<Tr> <Th> Specialty </Th> <Td> medical genetics </Td> </Tr> <Tr> <Td_colspan="2"> (edit on Wikidata) </Td> </Tr> <P> Maffucci syndrome is a sporadic disease characterized by the presence of multiple enchondromas associated with multiple hemangiomas . Also lymphangiomas may be apparent . </P> <P> Patients are normal at birth and the syndrome manifests during childhood and puberty . The enchondromas affect the extremities and their distribution is asymmetrical . The most common sites of enchondromas are the metacarpal bones and phalanges of the hands . The feet are less commonly afflicted . Disfigurations of the extremities are a result . Pathological fractures can arise in affected metaphyses and diaphyses of the long bones and are common (26%). The risk for sarcomatous degeneration of enchondromas, hemangiomas, or lymphangiomas is 15 - 30% in the setting of Maffucci syndrome . Maffucci syndrome is associated with a higher risk of CNS, pancreatic, and ovarian malignancies . Multiple enchondromas may present in 3 disorders: Ollier disease, Maffucci syndrome, and metachondromatosis . It is important to make the distinction between these diseases, particularly Ollier disease and Maffucci syndrome . Ollier disease is more common than Maffucci syndrome, and presents with multiple enchondromas often in a unilateral distribution . However, hemangiomas and lymphangiomas are not seen in Ollier disease . Metachondromatosis demonstrates autosomal - dominant transmission and presents with both multiple osteochondromas and enchondromas . </P>

Multiple enchondromatosis (ollier's and maffucci's syndrome) pictures