Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.

ABSTRACT: Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the Europ

SUBMITTER: Stacchiotti S 

PROVIDER: S-EPMC8182432 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature