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Grewal K, Gajagowni S, Lopez E, Grewal K, Nguyen SV. Epithelioid Hemangioendothelioma (EHE) Mimicking Mesothelioma: Case Presentation and Review. Case Rep Oncol Med 2025; 2025:9459565. [PMID: 39968515 PMCID: PMC11835476 DOI: 10.1155/crom/9459565] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2024] [Accepted: 01/11/2025] [Indexed: 02/20/2025] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular sarcoma that can initially present with a myriad of symptoms anywhere in the body. Imaging findings are often nonspecific, and the pathology tends to have overlap with other malignancies. As a result, it can be quite difficult to suspect and diagnose EHE. We present a case of pulmonary EHE in a 41-year-old female with left pleural thickening and subclavian tumor thrombus who was initially misdiagnosed and treated as mesothelioma. This instance demonstrates the importance of maintaining a broad differential and the utilization of repeat biopsies and next-generation sequencing for questionable diagnoses of atypical malignancies.
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Affiliation(s)
- Kabir Grewal
- Department of Internal Medicine, Baylor College of Medicine, Houston, Texas, USA
| | - Saivaroon Gajagowni
- Department of Internal Medicine, Baylor College of Medicine, Houston, Texas, USA
| | - Elyse Lopez
- Department of Cancer Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
| | - Kayanaat Grewal
- Department of Medicine, Florida State University College of Medicine, Tallahassee, Florida, USA
| | - Son Viet Nguyen
- Department of Hospital Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
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Pateras IS, Kostopanagiotou K, Samaras MG, Koumarianou A, Koutrouli M, Korodimos N, Kampoli K, Apollonatou V, Manali E, Loukides S, Tomos P, Tsiodras S, Panayiotides IG. Rare cancer with primary pleural epithelioid hemangioendothelioma diagnosed by thoracoscopic biopsy achieving disease control after 16 months: case report and literature review. Front Pharmacol 2024; 15:1482154. [PMID: 39650162 PMCID: PMC11621942 DOI: 10.3389/fphar.2024.1482154] [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: 08/17/2024] [Accepted: 10/21/2024] [Indexed: 12/11/2024] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor whose pleural EHE (pEHE) type is even more uncommon, with significant heterogeneity in the clinical behavior as well as challenging diagnosis and treatment decisions. Herein, we present a 74-year-old woman admitted to the hospital with dyspnea, pleural effusion, and refractory euvolemic hyponatremia. Chest computed tomography revealed a large right-side pleural effusion with irregular thickening of the parietal pleura and localized nodularity. Histologic evaluation of the thoracoscopic pleural biopsy tissue was used to confirm the diagnosis of pEHE. Assessment of the volume status suggested that the hyponatremia was attributable to a paraneoplastic endocrine syndrome. Administration of the multitarget tyrosine kinase inhibitor pazopanib helped achieve disease control, with the patient remaining free of symptoms after 16 months of follow-up. This case report adds to the knowledge base of this exceptionally rare condition, highlighting the need for a multidisciplinary approach.
