EVALUATION OF TREATMENT OUTCOMES AND PROGNOSTIC FACTORS IN PATIENTS AFTER LIVER TRANSPLANTATION DUE TO HEPATIC EPITHELIOID HEMANGIOENDOTHELIOMA

Authors

DOI:

https://doi.org/10.31435/ijitss.2(50).2026.5757

Keywords:

Epithelioid Hemangioendothelioma, Hepatic, Liver Tumor, Liver Transplantation, Prognostic Factors, Survival

Abstract

Introduction: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of low-to-intermediate malignant potential with an incompletely defined biological behaviour, exhibiting both benign and malignant features. It most commonly affects the liver, but may also involve other organs, such as the lungs and bones. Due to its heterogeneous clinical presentation and similarity to other vascular tumors primarily affecting the liver, misdiagnosis remains a common clinical challenge. Currently, several therapeutic approaches are used depending on various factors, but liver transplantation (LTx) is increasingly considered as the treatment option for patients with metastatic HEHE, particularly in unresectable cases. Several studies indicate a favorable prognosis following transplantation for HEHE.

Aim of the Study: The aim of this study is to review the existing literature on treatment outcomes and to identify prognostic factors in patients undergoing LTx due to HEHE.

Materials and Methods: A comprehensive literature review was performed using the PubMed database.

Results and Conclusions: LTx is a crucial treatment option for patients with unresectable and multifocal HEHE. Current data indicate that post-transplant survival rates are favorable, with the potential to achieve durable, recurrence-free survival - even in the presence of extrahepatic metastases. Several prognostic factors have been identified that may influence post-transplant outcomes, including tumor burden, presence of extrahepatic disease (EHD), vascular invasion and tumor growth dynamics. In light of the limited efficacy of available systemic therapies, LTx should be considered the preferred treatment option in carefully selected patients with advanced HEHE. Nevertheless, long-term follow-up remains crucial to identify potential recurrence or complications.

References

Mehrabi, A., Kashfi, A., Fonouni, H., Schemmer, P., Schmied, B. M., Hallscheidt, P., et al. (2006). Primary malignant hepatic epithelioid hemangioendothelioma: A comprehensive review of the literature with emphasis on the surgical therapy. Cancer, 107(9), 2108–2121. https://doi.org/10.1002/cncr.22225

d’Annibale, M., Piovanello, P., Carlini, P., Del Nonno, F., Sciarretta, F., Rossi, M., et al. (2002). Epithelioid hemangioendothelioma of the liver: Case report and review of the literature. Transplantation Proceedings, 34(4), 1248–1251. https://doi.org/10.1016/S0041-1345(02)02751-3

Dogeas, E. (2023). Hepatic epithelioid hemangioendothelioma: Pitfalls in the treatment of a rare liver malignancy. Translational Gastroenterology and Hepatology, 8, Article 3. https://doi.org/10.21037/tgh-22-80

Thin, L. W., Wong, D. D., De Boer, B. W., Ferguson, J. M., Adams, L., Macquillan, G., et al. (2010). Hepatic epithelioid haemangioendothelioma: Challenges in diagnosis and management. Internal Medicine Journal, 40(10), 710–715. https://doi.org/10.1111/j.1445-5994.2009.02043.x

Studer, L. L., & Selby, D. M. (2018). Hepatic epithelioid hemangioendothelioma. Archives of Pathology & Laboratory Medicine, 142(2), 263–267. https://doi.org/10.5858/arpa.2016-0171-RS

Makhlouf, H. R., Ishak, K. G., & Goodman, Z. D. (1999). Epithelioid hemangioendothelioma of the liver: A clinicopathologic study of 137 cases. Cancer, 85(3), 562–582. https://doi.org/10.1002/(SICI)1097-0142(19990201)85:3%3C562::AID-CNCR7%3E3.0.CO;2-T

Ostojić, A., Mrzljak, A., & Mikulić, D. (2021). Liver transplantation for benign liver tumors. World Journal of Hepatology, 13(9), 1098–1106. https://doi.org/10.4254/wjh.v13.i9.1098

Grotz, T. E., Nagorney, D., Donohue, J., Que, F., Kendrick, M., Farnell, M., et al. (2010). Hepatic epithelioid haemangioendothelioma: Is transplantation the only treatment option? HPB, 12(8), 546–553. https://doi.org/10.1111/j.1477-2574.2010.00213.x

