title: Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature.
abstract: BACKGROUND
  Primary chondrosarcoma of the head and neck in the pediatric age group is rare. The literature contains several single cases and small series; however, to the authors' knowledge, there has been no previous comprehensive larger study to evaluate the clinicopathologic aspects of these tumors.
  
  
  METHODS
  Fourteen cases of chondrosarcoma of the head and neck from patients age 18 years or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. No secondary sarcomas (radiation-induced or arising in association with Maffucci syndrome or Ollier disease) were included. Clinical, radiographic, and histologic features were reviewed and patient follow-up obtained.
  
  
  RESULTS
  The patients included 6 girls and 8 boys ages 3-18 years (mean, 11.8 years). Patient symptoms (nasal stuffiness or discharge, sinusitis, headaches, or a mass lesion) were related to tumor location and were present for an average of 7.2 months. No genetic abnormalities were documented. The tumors most frequently involved the maxillary sinus (n=4), followed by the mandible (n=3), nasal cavity (n=2), and neck (n=2), with 1 each of the nasopharynx, 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 determined 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/ recurrent tumor (at 16.6 years).
  
  
  CONCLUSIONS
  Primary head and neck chondrosarcoma in the pediatric population is typically low grade and occurs in the maxillary sinus or mandible. Despite 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.
authors.affiliation: Department of Orthopedic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
authors.forename: S R
authors.initials: SR
authors.lastname: Gadwal
authors.forename: J C
authors.initials: JC
authors.lastname: Fanburg-Smith
authors.forename: F H
authors.initials: FH
authors.lastname: Gannon
authors.forename: L D
authors.initials: LD
authors.lastname: Thompson
mesh_terms: D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D002813:Chondrosarcoma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D006261:Headache / Q000175:diagnosis; D006801:Humans; D008297:Male; D008339:Mandibular Neoplasms / Q000473:pathology; D008444:Maxillary Sinus Neoplasms / Q000473:pathology; D015508:Nasal Obstruction / Q000175:diagnosis; D009361:Neoplasm Invasiveness; D009669:Nose Neoplasms / Q000473:pathology; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012220:Rhinitis / Q000175:diagnosis; D012852:Sinusitis / Q000175:diagnosis