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Affiliation(s)
- Ioannis S. Pateras
- Second Department of Pathology, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
| | - Konstantinos Kostopanagiotou
- Deparmtent of Thoracic Surgery, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Menelaos G. Samaras
- Second Department of Pathology, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
| | - Anna Koumarianou
- Hematology Oncology Unit, Fourth Department of Internal Medicine, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Mina Koutrouli
- Deparmtent of Thoracic Surgery, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Nikolaos Korodimos
- Deparmtent of Thoracic Surgery, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Katerina Kampoli
- Hematology Oncology Unit, Fourth Department of Internal Medicine, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Vasiliki Apollonatou
- Second Respiratory Medicine Department, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
| | - Effrosyni Manali
- Second Respiratory Medicine Department, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
| | - Stylianos Loukides
- Second Respiratory Medicine Department, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
| | - Periklis Tomos
- Deparmtent of Thoracic Surgery, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Sotirios Tsiodras
- Fourth Department of Internal Medicine, Attikon University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Ioannis G. Panayiotides
- Second Department of Pathology, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
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Weissferdt A, Moran CA. Primary epithelioid hemangioendotheliomas and angiosarcomas of the pleura: a clinicopathological and immunohistochemical analysis of 13 cases. Hum Pathol 2023; 138:68-75. [PMID: 37364825 DOI: 10.1016/j.humpath.2023.06.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2023] [Revised: 06/13/2023] [Accepted: 06/21/2023] [Indexed: 06/28/2023]
Abstract
Thirteen cases of primary epithelioid hemangioendotheliomas (EHE) and epithelioid angiosarcomas (EA) of the pleura are presented. The patients were 7 men and 6 women between the ages of 34 and 65 years (mean: 47 years). The patients presented with non-specific symptoms of cough, dyspnea, and chest pain. Diagnostic imaging revealed the presence of either diffuse pleural thickening or pleural nodules involving the serosal surfaces. Open surgical biopsies were obtained in all cases. Histologically, eight tumors were characterized by the presence of a cellular proliferation composed of medium-sized epithelioid cells embedded in a myxohyaline stroma and a variable spindle cell component. Cellular atypia was mild to moderate and mitotic activity ranged from 1 to 2 per 2 mm2. Immunohistochemical stains for vascular markers, including CAMTA1 were positive, confirming a diagnosis of EHE. Five cases of epithelioid angiosarcomas were characterized by a neoplastic cellular proliferation admixed with areas of necrosis and hemorrhage and characterized by medium-sized epithelioid to spindle-shaped cells with eosinophilic cytoplasm, round to oval nuclei and prominent nucleoli. In addition, marked cytologic atypia and a mitotic activity ranging from 3 to 5 per 2 mm2 were identified. Immunohistochemical studies demonstrated positive staining for vascular markers; however, CAMTA1 was negative. Clinical follow-up obtained in eleven cases showed that all patients had died within 30 months post diagnosis. The current study highlights that even though it may be important to histologically separate EHE from EA for academic purposes, primary pleural origin of these tumors appears to portent an aggressive clinical behavior.
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Affiliation(s)
- Annikka Weissferdt
- Department of Pathology, University of Texas, MD Anderson Cancer Center, Houston, TX 77030, USA.
| | - Cesar A Moran
- Department of Pathology, University of Texas, MD Anderson Cancer Center, Houston, TX 77030, USA
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Liu T, Pan W, Lai S, Luo J. Can angiogenesis inhibitor therapy cause changes in imaging features of hepatic hemangioma- Initial study. Front Oncol 2023; 13:1134179. [PMID: 36969035 PMCID: PMC10036792 DOI: 10.3389/fonc.2023.1134179] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2022] [Accepted: 02/27/2023] [Indexed: 03/12/2023] Open
Abstract
BackgroundTo observe whether anti-angiogenesis therapy can induce changes in size and enhancement characteristics of hepatic hemangioma.Method133 patients with hepatic hemangioma lesions were analyzed and classified into a Bevacizumab group (n=65) and the control group (n=68). The parameters (Volume, CT enhancement ratio, enhancement patterns) of pre-and post-treatment in the bevacizumab and control groups independently calculated and compared by two radiologists. Correlation among the systolic blood pressure, diastolic blood pressure, heart rate with the hemangioma volume was evaluated using Pearson’s correlation analysis.ResultsThe hepatic hemangioma volume was significantly decreased after treatment in the Bevacizumab group (8.6 ± 18.7mL vs.7.3 ± 16.3mL, P<0.05), and there was no significant change in the control group (15.1 ± 19.8mL vs.15.4 ± 20.7mL, P = 0.504). A significant difference in enhancement patterns of hepatic hemangiomas was observed after treatment with Bevacizumab (P<0.01). There was no significant difference in arterial phase (AP)enhancement rate and arterial phase-portal venous phase (AP-PVP) enhancement ratios after treatment in the Bevacizumab and control groups (Ps>0.05).The Pearson correlation results showed that blood pressure, heart rate, and hemangioma volume were unrelated or weakly related before and after bevacizumab treatment under the control of factors including weight, contrast injection scheme and CT scanning scheme.ConclusionsAnti-angiogenesis therapy can cause changes in enhancement pattern and volume of hepatic hemangioma. Radiologists should pay more attention to the reexamination of tumor patients treated with anti-angiogenesis therapy.
