Epithelioid hemangioendothelioma of the lung: a clinical case
https://doi.org/10.18027/2224-5057-2025-052
Abstract
Epithelioid hemangioendothelioma (EGE) is an extremely rare vascular sarcoma characterized by unique morphological and molecular features. The incidence is about 1 case per million people. This publication presents data on clinical cases with various localizations of EGE published in the Russian literature. Our unique clinical example of lung EGE is presented, which was accompanied by significant difficulties in histological verification. The paper analyzes the features of the diagnosis of this disease, as well as presents data on the treatment and its results.
About the Authors
A. N. Turomsha
AVA-PBTBR LLC
Russian Federation
Competing Interests:
Russian Federation
Alesya Nikolaevna Turomsha
4/1 Ilyushina St., Saint Petersburg 197372
Competing Interests:
The authors declare that there are no possible conflicts of interest.
A. E. Protasova
AVA-PBTBR LLC ; V. A. Almazov National Medical Research Centre, Ministry of Health of Russia ; Saint Petersburg State University
Russian Federation
Competing Interests:
Russian Federation
Anna Eduardovna Protasova
4/1 Ilyushina St., Saint Petersburg 197372
2 Akkuratova St., Saint Petersburg 197341
7-9 Universitetskaya Emb., Saint Petersburg 19903
Competing Interests:
The authors declare that there are no possible conflicts of interest.
M. Yu. Sheremet
AVA-PBTBR LLC
Russian Federation
Competing Interests:
Russian Federation
Marina Yurevna Sheremet
4/1 Ilyushina St., Saint Petersburg 197372
Competing Interests:
The authors declare that there are no possible conflicts of interest.
References
1. Liu Z., Не S. Epithelioid hemangioendothelioma: incidence, mortality, prognostic factors, and survival analysis using the surveillance, epidemiology, and end results database. J Oncol 2022;2022:2349991. https://doi.org/10.1155/2022/2349991
2. Amin R.M.S., Hiroshima K., Kokubo T., et al. Risk factors and independent predictors of survival in patients with pulmonary epithelioid haemangioendothelioma. Review of the literature and a case report. Respirology 2006;ll(6):818-825. https://doi.Org/10.llll/j.1440-1843.2006.00923.x
3. Tsuchihashi K., Baba E. Epithelioid hemangioendothelioma-its history, clinical features, molecular biology and current therapy.jpnj Clin Oncol 2024;54(7):739-747. https://doi.org/10.1093/jjco/hyae037
4. Errani C., Zhang L., Sung Y.S., et al. A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes Chromosomes Cancer 2011;50(8):644-653. https://doi.org/10.1002/gcc.20886
5. Weiss S.W., Enzinger F.M. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 1982;50(5):970-81. https://doi.org/10.1002/1097-0142(19820901)50:5<970::aid-cncr2820500527>3.0.co;2-z
6. Travis W.D., Brambilla E., Nicholson A.G., et al. The 2015 World Health Organization classification of lung tumors: impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol 2015;10(9):1243—1260. https://doi.org/10.1097/JTO.0000000000000630
7. Mentzel T., Beham A., Calonje E., et al. Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases. Am J Surg Pathol 1997;21(4):363—374. https://doi.org/10.1097/00000478-199704000-00001
8. Hornick J.L., Dal C.P., Fletcher C.D. Loss of INI1 expression is characteristic of both conventional and proximal-type epithelioid sarcoma. AmJ Surg Pathol 2009;33(4):542—550. https://doi.org/10.1097/PAS.0b013e3181882c54
9. Lau K., Massad M., Poliak C., et al. Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer. Chest 2011;140:1312-1318. https://doi.org/10.1378/chest.ll-0039
10. Tong D., Constantinidou A., Engelmann B., et al. The role of local therapy in multi-focal epithelioid haemangioendothelioma. Anticancer Res 2019;39(9):4891-4896. https://doi.org/10.21873/anticanres.13675
