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- W2912011006 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." @default.
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- W2912011006 date "2019-02-20" @default.
- W2912011006 modified "2023-10-15" @default.
- W2912011006 title "Unsatisfactory response to sirolimus in Maffucci syndrome‐associated spindle cell hemangiomas" @default.
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- W2912011006 doi "https://doi.org/10.1111/dth.12851" @default.
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