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Rezvani A, Shahriarirad R, Erfani A, Ranjbar K. Primary malignant epithelioid hemangioendothelioma of the pleura: A review and report of a novel case. Clin Case Rep 2022; 10:e6211. [PMID: 35979384 PMCID: PMC9366930 DOI: 10.1002/ccr3.6211] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2022] [Revised: 06/27/2022] [Accepted: 07/23/2022] [Indexed: 11/06/2022] Open
Abstract
Epithelioid hemangioendothelioma is considered an uncommon tumor originating from vascular tissues. Although this disease is an extremely rare malignant cancer, its pleural subtype is even less common. We discuss a 68-year-old man with isolated pleural epithelioid hemangioendothelioma, along with a literature review of all similar cases.
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Affiliation(s)
- Alireza Rezvani
- Bone Marrow Transplantation Center, Nemazi HospitalShiraz University of Medical SciencesShirazIran
- Thoracic and Vascular Surgery Research CenterShiraz University of Medical ScienceShirazIran
| | - Reza Shahriarirad
- Thoracic and Vascular Surgery Research CenterShiraz University of Medical ScienceShirazIran
- Student Research CommitteeShiraz University of Medical SciencesShirazIran
| | - Amirhossein Erfani
- Thoracic and Vascular Surgery Research CenterShiraz University of Medical ScienceShirazIran
- Student Research CommitteeShiraz University of Medical SciencesShirazIran
| | - Keivan Ranjbar
- Thoracic and Vascular Surgery Research CenterShiraz University of Medical ScienceShirazIran
- Student Research CommitteeShiraz University of Medical SciencesShirazIran
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Muthu S, Kandasamy V, Chellamuthu G, Thakur A. Custom Mega Prosthesis in a Rare case of Epithelioid Hemangioendothelioma of Proximal Tibia - A Case Report. J Orthop Case Rep 2022; 12:5-9. [PMID: 36659885 PMCID: PMC9826681 DOI: 10.13107/jocr.2022.v12.i07.2892] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2022] [Revised: 04/13/2022] [Indexed: 02/08/2023] Open
Abstract
INTRODUCTION Lytic lesions of the proximal tibia include a plethora of differential diagnoses. The most common ones are the Giant cell tumor, fibrous dysplasia, adamantinoma, chondromyxoid fibroma, and osteoblastoma. The rarer ones include vascular tumors such as hemangioma and hemangioendothelioma. A systematic line of investigations is essential to pick up the right diagnosis especially in case of rarer conditions. In this background, we present a case of lytic lesion of the proximal tibia which turned out to be epitheloid hemangioendothelioma (EHE). CASE REPORT A 37-year-old female presented with pain and swelling in the left knee for 2 years. On examination, the patient had a 3 × 4 cm firm, non-tender, and well-defined swelling on the anterolateral aspect of the proximal tibia. X-ray showed a lytic lesion of the proximal tibia. Magnetic resonance imaging was suggestive of a giant cell tumor. However, the biopsy revealed a rare diagnosis of epithelioid hemangioendothelioma. CONCLUSION The patient was managed with wide excision. In a young adult, arthrodesis may not be the best option; hence, we reconstructed the joint with a custom mega prosthesis (CMP). At 3 years follow-up, our patient had no signs of recurrence. To our best knowledge, this is the first report on the use of CMP in a case of EHE.
