Thoracic Epithelioid Hemangioendothelioma: clinical demonstration and therapeutic procedures


  • Luís Lourenço Graça Cardiothoracic Surgery Department, Centro Hospitalar Universitário de Coimbra, Portugal
  • Sara Almeida Cunha Cardiothoracic Surgery Department, Centro Hospitalar Universitário de Coimbra, Portugal
  • Rita Susana Lopes Pathology Department, Centro Hospitalar Universitário de Coimbra, Portugal
  • Lina Carvalho Cardiothoracic Surgery Department, Centro Hospitalar Universitário de Coimbra, Portugal
  • David Prieto Cardiothoracic Surgery Department, Centro Hospitalar Universitário de Coimbra, Portugal



Epithelioid hemangioendotheliomais a low to intermediate grade malignant vascular tumors that can involve any organ. About 60-80%of patients are women, patient ages range 7 to 81 years, with a median age of 38 years. Four cases of thoracic epithelioid hemangioendotheliomas with different clinical presentation and disease progression are reported. Cases 1 and 2 are pulmonary epithelioid hemangioendotheliomas diagnosed at different advanced stages and patients died after 6 and 2,5 months of medical treatment, respectively. Case 3 corresponds to pleural epithelioid hemangioendothelioma, submit- ted to left lung decortication and pleuro-pericardial window; patient is free either from symptoms and radiographic manifestations for 10 months of follow-up. Case 4, of mediastinal epithelioid hemangioendothelioma, represented by a mass in the upper left mediastinum adherent to the aortic arch; patient underwent block excision of the mass followed by chemotherapy; subsequent recurrence 41 months later and the patient died 8 months after. The reported 4 cases reveal the heterogeneous clinical presentation of epithelioid hemangioendotheliomas with behavior in between benign and high-grade tumors, raising difficulty in either differentiating from other vascular tumors and previewing clinical outcome.


Download data is not yet available.


Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev. 2014;8(2):259.

Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 1982;50:970–981

Salech, F., Valderrama, S., OksenbergReisberg, D., et al. (2011 JaneMar). Thalidomide for the treatment of metastatic hepatic epithelioid hemangioendothelioma: a case report with a long term follow-up. Ann Hepatol, 10, 99e102.

Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, et al. Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma. Sci Transl Med. 2011;3(98):98ra82.

WHO Classification of Tumours Editorial Board (2020). Soft Tissue and Bone Tumours, WHO Classification of Tumours, 5th Edition, Volume 3.

Rosenberg A, Agulnik M. Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment. Curr. Treat. Options in Oncol. 2018; 19: 19

Kitaichi, M., Nagai, S., Nishimura, K., Itoh, H., Asamoto, H., Izumi, T., &Dail, D. H. (1998). Pulmonary epithelioid haemangioendothelioma in 21 patients , 89–96.

Bagan, P., Hassan, M., Le, F., Barthes, P., Peyrard, S., Souilamas, R., …Riquet, M. (2006). Prognostic Factors and

Surgical Indications of Pulmonary Epithelioid Hemangioendothelioma: A Review of the Literature.

Amin, R. M. S., Hiroshima, K., Kokubo, T., Nishikawa, M., & Narita, M. (2006). CASE REPORT Risk factors and independent predictors of survival in patients with pulmonary epithelioid haemangioendothelioma . Review of the literature and a case report, 818–825.

Lau, K., Massad, M., Pollak, C., Rubin, C., Yeh, J., Wang, J., … Weinberg, G. (2011). Clinical Patterns and Outcome in Epithelioid HemangioendotheliomaWith or Without Insights From an Internet Registry in the Study. CHEST, 140(5), 1312–1318.

Woo, J., Kim, T., Lee, K., Kim, T., & Kim, B. (2016). Epithelioid hemangioendothelioma in the thorax. Medicine,

(30), 1–8.

Fan, Y., Wang, F., Li, S., Ye, C., Ying, Y., & Mao, H. (2016). Pleural Epithelioid Hemangioendothelioma: A Case Report and Literature Review. Journal of the National Medical Association, 108(2), 124–129.

Ha, S. Y., Choi, I. H., Han, J., Choi, Y., Cho, J. H., Lee, K.- J., & Sun, J.-M. (2014). Lung Cancer Pleural epithelioid hemangioendothelioma harboring CAMTA1 rearrangement. Lung Cancer, 83(3), 411–415.

Lin, B. T., Colby, T., Gown, A. M., et al. (1996). Malignant vascular tumors of the serous membranes mimicking

meso- thelioma: a report of 14 cases. Am J SurgPathol, 20, 1431e1439.

