Craniofacial osseous and chondromatous sarcomas in British Columbia--a review of 34 cases.

Abstract

Head and neck sarcomas are very rare diseases. The aim of this study was to assess the prevalence and presentation of hard tissue sarcomas in the head and neck and jaws. A search of the British Columbia Tumor Registry identified 34 craniofacial hard tissue sarcomas (11 chondrosarcomas, 23 osteosarcomas) over a period of 29 years. A male predominance (1.8:1) and a mean age of 40.4 years at diagnosis were seen. Of the 23 patients with osteosarcoma, one-third survived for 5 years and 12 (52%) died within a mean of 20 months. Of the 11 patients with chondrosarcoma, 45% survived for 5 years and two (18%) of the patients died within a mean of 6 months. In nine of the osteosarcoma patients (39%) the jaws were involved, and in six of those cases (67%) there was mandibular involvement. Swelling was the first sign in all jaw patients, with a mean age of 41.1 years at diagnosis and a male predominance with a ratio of 8:1. Three of the eight male patients with osteosarcoma of the jaw (38%) died within a mean of less than 1 year. Improved prognosis is related to early recognition and diagnosis to allow for adequate surgical resection. The benefit of a combination of surgery with irradiation or chemotherapy continues to be investigated.