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Pulle MV, Bhan A, Bishnoi S, Asaf BB, Puri HV, Bangeria S, Parikh MB, Kumar A. Epithelioid hemangioendothelioma of the anterior mediastinum. Indian J Thorac Cardiovasc Surg 2025; 41:456-458. [PMID: 40144603 PMCID: PMC11933484 DOI: 10.1007/s12055-024-01819-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2024] [Revised: 08/12/2024] [Accepted: 08/13/2024] [Indexed: 03/28/2025] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a very rare vascular neoplasm in the anterior mediastinum. It is considered an intermediate-grade vascular neoplasm between benign hemangioma and highly aggressive angiosarcoma, with the potential for distant metastasis. The management of this disease is dependent on various factors such as the extent of tumor involvement, presence/absence of metastasis, and the general condition of the patient. Surgery is the treatment of choice with/without adjuvant chemo-radiotherapy in resectable disease. This case report presents the successful surgical management of a large invasive EHE in the anterior mediastinum in a 50-year-old woman.
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Affiliation(s)
- Mohan Venkatesh Pulle
- Institute of Chest Surgery, Medanta, Room No. 12, 4th Floor, OPD Block, Medanta – The Medicity, Sector-38, Gurugram, Haryana 122001 India
| | - Anmol Bhan
- Dayanand Medical College, Ludhiana, Punjab India
| | - Sukhram Bishnoi
- Institute of Chest Surgery, Medanta, Room No. 12, 4th Floor, OPD Block, Medanta – The Medicity, Sector-38, Gurugram, Haryana 122001 India
| | - Belal Bin Asaf
- Institute of Chest Surgery, Medanta, Room No. 12, 4th Floor, OPD Block, Medanta – The Medicity, Sector-38, Gurugram, Haryana 122001 India
| | - Harsh Vardhan Puri
- Institute of Chest Surgery, Medanta, Room No. 12, 4th Floor, OPD Block, Medanta – The Medicity, Sector-38, Gurugram, Haryana 122001 India
| | - Sumit Bangeria
- Institute of Chest Surgery, Medanta, Room No. 12, 4th Floor, OPD Block, Medanta – The Medicity, Sector-38, Gurugram, Haryana 122001 India
| | - Manan Bharatkumar Parikh
- Institute of Chest Surgery, Medanta, Room No. 12, 4th Floor, OPD Block, Medanta – The Medicity, Sector-38, Gurugram, Haryana 122001 India
| | - Arvind Kumar
- Institute of Chest Surgery, Medanta, Room No. 12, 4th Floor, OPD Block, Medanta – The Medicity, Sector-38, Gurugram, Haryana 122001 India
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Liţescu M, Abduraim T, Paverman L, Vrabie CD, Dina I, Pleşea IE, Grigorean VT. Epithelioid hemangioendothelioma - an unexpected diagnosis of a mediastinal tumor with extensive local thrombosis. ROMANIAN JOURNAL OF MORPHOLOGY AND EMBRYOLOGY = REVUE ROUMAINE DE MORPHOLOGIE ET EMBRYOLOGIE 2022; 63:197-202. [PMID: 36074684 PMCID: PMC9593112 DOI: 10.47162/rjme.63.1.21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/20/2022] [Accepted: 08/02/2022] [Indexed: 06/15/2023]
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor and the mediastinal localization is amongst the most infrequent. We present the case of a 37-year-old woman with a history of resected left thyroid tumor that presented to our department for evaluation of a left supraclavicular palpable mass in close contact with local vascular structures, and with heterogeneous contrast enhancement as described by computed tomography (CT) and magnetic resonance imaging (MRI). Considering the history of the patient, the presumptive diagnosis of thyroid tumor recurrence was established, and the patient was referred to surgical department. During procedure, we encountered important bleeding from a ruptured jugular vein branch, which we assumed to be a newly formed tumor blood vessel. After surgery (48 hours postoperatively), the patient developed important local thrombosis that encompassed the left internal jugular vein, left subclavian vein and the left brachiocephalic trunk that partially subsided after anticoagulant therapy. The histological examination revealed the presence of a vascular tumor proliferation of epithelioid endothelial cells that was characteristic of an EHE confirmed later on the immunohistochemical studies as Yes-associated protein 1-transcription factor E3 (YAP1-TFE3) subtype. In addition to the case report, some relevant information from the scarce literature data about mediastinal EHE were reviewed here.
