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Journal of the Korean Society for Surgery of the Hand 2000;5(1):132-136.
Published online January 1, 2000.
Maffucci's Syndrome -A Case Report-
Joon-Min Song, Sai-Won Kwon, Dae-Joong Kim
Maffucci 증후군 -증례보고-
송준민, 권세원, 김대중
Abstract
First described by Angelo Maffucci in 1881, Maffucci's syndrome is a disease that accompanies multiple enchondromatosis and hemangiomas caused by mesodermal dysplasia, often detected before the patient reaches the puberty in his/her long bones, most commonly penetrating metacarpal and phalangeal bones of the patients hand on both sides. X-ray is by far the fastest way to detect Maffucci's syndrome and tissue biopsy is required for confirmation of disease. Deformity caused by enchondroma is dealt with a surgical procedure, while hemangioma is treated with chemotherapy, radiotherapy, steroid injection and/or simple excision. Having experienced a case that developed into malignant chondrosarcoma, on which we performed excision and biopsy, we hereby report the case with relevant documents.
 


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