Epithelioid haemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts
An international group of experts and patient advocates produced the first consensus on managing epithelioid haemangioendothelioma, including active surveillance for stable disease, surgery or transplant for localised disease, and sirolimus for progressing tumours.
Overview
Consensus paper from sarcoma experts and the EHE patient community covering diagnosis (including WWTR1-CAMTA1 and YAP1-TFE3 fusions), the highly variable natural history, indications for active surveillance, surgery, liver transplantation and radiotherapy, systemic therapy with mTOR inhibitors and anti-angiogenic agents, and priorities for research.
The observation-first approach and the sequencing of sirolimus before other systemic options on the EHE page come from this consensus.
- Based largely on retrospective series; no randomised trials exist.