Multicentric Non-monomelic Synchronous Chondrosarcoma Secondary to Maffucci Syndrome: Approach to Diagnosis and Treatment

Introduction Maffucci’s syndrome is rare, congenital, non hereditary mesodermal dysplasia clinically presents as enchondromas and hemangiomas. Maffucci syndrome was first described in 1881 and more than 200 cases are reported since then [1]. It has unknown etiology and no predilection for sex or race. Enchondromas are benign cartilaginous tumors most commonly seen in phalanges and long bones. Hemangiomas are most frequently located in the dermis and subcutaneous fat adjacent to areas of enchondromatosis. The development of lesions usually occurs in puberty. It is associated with secondary musculoskeletal deformities and rarely sarcomatous transformation. Complications are fracture, sarcomatous degeneration or chondrosarcomatous transformation. The risk of spontaneous fracture through area of advanced rarefaction in 25-30% and sarcomatous transformation is about 15-30%. Chondrosarcomatous transformation occurs in approximately 40-50% of enchondromas. We present a rare case of non monomelic synchronous developement of chondrosarcoma in a case of Maffucci syndrome. We have stressed on the importance of careful surveillance for malignant degeneration of both skeletal and non-skeletal lesions.