Sorafenib in patients with progressive epithelioid hemangioendothelioma

Abstract

BACKGROUND There is no standard treatment for progressive epithelioid hemangioendothelioma (EHE). To investigate the significant vascularization of EHE, the activity/toxicity of sorafenib in patients with progressive EHE was explored. METHODS In this multicenter, 1‐stage, phase 2 trial of sorafenib (800 mg daily), the primary endpoint, which was chosen by default, was the 9‐month progression‐free rate. All patients had documented progressive disease at the time of study entry. RESULTS Fifteen patients were enrolled between June 2009 and February 2011. The median age was 57 years (range, 31‐76 years), and the ratio of men to women was 9:6. The performance status was zero in 10 patients and 1 in 5 patients. Twelve patients had metastases, mainly in the lung (12 patients), liver (5 patients), and bone (3 patients). Five patients had received prior chemotherapy (doxorubicin in 5 patients and taxane in 3 patients). The median sorafenib treatment duration was 124 days (range, from 27 to >271 days). Seven patients required dose reductions or transient treatment discontinuation. The 9‐month progression‐free rate was 30.7% (4 of 13 patients). The 2‐month, 4‐month, and 6‐month progression‐free rate was 84.6% (11 of 13 patients), 46.4% (6 of 13 patients), and 38.4% (5 of 13 patients), respectively. Two partial responses were observed that lasted 2 months and 9 months. CONCLUSIONS Further clinical trials exploring sorafenib as treatment of progressive EHE are needed. Cancer 2013;119:2639–2644. © 2013 American Cancer Society.

The Feature of Solitary Small Nodular Type of Hepatic Epithelioid Hemangioendothelioma

“…Sorafenib is a therapeutic option for unresectable HEHE. It is a tyrosine kinase inhibitor associated with this signaling pathway [19]. It is reported that, with the administration of thalidomide, the progression of diffuse metastatic HEHE is hindered successfully [20, 21].…”

Highly aggresive pulmonary epithelioid hemangioendothelioma: a case report

“…Así, una mujer de 72 años con un HEE pulmonar metastásico en progresión a tratamiento de paclitaxel concomitante con radioterapia, presentó una respuesta parcial a sunitinib, seguida de estabilización de la enfermedad en un TC realizado diez meses después 6 . El Grupo Francés de Sarcoma publicó un estudio fase II en el que dos de quince pacientes respondieron a sorafenib y cinco experimentaron enfermedad estable 16 . En cuanto a pazopanib, recientemente se ha comunicado que una paciente de 62 años con enfermedad metastásica pulmonar consiguió una respuesta prolongada 17 , y lo mismo fue relatado en relación con una paciente con un HEE hepático con diseminación pulmonar, superando en este caso los diez años de supervivencia libre de progresión 18 .…”

A trimodality approach in the management of metastatic low-grade epithelioid hemangioendothelioma of the bone

“…Chevreau et al 20 studied 15 patients, who were not candidates for curative surgery and radiotherapy, who received sorafenib. The 9-month progression-free rate was 30.7% (4 of 13 patients).…”