Maffucci syndrome with unilateral limb: a case report and review of the literature

作者:Gao Hua; Wang Baojun; Zhang Xi; Liu Fengqi; Lu Ying*
来源:Chinese Journal of Cancer Research, 2013, 25(2): 254-258.
DOI:10.3978/j.issn.1000-9604.2013.03.05

摘要

Maffucci syndrome is a congenital, non-hereditary mesodermal dysplasia manifested by multiple enchondromas and hemangiomas. It is associated with diverse secondary musculoskeletal deformities, which is exceedingly rare. We report a case of hemangiomas and enchondromas localized in the unilateral limb in a patient with Maffucci syndrome. Treatment consists of orthopedic and surgical intervention to minimize deformities and for cosmetic purpose. Careful surveillance for malignant degeneration of both skeletal and non-skeletal tumors, especially in the brain and abdomen, is essential.

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