Primary osteosarcoma of the head and neck in pediatric patients

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. Cancer 2001;91:598–605. © 2001 American Cancer Society.

Telangiectatic osteosarcoma affecting the mandible

“…Osteosarcoma of the jaw bones are quite rare with an incidence of 1.5-7% of all head and neck primary tumors. [ 9 ] TOS was first described by Paget in the year 1854. [ 7 ] Originally it was considered to be a malignant bone aneurysm.…”

“…[ 5 ] In pediatric patients, OS accounts for less than 1% of all head and neck tumors. [ 9 ] and while TOS alone is considered it is still very rare. Few reports suggest that OS in jaw affects more males than females and few other reports suggest that there is no sex predilection.…”

Osteosarcoma: A Comparison of Jaw versus Nonjaw Localizations and Review of the Literature

“…It is further claimed that tumors in mandible and maxilla are less malignant as based on more often occurrence of low malignant histology and in particular on clinical outcome, that is, a better event, and overall prognosis, and lower incidence of metastatic spread as compared with osteosarcomas arising elsewhere in the body. Metastatic spread at initial presentation up to 16% is reported [3, 7, 11–16]. In this paper the nationwide data on osteosarcomas, as registered by the Netherlands, Cancer Registry of the Comprehensive Cancer Centers of the Netherlands are described.…”

Osteosarcoma of the Maxilla: A Rare Case with Unusual Clinical Presentation

“…Head and neck OSs have lower tendency to distant metastasis than their long bone counterparts, and they have a better 5-year survival rate. 4-6 …”