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In patients with progressing epithelioid haemangioendothelioma, the mTOR inhibitor sirolimus stabilised the disease in most, with a median progression-free survival of about a year, but did not help patients who had developed serosal effusions.
Retrospective case series of 38 patients with progressive epithelioid haemangioendothelioma treated with sirolimus within the Italian Rare Cancer Network.
Best response was partial response in 11 percent and stable disease in 71 percent, with median progression-free survival of 13 months and median overall survival of 19 months in patients with serosal effusions against not reached in those without.
Sirolimus is the first-choice systemic therapy for progressing EHE, ideally started before pleural or peritoneal effusions develop.