Epithelioid hemangio-endothelioma (EHE) in NETSARC: The nationwide series of 267 patients over 12 years
Highlighting a study in ultra-rare sarcomas (sarcomas that occur in fewer than 1/1,000,000 people per year).
The study titled “Epithelioid hemangio-endothelioma (EHE) in NETSARC: The nationwide series of 267 patients over 12 years.” EHE is a rare sarcoma that is frequently metastatic at diagnosis. This study describes the outcome of 267 patients with EHE in the NETSARC+ network in France for 12 years beginning in 2010. The study indicates most patients (both localized and metastatic) progressed but were still alive at 10 years. The authors conclude that in the absence of a clinical trial, anthracyclines remain a reasonable first treatment option, but better second line treatments are needed. Results also indicate that a subset of localized and disseminated EHE are life threatening, but tools to identify these patients are not available and additional studies are needed to develop them.