Osteosarcoma in enchondromatosis (Ollier's disease). Report of a case.

or Ollier's disease, is best called enchondromatosis, and was defined by Jaffe (1958) as the presence of either circumscribed foci or large masses of cartilage in the interior of bones. It may be confined to limb bones and often almost to one side of the body, as in the patients described by Ollier in 1900. The possibility of malignant change in a focus of enchondromatosis was considered to be rare by Fairbank (1948), whereas Jaffe (1958) found that chondrosarcomata developed in half his cases, and bore no relation to the severity of the enchondromatosis. Dahlin and Henderson (1956) and Dahlin (1957), although finding chondrosarcoma a common complication of multiple chondromata, stated that chondrosarcoma secondary to a proven benign enchondroma did not occur in their series. Murray and Cruickshank (1960) thought that malignancy did not occur unless the enchondromatosis was associated with cutaneous haemangiomata (Maffucci's syndrome). referred to the possible development of osteosarcoma. The following case report describes an osteosarcoma developing in a man of sixty-eight who suffered from enchondromatosis. CASE REPORT The patient, a man aged sixty-eight, complained of an aching pain over the outer aspect of the left hip, present for three weeks. He said that a swelling had been excised from the right leg when he was sixteen years old, but apart from this there was no relevant history. On examination he was in apparently good general health. There was left genu varum of 20 degrees and a bony swelling, thought to be an exostosis, was visible and palpable on the upper third of the left tibia. There was faint brown pigmentation on the outer aspect of the left thigh. There was tenderness over the left greater trochanter, and, although movement at the left hip was full, forced flexion caused pain in the groin. There was three-quarters of an inch of shortening in the left lower limb. The other systems were normal. Radiographic examination-Unusual bony changes were present in the left femur, tibia, fibula and right fourth metatarsal : they took the form of areas of focal calcification, sometimes with alteration of the texture and outline of the surrounding bone (Fig. 1), and were interpreted as lesions of enchondromatosis. There was an area of increased density in the neck of the left femur above the lesser trochanter, and adjacent to it was a soft-tissue mass containing flecks ofcalcification (Fig. 2) : this was interpreted as a malignant tumour, …