consists of polyostotic fibrous dysplasia, cafe'-au-lait spots of "coast of Maine" variety, and premature menses;
it is more common in girls than boys and accompanies approx 1 in 30 cases of polyostotic fibrous dysplasia;
there is appreciable coincidence of other endocrinologic dz including hyperthyroidism & Cushing's dz as well as hyperparathyroidism and hypophosphatemic rickets;
in polyostotic cases of fibrous dysplasia, dx is usually not difficult;
in monostotic cases, the radiographs are frequently not diagnostic and biopsy may be required;
malignant transformation is seen in less than 0.5%, with osteogenic sarcoma being the most common neoplasm
