Review Question - QID 1274

The radiograph, bone scan, and histology slide are consistent with Paget's sarcoma of the proximal humerus, correctly treated with chemotherapy, wide resection, and reconstruction. The bone scan shows this as a polyostotic disease where the proximal humerus sarcoma has developed from pagetoid bone. Pagetoid bone shows up very "hot" on bone scan, even without sarcomatous change. The histology slide shows a pleomorphic spindle cell sarcoma with many giant cells, large amount of cellular atypia, and high nuclear to cytoplasmic ratio, typical of Paget's sarcoma.

Harrington reviews the diagnosis, treatment, and outcome of patients with Paget's sarcoma. While this review emphasizes the use of amputation for Paget's sarcoma, limb salvage, when appropriate, is still the best choice to decrease patient morbidity. Histologically, Harrington discusses the difficulty in differentiation of Paget's sarcoma, metastatic carcinoma in pagetoid bone, and osteosarcoma and suggests that appropriate pathological training in sarcoma is necessary to make this diagnosis.

Moore et al studied 22 patients with Paget disease in order to better define which patients with Paget's disease are likely to develop these tumors and what radiologic findings confirm the diagnosis. They found that Paget's sarcomas can develop in any bone affected by the disease but are more likely to occur in patients with advanced disease. Radiographically, Paget's sarcoma typically presents as a destructive lesion without periosteal reaction.