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Affiliation(s)
- Sathish Muthu
- Department of Orthopaedics, Orthopaedic Research Group, Coimbatore, Tamil Nadu, India
- Department of Orthopaedics, Dindigul Government Medical College Hospital, Dindigul, Tamil Nadu, India
- Address of Correspondence: Dr. Girinivasan Chellamuthu, Research Associate, Orthopaedic Research Group, Church Road, Ramanathapuram, Coimbatore - 641 045, Tamil Nadu, India. E-mail:
| | - Velmurugan Kandasamy
- Department of Orthopaedics, Orthopaedic Research Group, Coimbatore, Tamil Nadu, India
- Department of Orthopaedics, Government Royapettah Hospital, Chennai, Tamil Nadu, India
| | | | - Aditya Thakur
- Department of Orthopaedics, Orthopaedic Research Group, Coimbatore, Tamil Nadu, India
- Department of Orthopaedics, Government Hospital, Rameshwaram, Tamil Nadu, India
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Tossan P, Pichon E, Arbion F, Sizaret D, Simionca G, Marchand-Adam S. Hémangioendothéliome épithélioïde pleural, une tumeur rare de diagnostic difficile : à propos d’un cas et revue de la littérature. Rev Mal Respir 2022; 39:398-406. [DOI: 10.1016/j.rmr.2022.03.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2021] [Accepted: 02/24/2022] [Indexed: 11/26/2022]
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Surgical Resection for Extensive Pleural Epithelioid Hemangioendothelioma. Ann Thorac Surg 2021; 114:e205-e207. [PMID: 34958774 DOI: 10.1016/j.athoracsur.2021.11.051] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2021] [Accepted: 11/14/2021] [Indexed: 11/21/2022]
Abstract
A 35-year-old male who presented with months of symptomatic nonproductive cough was found to have a large right-sided pleural effusion, diffuse pleural thickening, lymphadenopathy, and partial collapse of the right lung. He was diagnosed with a rare form of vascular tumor; pleural epithelioid hemangioendothelioma (PEHE). He underwent a successful right extrapleural pneumonectomy with diaphragm and pericardial resection and reconstruction. While often managed with first line chemotherapy or radiation therapy, our case shows that up front surgical resection is feasible for extensive PEHE at a high-volume regional center. Whether aggressive resection improves survival is yet to be determined.
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Askari E, Yaghmaei S, Haseli S, Pouradollah Totkaboni M. Primary Pleural Hemangioendothelioma: A Case Report and Literature Review. Case Rep Oncol 2021; 14:1201-1211. [PMID: 34703437 PMCID: PMC8460936 DOI: 10.1159/000518243] [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: 05/28/2021] [Accepted: 06/26/2021] [Indexed: 12/02/2022] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare tumor of the vascular endothelial cells. It can originate from any tissue; however, it occurs most frequently in the liver and lung. Pleural epithelioid hemangioendothelioma (PEH), EHE developing from pleura, is even more infrequent and less reported in the literature. In the following report, we discuss a 40-year-old man who initially presented with right-sided chest pain. Computed tomography revealed pleural thickening and effusion in his right thoracic cavity. PEH diagnosis was confirmed with immunohistochemistry. In order to provide the readers with an inclusive understanding of the disease, we collected the PEH cases reported in the literature. Despite the scarcity of the reported PEH cases (to our best knowledge), the compiled literature review of the disease enables the readers to grasp a better comprehension of the disease.
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Affiliation(s)
- Elham Askari
- Department of Pathology, Chronic Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Shekoofeh Yaghmaei
- Department of General Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Sara Haseli
- Department of Radiology, Chronic Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Mihan Pouradollah Totkaboni
- Department of Pathology, Chronic Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
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Witte S, Weidema M, Kaal S, Versleijen-Jonkers Y, Flucke U, van der Graaf W, Desar I. The heterogeneity of Epithelioid Hemangioendothelioma (EHE): A case series and review of the literature with emphasis on treatment options. Semin Oncol 2021; 48:111-118. [PMID: 34176654 DOI: 10.1053/j.seminoncol.2021.04.002] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/16/2019] [Revised: 04/07/2021] [Accepted: 04/14/2021] [Indexed: 12/28/2022]
Abstract
Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular sarcoma with a very heterogeneous presentation and prognosis. We here present five cases of EHE emphasizing diversity in presentation, treatment, and prognosis. Furthermore, we present a review of the literature on EHE treatment options.
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Affiliation(s)
- Stijn Witte
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Marije Weidema
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Suzanne Kaal
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | | | - Uta Flucke
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Winette van der Graaf
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands
| | - Ingrid Desar
- Department of Medical Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands.
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11
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Zhang XQ, Chen H, Song S, Qin Y, Cai LM, Zhang F. Effective combined therapy for pulmonary epithelioid hemangioendothelioma: A case report. World J Clin Cases 2020; 8:2009-2015. [PMID: 32518794 PMCID: PMC7262721 DOI: 10.12998/wjcc.v8.i10.2009] [Citation(s) in RCA: 5] [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: 02/13/2020] [Revised: 04/13/2020] [Accepted: 04/24/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare disease. Thus far, consensus on a standard treatment for P-EHE has not been established given its low incidence worldwide. Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide has been used as an effective combination treatment for human malignancies. However, the efficacy of this combination has not been reported in P-EHE cases.
CASE SUMMARY We present the case of a 64-year-old woman with chest tightness, cough, and chest pain. Computed tomography showed multiple unresectable pulmonary nodules. She had been misdiagnosed with lung carcinoma and underwent gefitinib treatment at a hospital. Subsequently, the patient underwent a cardiothoracic surgery for further disease investigation. CD31, CD34, and Vimentin expression were detected in the resected nodule specimens by immunohistochemical analyses, and pathological analyses confirmed the diagnosis of P-EHE. Following this, four cycles of apatinib combined with chemotherapy with doxorubicin/cyclophosphamide were initiated. The patient demonstrated stabilization of multiple bilateral nodules and showed a dramatic improvement in the clinical presentation after combination treatment. The patient could not tolerate the side effects of chemotherapy. Therefore, she then continued apatinib monotherapy, which is ongoing to date. The patient was stable at the last follow-up after 24 mo.
CONCLUSION Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide may be an effective therapeutic option for P-EHE treatment.
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Affiliation(s)
- Xiu-Qin Zhang
- Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
| | - Heng Chen
- Department of Hematology Medicine, Wuxi People’s Hospital, Wuxi 214000, Jiangsu Province, China
| | - Shu Song
- Department of Pathology Medicine, Shanghai Public Health Clinical Center, Fudan University, Shanghai 200000, China
| | - Yan Qin
- Department of Pathology Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
| | - Li-Ming Cai
- Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
| | - Fang Zhang
- Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
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Cousin S, Le Loarer F, Crombé A, Karanian M, Minard-Colin V, Penel N. [Epithelioid hemangioendothelioma]. Bull Cancer 2018; 106:73-83. [PMID: 30527817 DOI: 10.1016/j.bulcan.2018.11.004] [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: 07/24/2018] [Revised: 10/28/2018] [Accepted: 11/01/2018] [Indexed: 12/14/2022]
Abstract
We describe herein the inaugural manifestations, the radiological and histological diagnosis criteria for and the outcome of epithelioid hemangioendothelioma (EHE). Most of EHE (90%) display a specific reciprocal chromosomic translocation t(1;3)(p36;q23-25), which is associated with the synthesis of fusion protein WWTR1/CAMTA1. EHE are low-grade vascular sarcomas. EHE could be initially localized or multifocal. At localized stage, EHE are best treated with focal treatments. At multifocal stage, the upfront strategy is watchful follow-up. Some multifocal EHE display very indolent course with spontaneous stable disease for years or decades. In case of progressive multifocal EHE, there is no consensual treatment. Diagnostic and clinical management of EHE requires interdisciplinary expertise from labeled centers.
