Modern multimodality therapy for pediatric nonorbital parameningeal sarcomas.
BACKGROUND
Nonorbital parameningeal sarcomas are rare, aggressive tumors that arise predominantly in the pediatric population. The purpose of this study was to review their management and to clarify the role of surgery in their treatment.


METHODS
Treatment, prognostic factors, and outcomes for patients over a 10-year period were reviewed.


RESULTS
In all, 34 patients were identified. The most common tumor site was the maxillary sinus, and 73% were rhabdomyosarcoma. Six patients (18%) presented with positive nodal metastases and 3 patients (10%) presented with distant metastases. Overall, 18 patients were treated surgically. Locoregional recurrence rate was 43%; no patients developed distant recurrence. Five-year overall survival was 66.8%, disease-specific survival was 68.1%, and disease-free survival was 31.9%.


CONCLUSIONS
Pediatric nonorbital parameningeal sarcomas require aggressive multimodality treatment. Although chemotherapy and radiation constitute the treatment of choice in most cases, surgery may have a role as salvage therapy and in cases that fail to respond to chemoradiation.
Zevallos
Jose P
JP
Department of Head and Neck Surgery, The University of Texas M. D. Anderson Cancer Center, Houston, Texas, USA.
Jain
Kunal
K
Roberts
Dianna
D
Santillan
Alfredo A
AA
Huh
Winston
W
Hanna
Ehab Y
EY
Kupferman
Michael E
ME
D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004844:Epistaxis / Q000209:etiology; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D009296:Nasal Cavity / Q000473:pathology / Q000601:surgery; D015508:Nasal Obstruction / Q000209:etiology; D009303:Nasopharyngeal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009364:Neoplasm Recurrence, Local; D009669:Nose Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012509:Sarcoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*