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  • Title: Unsatisfactory response to sirolimus in Maffucci syndrome-associated spindle cell hemangiomas.
    Author: Gupta V, Mridha AR, Khaitan BK.
    Journal: Dermatol Ther; 2019 May; 32(3):e12851. PubMed ID: 30724000.
    Abstract:
    Maffucci syndrome is characterized by multiple benign vascular anomalies and enchondromas present on the distal extremities. Effective treatment options are currently not available for Maffucci syndrome-associated vascular lesions. Sirolimus is a mTOR pathway inhibitor, and has been tried successfully in the treatment of various vascular anomalies. We treated a 23-year-old female with Maffucci syndrome-associated spindle cell hemangiomas with oral sirolimus (2mg/day, 0.04mg/kg/day). There was improvement in pain, but no change in colour or size of the vascular nodules. In view of unsatisfactory response and treatment-related adverse effects (oral aphthae, mild transaminitis), sirolimus was stopped after 6 months.
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