Sarcoma in Paget's disease (11 cases)

Eleven cases of sarcoma in Paget's disease of bone are presented, together with an extensive review of the literature. These neoplasms are rare but not exceptional, and mostly affect male subjects in the seventh decade of life. The sites most frequently affected are the pelvis, femur and humerus. In 30 per cent of cases these neoplasms are multifocal. Radiographically the lesions are nearly always osteolytic. The radiographic diagnosis may, however, be quite difficult, especially in the presence of the severe (but benign) osteolyic lesions that sometimes occur in Paget's disease. Morphologically these tumours are mostly highly polymorphic sarcomas. The cases in this series were diagnosed histologically as osteosarcoma, grade 3 fibrosarcoma, and malignant fibrous histiocytoma; but there are no prognostic differences between the various histological types. The prognosis is very serious, only about 3 per cent of patients surviving for five years from the time of diagnosis. Therapy, unfortunately nearly always palliative, is based on amputation or disarticulation after sections biopsy in cases where the disease is localised to the limbs, and on radiotherapy in cases not amenable to surgery.