the most accepted and accurate method for diagnosis and treatment. Surgery before rupture increases the life expectancy. A 65-year-old woman who presented to the emergency room with abdominal pain, abdominal distension, and anemia was found to have a splenic mass and massive ascites. After getting a hemorrhagic sample from the abdomen, the patient was operated with splenic rupture prediagnosis. The spleen material was reported as splenic angiosarcoma. The staging 18F-FDG-Positron Emission Tomography-Computed Tomography did not show any metastasis. Five months later, paclitaxel treatment was initiated upon liver and bone metastasis, and the treatment still continues. Splenic angiosarcoma has a place among splenic parenchymal lesions. The splenectomy material names the diagnosis. Pathologic examination of splenectomy material is revealed certain diagnosis.
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Affiliation(s)
- Barış Özcan
- Department of General Surgery, Medstar Antalya Hospital, Antalya, Turkey
| | - Metin Çevener
- Department of Radiology, Medstar Antalya Hospital, Antalya, Turkey
| | | | - Hilmi Dikici
- Department of Gastroenterology, Medstar Antalya Hospital, Antalya, Turkey
| | - Akın Yıldız
- Department of Nuclear Medicine, Medstar Antalya Hospital, Antalya, Turkey
| | - Mustafa Özdoğan
- Department of Medical Oncology, Medstar Antalya Hospital, Antalya, Turkey
| | - Alihan Gürkan
- Department of General Surgery, Memorial Antalya Hospital, Antalya, Turkey
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Sharma S, Singh P, Gupta P, Lal A, Srinivasan R. Primary Splenic Angiosarcoma with Liver Metastasis: A Rare Neoplasm Diagnosed on Fine-needle Aspiration Cytology and Cell Block Immunocytochemistry. J Cytol 2018; 35:114-116. [PMID: 29643660 PMCID: PMC5885599 DOI: 10.4103/joc.joc_148_16] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
Primary splenic angiosarcoma is a rare malignant vascular neoplasm of mesenchymal origin. The tumor is highly aggressive and has a high metastatic potential. It is usually diagnosed on histopathological examination of splenectomy specimen. Only few cases of angiosarcoma diagnosed by fine-needle aspiration (FNA) cytology alone have been reported in the literature. The cytologic features of angiosarcoma are heterogeneous, however, diagnosis can be suggested by FNA when vasoformative features are present. A 55-year-old female presented with abdominal pain and hepatosplenomegaly. Computed tomography scan revealed a heterogeneous splenic lesion with liver metastases. FNA from the splenic and liver lesions showed moderately pleomorphic tumor cells closely associated with anastomosing vascular channels. Cell block immunocytochemistry (ICC) showed tumor cells positive for CD31, CD34, CD68 as well as for CD99. FNA supplemented by cell block ICC can render a definite diagnosis of primary splenic angiosarcoma with liver metastasis.
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Affiliation(s)
- Saniya Sharma
- Department of Cytology and Gynaecological Pathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Priya Singh
- Department of Cytology and Gynaecological Pathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Pankaj Gupta
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Anupam Lal
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Radhika Srinivasan
- Department of Cytology and Gynaecological Pathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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Gao L, Xu W, Li T, Luo H, Gai S, Xing R, Chen G, Chen Y. A rare case of angiosarcoma with skull masses and erythropenia and thrombocytopenia: A case report and review of literature. Medicine (Baltimore) 2017; 96:e8787. [PMID: 29245237 PMCID: PMC5728852 DOI: 10.1097/md.0000000000008787] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022] Open
Abstract
RATIONALE Primary splenic angiosarcoma (PSA) is a rare, fatal neoplasm originating from sinusoidal vascular endothelial cells, and usually metastasizes and almost always has a poor prognosis. Surgical excision is the main treatment of this highly malignant disease. PATIENT CONCERNS We reported a special case of a 68-year-old female who had a 6-month history of scalp masses. DIAGNOSIS The patient was found to have 2 skull masses on computed tomography (CT). Laboratory findings revealed erythropenia and thrombocytopenia. Enhanced abdomen magnetic resonance imaging (MRI) showed multiple masses in liver and spleen. The pathological result of the skull masses was revealed to be metastatic angiosarcoma. INTERVENTIONS The patient underwent surgical excision of skull masses, and no subsequent radiotherapy or chemotherapy was done. OUTCOMES The patient died due to dyscrasia at August 12, 2015, with a survival of nearly 1 month. LESSONS We highlight the importance for clinicians to be aware of this rare neoplasm, and to consider it in the differential diagnosis when encountering a skull mass. Early confirmation and treatment may improve the prognosis.
