Maffucci syndrome (MS) is a very rare, non-hereditary dysplasia, manifested by multiple enchondromas and haemangiomas. Malignant transformation of these lesions is seen in up to 33% of the cases. There is also a very rare association with secondary musculoskeletal deformaties. We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the scapula. Treatment consisted of surgical resection. Because of the low grade of the tumor (G1), additional treatment radiotherapy/chemotherapy), was not necessary. Maffucci syndrome is an extremely rare mesodermal dysplasia. Malignant transformation of the associated enchondromas is common (33%) and should be considered whenever a change of the clinical course occurs. Clinical, as well as imaging follow-up (MRI) is required because of the relatively high rate of malignant degeneration of the skeletal lesions. However, random, X-ray examinations give little additional information on malignant transformation and are considered useless.
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