The benefit of everolimus in recurrent/epithelioid angiosarcoma patients: Case reports and literature review

Epithelioid angiosarcoma (EA) is a kind of rare malignant soft tissue sarcoma, with high recurrence/metastatic rate and poor prognosis. To date, no effective standardized treatment regimen has been available for patients with recurrence/metastatic EA. Everolimus is an oral rapamycin derivative that highly inhibits the mechanistic target of rapamycin(mTOR) signal pathway. Previous studies have suggested that everolimus is effective and safe in some soft tissue sarcoma. We reported two cases with recurrence/metastatic EA, who received everolimus after failure of surgery, radiotherapy, chemotherapy or interventional therapy. Two cases obtained clinical benefit within 1 week, and were evaluated as partial response (PR). The progression free survival (PFS) time was nearly 12.0 and 6.0 months, respectively. The overall survival (OS) time was 18.0 and 10.0 months, respectively. The main adverse event was stomatitis syndrome (grade 1-2), which was well controllable and tolerable. It indicated that everolimus may be more beneficial for recurrence/metastatic EA patients.