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Affiliation(s)
- Sophie Cousin
- Institut Bergonié, département d'oncologie médicale, 229, cours de l'Argonne, 33000 Bordeaux, France
| | - François Le Loarer
- Institut Bergonié, département d'anatomopathologie, 229, cours de l'Argonne, 33000 Bordeaux, France
| | - Amandine Crombé
- Institut Bergonié, département d'imagerie médicale, 229, cours de l'Argonne, 33000 Bordeaux, France
| | - Marie Karanian
- Centre Léon Bérard, département d'anatomopathologie, 28, Promenade Léa et Napoléon Bullukian, 69008 Lyon, France
| | - Véronique Minard-Colin
- Institut Gustave Roussy, département d'oncologie pédiatrique, 114, rue Edouard-Vaillant, 94800 Villejuif, France
| | - Nicolas Penel
- Centre Oscar Lambret, département d'oncologie médicale, 3, rue Frédéric Combemale, 59000 Lille, France; Hôpital Claude Huriez, service d'oncologie médicale, rue Michel Polonowski, 59000 Lille, France.
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Lamar JM, Motilal Nehru V, Weinberg G. Epithelioid Hemangioendothelioma as a Model of YAP/TAZ-Driven Cancer: Insights from a Rare Fusion Sarcoma. Cancers (Basel) 2018; 10:cancers10070229. [PMID: 29996478 PMCID: PMC6070876 DOI: 10.3390/cancers10070229] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2018] [Revised: 07/08/2018] [Accepted: 07/09/2018] [Indexed: 02/07/2023] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare soft-tissue sarcoma involving cells with histologic markers that suggest an endothelial origin. Around 90% of EHEs are caused by the fusion of Transcriptional Co-activator with a PDZ-motif (TAZ) with Calmodulin Binding Transcription Activator 1 (CAMTA1), a central nervous system-specific transcription activator. The 10% of EHEs that lack the TAZ–CAMTA1 fusion instead have a fusion of Yes-associated Protein (YAP) and Transcription Factor E3 (TFE3) genes (YAP-TFE3). YAP and TAZ are well-defined downstream effectors in the Hippo pathway that promote cell growth when translocated to the nucleus. The TAZ–CAMTA1 fusion transcript is insensitive to the Hippo inhibitory signals that normally prevent this process and thus constitutively activates the TAZ transcriptome. In EHE, this causes tumors to form in a variety of organs and tissue types, most commonly the liver, lung, and bone. Its clinical course is unpredictable and highly variable. TAZ activation is known to contribute to key aspects of the cancer phenotype, including metastasis and fibrosis, and increased expression of TAZ is thought to be causally related to the progression of many cancers, including breast, lung, and liver. Therefore, understanding TAZ biology and the molecular mechanisms by which it promotes unregulated cell proliferation will yield insights and possibly improved treatments for both EHE as well as much more common cancers.
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Affiliation(s)
- John M Lamar
- Department of Molecular and Cellular Physiology, Albany Medical College, Albany, NY 12208, USA.
| | | | - Guy Weinberg
- Department of Anesthesiology, University of Illinois College of Medicine, and Jesse Brown VA Medical Center, Chicago, IL 60612, USA.
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Dolzhansky OV, Paltseva EM, Morozova MM, Bazarov DV, Boranov EV, Fedorov DN. [Solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal amyloidosis of the lung and Rosai-Dorfman disease]. Arkh Patol 2018; 80:52-59. [PMID: 29697673 DOI: 10.17116/patol201880252-59] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
The paper describes a case of solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal AL amyloidosis of the lung and Rosai-Dorfman disease in a 70-year-old woman. The core of the tumor was represented by bone tissue with dendriform ossification, as well as by amyloid that showed green apple birefringence at polarized light microscopy. The peripheral portions of the tumor and the myxohyaline stroma exhibited slit-like structures, epithelioid and fusiform cells with small cytoplasmic vacuoles. These cells expressed CD31, CD34, factor VIII, and cytokeratins 7 and 18. The Ki-67 proliferation index was 10%. S100- and CD68-positive histiocytes with the phenomenon of emperipolesis were revealed in the tumor and in the lymph nodes of the mediastinum and lung hilum. There was a positive reaction to immunoglobulin lambda light chains in the lymphocytic infiltration around amyloid clumps. The frequency of epithelioid hemangioendothelioma was less than 1 case per million people annually. We found only one case of its concurrence with pulmonary amyloidosis in the English-language literature. No relationship could be revealed between this tumor and Rosai-Dorfman disease.