Serafin, A., Suchołbiak, A., Baran, K., Podolak, M., Pachulska, K., & Mikołajczyk, O. (2025). Hepatoepithelioid hemangioendothelioma (HEHE): A comprehensive review of pathogenesis, characteristic features, diagnostic strategies and treatment approaches with emphasis on surgical therapies. Qualitative Sport, 43, Article 62330. https://doi.org/10.12775/QS.2025.43.62330

Frenette, C., Mendiratta-Lala, M., Salgia, R., Wong, R. J., Sauer, B. G., & Pillai, A. (2024). ACG clinical guideline: Focal liver lesions. The American Journal of Gastroenterology, 119(7), 1235–1271. https://doi.org/10.14309/ajg.0000000000002857

Stacchiotti, S., Miah, A. B., Frezza, A. M., Messiou, C., Morosi, C., Caraceni, A., et al. (2021). Epithelioid hemangioendothelioma, an ultra-rare cancer: A consensus paper from the community of experts. ESMO Open, 6(3), Article 100170. https://doi.org/10.1016/j.esmoop.2021.100170

Kaltenmeier, C., Stacchiotti, S., Gronchi, A., Sapisochin, G., Liu, H., Ashwat, E., et al. (2022). Treatment modalities and long-term outcomes of hepatic hemangioendothelioma in the United States. HPB, 24(10), 1688–1696. https://doi.org/10.1016/j.hpb.2022.03.013

Dove, L., Chadha, R. M., Lai, J. C., DiMartini, A., Liapakis, A., Parikh, N. D., et al. (2025). AASLD AST practice guideline on adult liver transplantation: Candidate evaluation. Hepatology. Advance online publication. https://doi.org/10.1097/HEP.0000000000001644

Lerut, J. P., Orlando, G., Adam, R., Schiavo, M., Klempnauer, J., Mirza, D., et al. (2007). The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: Report of the European Liver Transplant Registry. Annals of Surgery, 246(6), 949–957. https://doi.org/10.1097/SLA.0b013e31815c2a70

Brahmbhatt, M., Prenner, S., & Bittermann, T. (2020). Liver transplantation for hepatic epithelioid hemangioendothelioma is facilitated by exception points with acceptable long-term outcomes. Transplantation, 104(6), 1187–1192. https://doi.org/10.1097/TP.0000000000002982

Larson, E. L., Ciftci, Y., Jenkins, R. T., Zhou, A. L., Ruck, J. M., & Philosophe, B. (2025). Outcomes of liver transplant for hepatic epithelioid hemangioendothelioma. Clinical Transplantation, 39(2), Article e70087. https://doi.org/10.1111/ctr.70087

Lai, Q., Feys, E., Karam, V., Adam, R., Klempnauer, J., Oliverius, M., et al. (2017). Hepatic epithelioid hemangioendothelioma and adult liver transplantation: Proposal for a prognostic score based on the analysis of the ELTR-ELITA registry. Transplantation, 101(3), 555–564. https://doi.org/10.1097/TP.0000000000001603

Krasnodębski, M., Grąt, M., Morawski, M., Wierzchowski, M., Jastrzębski, M., et al. (2020). Hepatic epithelioid hemangioendothelioma: A rare disease with favorable outcomes after liver transplantation. Transplantation Proceedings, 52(8), 2447–2449. https://doi.org/10.1016/j.transproceed.2020.02.101

Sawma, T., Sultan, A., Abdulmoneim, S., Grotz, T., Rosen, C. B., et al. (2024). Management and long-term outcomes of patients with hepatic epithelioid hemangioendothelioma. Journal of Surgical Oncology, 130(5), 1062–1069. https://doi.org/10.1002/jso.27807

Liu, X., Yang, P., Liu, L., Si, S., Zhou, R., Liu, T., et al. (2024). Long-term prognosis and treatment modalities of hepatic epithelioid hemangioendothelioma: A retrospective study of 228 patients. BMC Cancer, 24, Article 1285. https://doi.org/10.1186/s12885-024-13053-4

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Published

2026-06-30

How to Cite

Serafin, A., Suchołbiak, A., Baran, K. ., Podolak, M., Pachulska, K., Mikołajczyk, O., Grygutis, I., Jarzęcka, U., & Chętko, Łukasz . (2026). EVALUATION OF TREATMENT OUTCOMES AND PROGNOSTIC FACTORS IN PATIENTS AFTER LIVER TRANSPLANTATION DUE TO HEPATIC EPITHELIOID HEMANGIOENDOTHELIOMA. International Journal of Innovative Technologies in Social Science, 4(2(50). https://doi.org/10.31435/ijitss.2(50).2026.5757

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