Maffucci Syndrome

12-year-old male with history of fetal alcohol syndrome and dermal vascular abnormalities. There is a T1 isointense to skeletal muscle, T2 heterogeneous hyper- and isointense lesion with lobular margins as well as a thin peripheral rim of sclerosis in the proximal left humerus. No diffusion restriction or loss of signal on out of phase images. A single postcontrast fat-saturated image through the proximal right hand demonstrates a serpiginous enhancing soft tissue lesion at the first webspace. These imaging findings are compatible with Maffucci syndrome. Maffucci syndrome is characterized by multiple enchondromas with soft tissue hemangiomas. Male patients are more commonly affected. Clinical symptoms are typically present birth. On physical examination hemangiomas are typically recognized as blue subcutaneous nodules. Enchondromas most commonly affected the hands and feet. The osseous and soft tissue vascular abnormalities are usually asymmetric. There is a risk of degeneration of the enchondromas to chondrosarcoma and soft tissue hemangiomas to vascular sarcomas.