TY - JOUR
T1 - Primary osteosarcoma of the head and neck in pediatric patients
T2 - A clinicopathologic study of 22 cases with a review of the literature
AU - Gadwal, Shyam R.
AU - Gannon, Francis H.
AU - Fanburg-Smith, Julie
AU - Becoskie, Elizabeth M.
AU - Thompson, Lester D.R.
PY - 2001/2/1
Y1 - 2001/2/1
N2 - 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.
AB - 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.
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U2 - 10.1002/1097-0142(20010201)91:3<598::AID-CNCR1040>3.0.CO;2-D
DO - 10.1002/1097-0142(20010201)91:3<598::AID-CNCR1040>3.0.CO;2-D
M3 - Article
C2 - 11169944
AN - SCOPUS:0035253653
SN - 0008-543X
VL - 91
SP - 598
EP - 605
JO - Cancer
JF - Cancer
IS - 3
ER -