Primary osteosarcoma of the head and neck in pediatric patients - A clinicopathologic study of 22 cases with a review of the literature

BACKGROUND. Primary osteosarcomas of the head and neck in the pediatric age group, not associated with previous irradiation or a known syndrome, are r are. The literature contains several single cases and small study series; h owever, to the authors's knowledge, there has been no comprehensive large s tudy to evaluate the clinicopathologic aspects of these tumors. METHODS. Twenty-two cases of osteosarcomas of the head and neck in patients 18 years of age or younger, diagnosed between 1970 and 1997, were retrieve d from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology (AFIP). No secondary sarcomas (radiation-induced or those arising after chemotherapy) or those associated with known syndromes were included. Clinical, radiographic, and histologic features were reviewe d, and patient follow-up was obtained. RESULTS. The patients included 11 girls and 11 boys, 1-18 years of age (mea n, 12.2 yrs). Patient symptoms related to tumor location were painless swel ling, loss of teeth, headaches, or a lesion, present for an average of 5.9 months. No genetic abnormalities were documented. The tumors most frequentl y involved the mandible (n = 19), followed by the sphenoid sinus (n = 2) an d the maxilla (n = 1). The tumors ranged in size from 1.1-10.0 cm (mean, 4. 5 cm). AU tumors were invasive and malignant by radiology and/or histology. The tumors were Grade 1 (n = 11), Grade 2 (n = 8), or Grade 3 (n = 3). AU cases, except one chondroblastic osteosarcoma, were osteoblastic osteosarco mas. Thirteen patients underwent initial surgical resection with (n = 5) or without (n = 9) additional radiation and/or chemotherapy. The remaining 9 patients had an initial biopsy for diagnosis followed by surgery (n = 4) or surgery and radiation and/or chemotherapy (n = 5). Follow-up was available for 19 patients: 13 were alive at last follow-up with no evidence of disea se (mean, 13.1 yrs); 1 was alive with disease (1.3 yrs); 3 had died without evidence of disease (mean, 23.2 yrs); and 2 had died of disease (mean, 7.8 yrs). The 3 patients with high-grade osteosarcoma were alive without disea se (mean, 20.0 yrs). CONCLUSIONS, Primary head and neck osteosarcomas in the pediatric populatio n are typically low- to moderate-grade lesions in the mandible. Despite the invasive nature and high grade of a few of these tumors, there is an excel lent overall long-term prognosis for patients in this age group with tumors in these locations. Cancer 2001;91:598-605. Published 2001 by the American Cancer Society.*.