Primary chondrosarcoma of the head and neck in pediatric patients - A clinicopathologic study of 14 cases with a review of the literature

BACKGROUND. Primary chondrosarcoma of the head and neck in the pediatric ag e group is rare. The literature contains several single cases and small ser ies; however, to the authors' knowledge, there has been no previous compreh ensive larger study to evaluate the clinicopathologic aspects of these tumo rs. METHODS, Fourteen cases of chondrosarcoma of the head and neck from patient s age 18 years or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces In stitute of Pathology. No secondary sarcomas (radiation-induced or arising i n association with Maffucci syndrome or Oilier disease) were included. Clin ical, radiographic, and histologic features were reviewed and patient follo w-up obtained. RESULTS. The patients included 6 girls and 8 boys ages 3-18 pears (mean, 11 .8 years). Patient symptoms (nasal stuffiness or discharge, sinusitis, head aches, or a mass lesion) were related to tumor location and were present fo r an average of 7.2 months. No genetic abnormalities were documented. The t umors most frequently involved the maxillary sinus (n=4), followed by the m andible (n=3), nasal cavity (n=2), and neck (n=2), with 1 each of the nasop harynx, orbit, and base of the skull. The tumors ranged in size from 2.0 to 15.0 cm (mean, 3.1 cm). All tumors were invasive and malignant as determin ed by radiology and/or histology. The tumors were Grade 1 (n=9), Grade 2 (n =1), or Grade 3 (mesenchymal, n=2; dedifferentiated n=2). All patients were treated by surgery, followed by radiation (n=5) and/or chemotherapy (n=2). Follow-up was available for 11 patients; all were alive (at a mean of 14.8 years), with only a single patient demonstrating evidence of residual/recu rrent tumor (at 16.6 years). CONCLUSIONS. Primary head and neck chondrosarcoma in the pediatric populati on is typically low grade and occurs in the maxillary sinus or mandible. De spite the invasive and high grade nature of some of these tumors, there is an excellent long term prognosis for patients in this age group with tumors in these locations. (C) 2000 American Cancer Society.