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Affiliation(s)
- Liansheng Gao
- Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou
- Department of Neurosurgery, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu
| | - Weilin Xu
- Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou
| | - Tao Li
- Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou
| | - Huali Luo
- Department of Pathology, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, Zhejiang, China
| | - Shiying Gai
- Department of Neurosurgery, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu
| | - Ruxin Xing
- Department of Neurosurgery, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu
| | - Gao Chen
- Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou
| | - Yili Chen
- Department of Neurosurgery, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu
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Spontaneous splenic artery rupture in a patient with an unclassified malignant spindle cell tumor of the spleen: report of a case. INTERNATIONAL JOURNAL OF SURGERY-ONCOLOGY 2017; 2:e14. [PMID: 29177212 PMCID: PMC5673126 DOI: 10.1097/ij9.0000000000000014] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/28/2016] [Accepted: 02/14/2017] [Indexed: 11/25/2022]
Abstract
The purpose of this paper was to describe an unusual manifestation of a sarcoma of the spleen and to raise awareness for spontaneous rupture of the splenic vessels in patients with splenic tumors. A 70-year-old man was admitted to our institution, suffering from left upper quadrant abdominal pain. Upon physical examination, a large and tender abdominal mass was palpated. Abdominal computed tomography showed a heterogenous enlarged spleen with active contrast extravasation from the splenic artery, free intraperitoneal fluid, and a retroperitoneal hematoma. The patient was treated with angioembolization of the splenic artery. Because of rebleeding, splenectomy was performed. Pathology revealed the spleen to be involved with an unclassified malignant spindle cell neoplasm. We concluded that in the case of spontaneous rupture of the splenic artery, accompanied with a radiologic appearance of an enlarged spleen, the diagnosis of sarcoma should be included in the differential diagnosis.
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FDG PET/CT in Primary Splenic Angiosarcoma With Diffuse Involvement of the Spleen. Clin Nucl Med 2017; 42:815-817. [DOI: 10.1097/rlu.0000000000001805] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
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Leon-Castillo A, Chrisinger JSA, Panse G, Samdani RT, Ingram DR, Ravi V, Prieto VG, Wang WL, Lazar AJ. Index report of cutaneous angiosarcomas with strong positivity for tyrosinase mimicking melanoma with further evaluation of melanocytic markers in a large angiosarcoma series. J Cutan Pathol 2017; 44:692-697. [DOI: 10.1111/cup.12968] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2017] [Revised: 05/21/2017] [Accepted: 05/22/2017] [Indexed: 01/20/2023]
Affiliation(s)
- Alicia Leon-Castillo
- Pathology Service; Hospital Universitario Marques de Valdecilla; Santander Spain
| | - John S. A. Chrisinger
- Department of Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
| | - Gauri Panse
- Department of Pathology; Yale University; New Haven Connecticut
| | - Rashmi T. Samdani
- Department of Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
| | - Davis R. Ingram
- Department of Translational Molecular Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
| | - Vinod Ravi
- Department of Sarcoma Medical Oncology; The University of Texas MD Anderson Cancer Center; Houston Texas
| | - Victor G. Prieto
- Department of Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
- Department of Dermatology; The University of Texas MD Anderson Cancer Center; Houston Texas
| | - Wei-Lien Wang
- Department of Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
- Department of Translational Molecular Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
| | - Alexander J. Lazar
- Department of Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
- Department of Translational Molecular Pathology; The University of Texas MD Anderson Cancer Center; Houston Texas
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