Lenalidomide stops progression of multifocal epithelioid hemangioendothelioma including intracranial disease

Abstract

Epithelioid Hemangioendothelioma (EH) is a rare soft-tissue tumor which may present as an isolated tumor or can spread to affect internal organs. The course of EH varies, based on the tissue of origin. This case report describes a young woman who developed cutaneous EH with concurrent intracranial disease during pregnancy. After resection, the lesions returned. Even after several courses of chemotherapy and radiotherapy, the patient developed multifocal disease including pulmonary, skeletal, and liver disease. She now exhibits stable disease after approximately 6 years of therapy with lenalidomide.

Malignant Epithelioid Hemangioendothelioma of the Liver Successfully Treated with Sorafenib

“…They found positivity in 6 out of 6 samples of HEH. 21 This result, together with the efficacy shown by interferon and other antiangiogenic agents, 6–8 such as thalidomide and lenalidomide, 22–24 in patients with advanced HEH supports the role of angiogenic pathways as possible targets in patients with HEH. Thus, the initiation of the treatment with the antiangiogenic agent sorafenib was supported by this background.…”

Successful treatment of metastatic hepatic epithelioid hemangioendothelioma with thalidomide: a case report

“…In contrast to an earlier report [11], we did not find any major reduction in disease bulk with thalidomide treatment; however, our patient has remained clinically and radiologically stable over a period in excess of seven years. More recently, the case of a patient treated with lenalidomide, a derivative of thalidomide, has been described, reporting long-term benefit in a patient with HEH progressing after earlier chemotherapy treatment [13]. …”

Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents

“…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 . 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 .…”

“…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. [49][50][51][52][53][54] Finally, the multityrosine kinase inhibitor pazopanib achieved one PR and one SD in two cases reported.…”