Paget's sarcoma of the mandible.

ABSTRACT

BACKGROUND: Sarcomatous change occurs in less than 1% of patients with Paget's disease. It has been reported in only 9 patients in the mandible, and has been uniformly fatal. Comparison of Paget's sarcoma of the mandible to osteosarcoma of the mandible and to osteosarcoma in other 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 prognosis of patients with mandibular Paget's sarcoma to patients with osteosarcoma in non-Pagetoid mandibles and with osteosarcoma outside of the head and neck. RESULTS: Both patients died within 2 years of lung metastases. The patients with mandibular Paget's sarcoma were markedly older, with an average age of 65.6 years, compared to 32.5 years for mandibular osteosarcoma. Paget's sarcoma of the mandible was uniformly rapidly fatal; in non-Pagetoid mandibles the 5-year survival is 40%. There have been reports of only 7 patients who have survived with Paget's sarcoma in other locations. In osteosarcoma of the extremities 5-year disease-free survival exceeds 75% with multimodality therapy. CONCLUSIONS: Paget's sarcoma of the mandible is a rare tumor which affects elderly patients. It progresses rapidly and has a much poorer prognosis than osteosarcoma occurring in a non-Pagetoid mandible. The prognosis may be improved with early detection and treatment with multimodality therapy.