Published online Jun 14, 2014. doi: 10.3748/wjg.v20.i22.7049
Revised: November 29, 2013
Accepted: January 19, 2014
Published online: June 14, 2014
Epithelioid hemangioendothelioma (EH) is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs. The most common visceral site is the liver, where it is often involved in a multifocal manner known as hepatic EH (HEH). Surgical resection with curative intent represents the gold standard therapy. When surgery is not feasible, or in cases of metastatic disease, no standard medical treatment is currently indicated. In small series, drugs with anti-angiogenic activity (such as bevacizumab, sorafenib, thalidomide, and lenalidomide) have been proposed with promising results. We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide. Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months. The patient was treated for a total of 39 mo with prolonged disease stabilization and, at the time of writing, is still under treatment with a good tolerance profile. During a short period of treatment discontinuation, the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide. Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities. This preliminary result merits further study in a large series.
Core tip: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor. Currently, no standard medical treatment is indicated in unresectable disease. We describe the case of a patient with HEH who obtained stable disease after 40 mo of treatment with lenalidomide. Tolerance to treatment was very good. This report highlights the possibility of obtaining very long stable disease with an anti-angiogenesis drug. In recent years, the introduction of angiogenesis inhibitors has offered an interesting medical approach to this rare tumor.