title: Primary osteosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 22 cases with a review of the literature.
abstract: BACKGROUND
  Primary osteosarcomas of the head and neck in the pediatric age group, not associated with previous irradiation or a known syndrome, are rare. The literature contains several single cases and small study series; however, to the authors's knowledge, there has been no comprehensive large study 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 retrieved 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 reviewed, and patient follow-up was obtained.
  
  
  RESULTS
  The patients included 11 girls and 11 boys, 1-18 years of age (mean, 12.2 yrs). Patient symptoms related to tumor location were painless swelling, loss of teeth, headaches, or a mass lesion, present for an average of 5.9 months. No genetic abnormalities were documented. The tumors most frequently involved the mandible (n = 19), followed by the sphenoid sinus (n = 2) and the maxilla (n = 1). The tumors ranged in size from 1.1-10.0 cm (mean, 4.5 cm). All 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). All cases, except one chondroblastic osteosarcoma, were osteoblastic osteosarcomas. 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 disease (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 disease (mean, 20.0 yrs).
  
  
  CONCLUSIONS
  Primary head and neck osteosarcomas in the pediatric population 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 excellent overall long-term prognosis for patients in this age group with tumors in these locations.
authors.affiliation: Department of Endocrine, Armed Forces Institute of Pathology, 6825 16th St NW, Washington, DC 20306-6000, USA.
authors.forename: S R
authors.initials: SR
authors.lastname: Gadwal
authors.forename: F H
authors.initials: FH
authors.lastname: Gannon
authors.forename: J C
authors.initials: JC
authors.lastname: Fanburg-Smith
authors.forename: E M
authors.initials: EM
authors.lastname: Becoskie
authors.forename: L D
authors.initials: LD
authors.lastname: Thompson
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D012516:Osteosarcoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D017063:Outcome Assessment, Health Care; D011859:Radiography
chemical_list: D000970:Antineoplastic Agents