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Affiliation(s)
- Mircea Liţescu
- Departments of Pathology, Carol Davila University of Medicine and Pharmacy, and Fundeni Clinical Institute, Bucharest, Romania;
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Zhi Q, Ma Z, Lin G, Pan J, Chen B. Long-Term Observation and Treatment of Epithelioid Haemangioendothelioma of the Mediastinum: A Case Report. Front Surg 2021; 8:678572. [PMID: 34676238 PMCID: PMC8525909 DOI: 10.3389/fsurg.2021.678572] [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: 05/26/2021] [Accepted: 09/03/2021] [Indexed: 11/21/2022] Open
Abstract
Epithelioid haemangioendothelioma is a rare angiogenic tumour originating from vascular endothelial or pre-endothelial cells, and it can occur anywhere in the body, such as the liver, lung, bone, spleen, lymph nodes, parotid gland, and thyroid. In the fifth revision of the WHO classification, epithelioid haemangioendothelioma (EHE) was described as a malignant vascular neoplasm composed of epithelioid endothelial cells, distinct from epithelioid angiosarcoma. We, herein, report one patient with EHE of the left upper mediastinum who underwent resection and radiotherapy during the first therapeutic process. Multiple metastases occurred in the thoracic vertebrae 6 years later, and resection and multiple radiotherapies were performed. The condition of the patient remained stable at the last review in October 2020, and it has been more than 8 years since her first admission. The reasonable “take-away” lessons from the case are active treatment and prolonged surveillance.
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Affiliation(s)
- Qiuli Zhi
- Department of Radiology, Affiliated Cixi Hospital, Wenzhou Medical University, Cixi, China
| | - Zhoupeng Ma
- Department of Radiology, Jinshan TCM-Integrated Hospital of Shanghai City, Shanghai, China
| | - Guansheng Lin
- Department of Radiology, Jinshan TCM-Integrated Hospital of Shanghai City, Shanghai, China
| | - Jiangfeng Pan
- Department of Radiology, Jinhua Hospital, Zhejiang University School of Medicine, Jinhua, China
| | - Bingye Chen
- Department of Surgery, Jinshan TCM-Integrated Hospital of Shanghai City, Shanghai, China
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Choi JH, Ro JY. Mesenchymal Tumors of the Mediastinum: An Update on Diagnostic Approach. Adv Anat Pathol 2021; 28:351-381. [PMID: 34050062 DOI: 10.1097/pap.0000000000000306] [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: 11/26/2022]
Abstract
Mesenchymal tumors of the mediastinum are a heterogenous group of rare tumors with divergent lineages. Mediastinal mesenchymal tumors are diagnostically challenging due to their diversity and morphologic overlap with nonmesenchymal lesions arising in the mediastinum. Accurate histologic diagnosis is critical for appropriate patient management and prognostication. Many mediastinal mesenchymal tumors affect distinct age groups or occur at specific mediastinal compartments. Neurogenic tumors, liposarcoma, solitary fibrous tumor, and synovial sarcoma are common mesenchymal tumors in the mediastinum. Herein, we provide an update on the diagnostic approach to mediastinal mesenchymal tumors and a review of the histologic features and differential diagnosis of common benign and malignant mesenchymal tumors of the mediastinum.
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Affiliation(s)
- Joon Hyuk Choi
- Department of Pathology, Yeungnam University College of Medicine, Daegu, South Korea
| | - Jae Y Ro
- Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Weill Medical College of Cornell University, Houston, TX
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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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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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