PAGETS SARCOMA OF THE MANDIBLE

Background. Sarcomatous change occurs in less than 1% of patients with Paget's disease. It has been reported in only 9 patients in the mandi ble, and has been uniformly fatal. Comparison of Paget's sarcoma of th e mandible to osteosarcoma of the mandible and to osteosarcoma in othe r locations was made to attempt to explain and improve the outcome of patients with these tumors. Methods. Two 78-year-old women with Paget' s sarcoma of the mandible treated with radical resection are reported. The literature is reviewed to compare the clinical presentation and p rognosis of patients with mandibular Paget's sarcoma to patients with osteosarcoma in non-Pagetoid mandibles and with osteosarcoma outside o f the head and neck. Results, Both patients died within 2 years of lun g metastases. The patients with mandibular Paget's sarcoma were marked ly older, with an average age of 65.6 years, compared to 32.5 years fo r mandibular osteosarcoma. Paget's sarcoma of the mandible was uniform ly rapidly fatal; in non-Pagetoid mandibles the 5-year survival is 40% . There have been reports of only 7 patients who have survived with Pa get's sarcoma in other locations, In osteosarcoma of the extremities 5 -year disease-free survival exceeds 75% with multimodality therapy. Co nclusions, Paget's sarcoma of the mandible is a rare tumor which affec ts elderly patients, It progresses rapidly and has a much poorer progn osis than osteosarcoma occurring in a non-Pagetoid mandible. The progn osis may be improved with early detection and treatment with multimoda lity therapy. (C) 1995 John Wiley & Sons, Inc.