11. Frezza A.M., Napolitano A., Miceli R. Clinical prognostic factors in advanced epithelioid haemangioendothelioma: a retrospective series within the Italian Rare Cancers Network. ESMO open 2021;6(2):100083. https://doi.org/10.1016/j.esmoop.2021.100083
12. Kollar A., Jones R.L., Stacchiotti S., et al. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis. Acta Oncol 2017;56(l):88—92. https://doi.org/10.1080/0284186X.2016.1234068
13. Schuetze S.M., Ballman K.V., Heise R., et al. A single-arm phase 2 trial of trametinib in patients with locally advanced or metastatic epithelioid hemangioendothelioma. Clin Cancer Res 2024;30(20):4584-4592. https://doi.org/10.1158/1078-0432.CCR-23-3817
14. Yousaf N., Maruzzo M., Judson .1, et al. Systemic treatment options for epithelioid haemangioendothelioma: the Royal Marsden Hospital experience. Anticancer Res 2015;35(l):473—80
15. Pranteda G., Magri F., Muscianese M., et al. The management of pseudomyogenic hemangioendothelioma of the foot: A case report and review of the literature. Dermatol Ther 2018;3l(6):el2725. https://doi.org/10.llll/dth.12725
16. Pinet C., Magnan A., Garbe L., et al. Aggressive form of pleural epithelioid haemangioendothelioma: complete response after chemotherapy. Eur RespirJ 1999;14(l):237-8. https://doi.Org/10.1034/j.1399-3003.1999.14a40.x
17. Stacchiotti S., Provenzano S., Dagrada G., et al. Sirolimus in advanced epithelioid hemangioendothelioma: a retrospective case-series analysis from the italian rare cancer network database. Ann Surg Oncol 2016;23(9):2735-44. https://doi.org/10.1245/sl0434-016-5331-z
18. Stacchiotti S., Simeone N., Lo Vullo S., et al. Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: A case-series analysis within the Italian Rare Cancer Network. Cancer 2021;127(4):569-576. https://doi.org/10.1002/cncr.33247
19. Stacchiotti S., Miah A.B., Frezza A.M., et al. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts. ESMO Open 2021;6(3):100170. https://doi.Org/10.1016/j.esmoop.2021.100170
20. Dvorakovskaya I.V., Bugrov S.N., Novikova L.N., et al. Pulmonary epithelioid hemangioendothelioma. Russian Journal of Archive of Pathology 2022;84(2):29-35. (In Russ.) https://doi.org/10.17116/patol20228402129
21. Korobkova I.Z., Dremin D.A., Kakalov S.M., et al. Hepatic epithelioid hemangioendothelioma. Journal of radiology and nuclear medicine 2019;100(6):372-378 (In Russ.), https://doi.org/10.20862/0042-4676-2019-100-6-372-378
22. Kuznecov M.S., Voronov A.V., Savello A.V., et al. Diagnosis and treatment of epitelioid hemangioedothelioma of synonasal localization: clinical case. Head and neck Russian Journal 2020;8(2):59-64 (in Russ.)
23. Bakotina A.V., Kabardiev S.R., Zhuravleva A.V., et al. A rare clinical case of nasal cavity epithelioid hemangioendothelioma. Russian Rhinology 2022;30(4):282-287 (In Russ.), https://doi.org/10.17116/rosrino202230041282
24. Denisov S.N., Gorgan I.V., Mayorov A.E., et al. Malignant epithelioid hemangioendotheliom of soft tissue of the right thigh (clinical case). Oncology Bulletin of the Volga Region 2024;15(l):125—131 (In Russ.), https://doi.org/10.32000/2078-1466-2024-1-125-131
25. Kolcheva М.А., Kumirova E.V., Livshits M.I., et al. Clinical case of primary multiple hemangioendothelioma in a child with CNS lesions. Russian Journal of Pediatric Hematology and Oncology 2022;9(4):88—95 (In Russ.). https://doi.org/10.21682/2311-1267-2022-9-4-88-95
Review
For citations:
Turomsha A.N., Protasova A.E., Sheremet M.Yu. Epithelioid hemangioendothelioma of the lung: a clinical case. Malignant tumours. 2025;15(3):61-67. (In Russ.) https://doi.org/10.18027/2224-5057-2025-052
Views:
12
Email this article 