[A case of Maffucci's syndrome]

A 14-year old girl had developed, at the age of 6, a swelling on the left shank. 4 years later, additional tumours appeared. 1990, at the age of 12, she had to be operated because of serious discomfort in the left ankle. The histological diagnosis was haemangioma plus enchondroma. At the age of 14 further haemangiomas developed and in early 1992 she had to undergo another operation of the left thigh. Histologically and immunohistochemically a Mafucci syndrome was diagnosed--a very rare affection belonging to the group of osteochondrodysplasia that usually goes with haemangioma and enchondroma. Radiological, scintigraphic and MTR findings are presented.