Yu, L., Gu, T., Xiu, Z., Shi, E., &Zhao, X. (2013). Primary pleural epithelioid hemangioendothelioma compressing

the myocardium. JournalofCardiacSurgery, 28(3), 266–268.

Saqi, A., Nisbet, L., Gagneja, P., & Leslie, K. O. (2007). Primary Pleural Epithelioid HemangioendotheliomaWithRhabdoid Phenotype: Report and Review of the Literature. DiagnosticCytopathology, 35(4), 203–208.

Cronin, P., &Arenberg, D. (2004). Pulmonary Epithelioid Hemangioendothelioma: An Unusual Case and a Review of the Literature. Chest, 125(2), 789–792.

Lazarus, A., Fuhrer, G., Malekiani, C., McKay, S., & Thurber, J. (2011). Primary pleural epithelioid hemangioendothelioma (EHE) - two cases and review of the literature. The Clinical Respiratory Journal, 5(1), e1–e5.

Martínez, D., Iturbe, J., Buzeki, R., et al. (2008). Hemangioen-dotelioma epitelioide de pleura. Presentacion de un caso conhemotorax. RevArgentMed Respire, 8,73e76.

Ishibashi H, Takasaki C, Akashi T, Okubo K, Successful excision of epithelioid hemangioendothelioma of the superior vena cava, The Annals of Thoracic Surgery (2019), doi: https://

De Palma, A., Pagliarulo, V., Ardò, N., &Loizzi, D. (2012). Surgical treatment of a rare case of epithelioid hemangioendothelioma of the azygos vein. Interactive Cardiovascular and Thoracic Surgery, 14(1), 91–93. icvts/ivr064

Deyrup, A. T., Tighiouart, M., Montag, A. G., & Weiss, S. W. (2008). Epithelioid hemangioendothelioma of soft tissue: A proposal for risk stratification based on 49 cases. AmericanJournalofSurgicalPathology, 32(6), 924–927. https://

Suster, S., Moran, C., & Koss, M. (1994). Epithelioid hemangioendothelioma of the anterior mediastinum. Clinicopath- ologic, immunohistochemical, and ultrastructural analysis of 12 cases. Am J Surg Pathol, 18(9), 871–881.

Flucke, U., Vogels, R. J. C., de Saint AubainSomerhausen, N., Creytens, D. H., Riedl, R. G., van Gorp, J. M., Milne, A. N., Huysentruyt, C. J., Verdijk, M. A. J., van Asseldonk, M. M., Suurmeijer, A. J. H., Bras, J., Palmedo, G., Groenen, P. J. T. A., &Mentzel, T. (2014). Epithelioid Hemangioendothelioma: Clinicopathologic, immunhistochemical, and molecular genetic analysis of 39 cases. Diagnostic Pathology, 9(1), 1–12.

Anderson, T., Zhang, L., Hameed, M., Rusch, V., Travis, W. D., &Antonescu, C. R. (2015). Thoracic epithelioid malig- nant vascular tumors: A clinicopathologic study of 52 cases with emphasis on pathologic grading and molecular stud- ies of WWTR1-CAMTA1 fusions. American Journal of Surgical Pathology, 39(1), 132–139. PAS.0000000000000346

Murali, R., Zarka, M. A., Ocal, I. T., &Tazelaar, H. D. (2011). Cytologic features of epithelioid hemangioendothelioma. American Journal of Clinical Pathology, 136(5), 739–746. JCP5NK0FJCGHTFM

Errani, C., Zhang, L., Shao, S., Hajdu, M., Singer, S., & Maki, R., et al. (2011). A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes Chromosom Cancer, 50(8), 644–653.

Antonescu, C., Le Loarer, F., Mosquera, J.-M., Sboner, A., Zhang, L., & Chen, C.-L. (2013). Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosom Cancer, 52(8), 775–84. https://doi. org/10.1002/gcc.22073.Novel

Lee, S. J., Yang, W. I., Chung, W. S., & Kim, S. K. (2016). Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements. Oncotarget, 7(7), 7480–7488. https://doi. org/10.18632/oncotarget.7060

Saste, A., Fernandez, D. F. C., Gulati, R., &Gamalski, S. (2015). A trimodality approach in the management of met- astatic low-grade epithelioid hemangioendothelioma of the bone. BMJ Case Reports, 1–8. bcr-2015-210196




How to Cite

Graça LL, Almeida Cunha S, Lopes RS, Carvalho L, Prieto D. Thoracic Epithelioid Hemangioendothelioma: clinical demonstration and therapeutic procedures. Rev Port Cir Cardiotorac Vasc [Internet]. 2022 Jul. 3 [cited 2022 Sep. 27];29(2):39-44. Available from:



Original Articles