Orthotopic Liver Transplantation in a Case of Novel YAP1-TFE3 Hepatic Epithelioid Hemangioendothelioma [HEHE]; Case Report and Review of Literature
Abstract
Epithelioid hemangioendothelioma is an extremely rare vascular neoplasm arising in soft-tissues and different visceral organs, with liver being the most commonly involved viscera. Hepatic epithelioid hemangioendothelioma (HEHE) is a malignant tumour with an indolent behaviour and unpredictable clinical course. It has a better prognosis among the malignant tumours of the liver, in spite of being a diffuse multifocal liver disease or metastatic at the time of presentation. HEHE is usually found to be noted in the fifth decade with slight female preponderance. No single treatment strategy has yet been established owing to its variable clinical course, ranging from an indolent tumour with prolonged survival to an aggressive, metastatic disease with a fatal outcome. Here, we present a case of a novel HEHE in a 25-year-old female who was treated successfully with orthotopic living donor liver transplantation and discuss the presentation, histopathology and management of this rare, fatal yet treatable malignant tumour.
Keywords
How to Cite
References
Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma. Cancer. 1982;50(5):970–81. 10.1002/1097-0142(19820901)50:5<970::AID-CNCR2820500527>3.0.CO;2-Z
Ishak KG, Sesterhenn IA, Goodman MZD, Rabin L, Stromeyer F. Epithelioid hemangioendothelioma of the liver: A clinicopathologic and follow-up study of 32 cases. Hum Pathol. 1984;15(9):839–52. 10.1016/S0046-8177(84)80145-8
Mehrabi A, Kashfi A, Fonouni H, Schemmer P, Schmied BM, Hallscheidt P, et al. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer. 2006;107(9):2108–2. 10.1002/cncr.22225
Studer LL, Selby DM. Hepatic epithelioid hemangioendothelioma. Arch Pathol Lab Med. 2018;142(2):263–7. 10.5858/arpa.2016-0171-RS
Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver. Cancer. 1999;85(3):562–82. 10.1002/(SICI)1097-0142(19990201)85:3<562::AID-CNCR7>3.0.CO;2-T
Redaelli D, Guraya SS. Primary hemangioendothelioma of liver; report of a case and review of literature. J Taibah Univ Sci. 2015;10(2):243–7. 10.1016/j.jtumed.2014.05.003
Bruegel M, Muenzel D, Waldt S, Specht K, Rummeny EJ. Hepatic epithelioid hemangioendothelioma: findings at CT and MRI including preliminary observations at diffusion-weighted echo-planar imaging. Abdom Imaging. 2011;36(4):415–24. 10.1007/s00261-010-9641-5
Zhou L, Cui MY, Xiong J, Dong Z, Luo Y, Xiao H, et al. Spectrum of appearances on CT and MRI of hepatic epithelioid hemangioendothelioma. BMC Gastroenterol 2015; 15: 69. 10.1186/s12876-015-0299-x
Alomari AI. The lollipop sign: a new cross-sectional sign of hepatic epithelioid hemangioendothelioma. Eur J Radiol 2006; 59: 460–464 10.1016/j.ejrad.2006.03.022
Lai Q, Feys E, Karam V, Adam R, Klempnauer J, Oliverius M, et al. Hepatic epithelioid hemangioendothelioma and adult liver transplantation: Proposal for a prognostic score based on the analysis of the ELTR-ELITA registry. Transplantation. 2017;101(3):555–64 10.1097/TP.0000000000001603
Antonius Suriawintana A. Miscellanous tumors and tumor likelesions. In: Saxena R, editor. Practical Hepatic Pathology: A Diagnostic Approach. Philadelphia, USA: Saunders; 2011. p. 549–51. 10.1016/B978-0-443-06803-4.00039-3
Antonescu CR, Le Loarer F, Mosquera JM, Sboner A, Zhang L, Chen CL, et al. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosomes Cancer. 2013 Aug;52(8):775–84. 10.1002/gcc.22073
Puls F, Niblett A, Clarke J, Kindblom LG, McCulloch T. YAP1-TFE3 epithelioid hemangioendothelioma: a case without vasoformation and a new transcript variant. Virchows Arch. 2015 Apr;466(4):473–8. 10.1007/s00428-015-1730-y
Virarkar M, Saleh M, Diab R, Taggart M, Bhargava P, Bhosale P. Hepatic Hemangioendothelioma: An update. World J Gastrointest Oncol. 2020;12(3):248–66. 10.4251/wjgo.v12.i3.248
Gurung S, Fu H, Zhang WW, Gu YH. Hepatic epithelioid hemangioendothelioma metastasized to the peritoneum, omentum and mesentery: A case report. Int J Clin Exp Pathol. 2015;8(5):5883.
Copyright and licence
Copyright (c) 2021 Sugi SRV, Elankumaran Krishnan, Kulaseharan Venugopal Haribabu, Hari Balakrishnan, Shantha Ravishankar
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
