Treatment of Hepatic Epithelioid Hemangioendothelioma: Finding Uses for Thalidomide in a New Era of Medicine

Abstract

Hepatic epithelioid hemangioendothelioma (HEH) is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Our case involved a 49-year-old female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing HEH. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized. Resection is only an option in 10% of the cases; therefore, chemotherapy is the only line of treatment. Newer chemotherapy alternatives are targeting angiogenesis via the vascular endothelial growth factor. Thalidomide was first used as an antiemetic, but, sadly, soon linked to phocomelia birth defects. Given the mechanism of action against angiogenesis, thalidomide has a valid role in vascular tumors. In conclusion, the use of thalidomide as chemotherapy is novel and promising, especially in the setting of a rare vascular liver tumor such as HEH.

Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents

“…Additionally, several case reports documented short‐term control of hepatic and/or pulmonary EHE by newer agents. Treatment with thalidomide achieved a partial response (PR) in two and SD in one of six cases reported; three patients progressed on thalidomide . The use of the thalidomide derivative lenalidomide resulted in SD in three of three cases reported .…”

“…16 These observations are consistent with the generally held notion that conventional/metronomic chemotherapy results in occasional reductions in tumor mass, but does not play a major role in disease control. 2,36,38 18,[41][42][43][44][45] The use of the thalidomide derivative lenalidomide resulted in SD in three of three cases reported. [46][47][48] The pleiotropic cytokine interferon-alpha yielded a CR in one, PR in one, and SD in two of six cases reported; two patients progressed on interferon-alpha.…”

<p>Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising?</p>

“…[23][24][25][26][27][28] Targeting angiogenesis with bevacizumab, interferon alpha 2b, thalidomide and lenalidomide revealed contradictory results. [29][30][31][32][33] Case reports using metronomic cyclophosphamide and sirolimus have also been published. 34,35 The largest cohort of patients reported includes 32 patients from the Royal Marsden Hospital, London, UK, and depicts the heterogeneous therapeutic approach of patients with these rare tumors ranging from observation to systemic treatment including cytotoxic regimens and targeted therapies.…”

Molecular characterization of hepatic epithelioid hemangioendothelioma reveals alterations in various genes involved in DNA repair, epigenetic regulation, signaling pathways, and cell cycle control

“…Therefore, clinicians need to focus on other therapeutic options. Until now, reliable options for target therapy approaches in larger patient cohorts do not exist although case reports 28,29 or smaller cohort studies 17 have been published especially on the successful usage of mTOR inhibitors. 30 In accordance to COSMIC several of the mutations found in our cohort might have the potential for targeted therapy but it is difficult to predict whether targeting these mutations might be an option for a larger cohort of patients.…”