Enchondromatosis – Maffucci Syndrome

  • Etiology: tumor of cartilage rests deposited in medullary cavity as growth plate moves away from diaphysis
  • Imaging: multiple enchondromas + hemangiomas / lymphangiomas, asymmetric involvement
  • Complications: chondrofibrosarcoma in 30%, associated with CNS / ovarian / pancreatic malignancy

Radiology Cases of Enchondromatosis – Maffucci Syndrome

Radiograph of multiple enchondromas of the hand in Maffucci syndrome
AP (left) and oblique (right) radiographs of the hand show, in the second / third / fourth phalanges, multiple metaphyseal lesions that are lytic with a narrow zone of transition and no periosteal reaction.