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Affiliation(s)
- O V Dolzhansky
- Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow, Russia
| | - E M Paltseva
- Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow, Russia
| | - M M Morozova
- Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow, Russia
| | - D V Bazarov
- Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow, Russia
| | - E V Boranov
- Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow, Russia
| | - D N Fedorov
- Acad. B.V. Petrovsky Russian Surgery Research Center, Moscow, Russia
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Abstract
OPINION STATEMENT Epithelioid hemangioendothelioma (EHE) is an extremely rare sarcoma, as such it can pose a clinical dilemma based solely on its rarity. Also, the spectrum of disease varies greatly between an indolent disease and aggressive disease with widespread metastases. In our clinical practice, the primary focus has been to get a handle on the aggressive nature of the disease, which will then dictate how urgently one needs to treat the patient. Pathological review with immunohistochemistry and molecular characterization is paramount. Our treatment strategy is watch-and-wait versus active therapy on clinical trial or based on results of prior clinical trials. There is evidence to support the use of chemotherapeutics and targeted therapies specifically focusing on anti-angiogenesis. The current landscape of oncology with the emergence and excitement of immunotherapy could also translate in a role for immunotherapy in this disease. While rare, there is certainly no reason that research and trials for patients with EHE should not remain on utmost importance for those of us who specialize in the treatment of sarcomas.
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Kanemura S, Kuribayashi K, Moriya Y, Shimizu S, Tsujimura T, Nakano T. Pemetrexed for epithelioid haemangioendothelioma of the pleura. Respirol Case Rep 2016; 4:e00191. [PMID: 28031827 PMCID: PMC5167279 DOI: 10.1002/rcr2.191] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2016] [Revised: 08/22/2016] [Accepted: 08/25/2016] [Indexed: 12/20/2022] Open
Abstract
A 31‐year‐old woman presented with a 2‐month history of dull left back pain. Chest roentgenography revealed pleural thickening in the left lung apex, whereas chest computed tomography showed a left pleural effusion with diffuse pleural thickening. Based on these findings, malignant mesothelioma was suspected. Video‐assisted thoracoscopic pleural biopsy was performed, and subsequent staining of the specimen revealed negative results for anti‐cytokeratin antibodies and positive results for vascular endothelial immunohistochemical markers. Therefore, a diagnosis of epithelioid hemangioendothelioma (EHE) was made, and chemotherapy with carboplatin, pemetrexed, and bevacizumab was initiated. After the fourth course of treatment, the disease was well controlled with a 90% reduction of the left pleural effusion. This case is very rare in that the EHE originated from the pleura. Additionally, there has been no report of pemetrexed for treatment of EHE.
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Affiliation(s)
- Shingo Kanemura
- Department of Respiratory Medicine Hyogo College of Medicine Hyogo Japan
| | - Kozo Kuribayashi
- Department of Respiratory Medicine Hyogo College of Medicine Hyogo Japan
| | - Yumiko Moriya
- Department of Respiratory Medicine Hyogo College of Medicine Hyogo Japan
| | - Shigeki Shimizu
- Department of Pathology Hyogo College of Medicine Hyogo Japan
| | - Tohru Tsujimura
- Department of Pathology Hyogo College of Medicine Hyogo Japan
| | - Takashi Nakano
- Department of Respiratory Medicine Hyogo College of Medicine Hyogo Japan
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