title: Soft-tissue sarcomas of the head and neck in children.
abstract: Thirty-two children aged three months to 17 years (median six years) were diagnosed with soft-tissue sarcoma of the head and neck and treated at the Children's Hospital of Philadelphia and the Hospital of the University of Pennsylvania from 1971 to 1981. Thirty-one received chemotherapy and all received radiation therapy (RT). Twenty-five patients had pre-treatment computed tomography (CT) scans, which were used for staging and treatment planning. Doses of radiation therapy ranged from 3000 to 7300 rad to the primary tumor (median 5000 rad). The overall five-year survival of the entire group of 32 patients was 75%. Ten of the 32 patients had invasive cranial parameningeal disease as demonstrated by bony erosion at the skull base, seen on CT in eight and plain radiographs in two patients. Eight of these 10 patients have developed recurrent sarcoma: four in the meninges, two locally, one regionally and one distantly. Five of these 10 children with invasive cranial parameningeal sarcoma received 3000 rad of prophylactic cranial irradiation, begun within the first 12 days of chemotherapy, and none developed meningeal disease. In contrast, only one of the 22 patients without invasive cranial parameningeal disease has relapsed (local recurrence). The data suggest that soft-tissue sarcomas of the head and neck in children without invasion into the base of the skull (invasive cranial parameningeal disease) are usually cured. CT scans are essential for staging. Patients with invasion of the base of the skull may be protected from meningeal relapse by early cranial irradiation, although they still are at high risk for relapse in other sites.
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005260:Female; D005354:Fibrosarcoma / Q000532:radiotherapy; D006258:Head and Neck Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D018219:Histiocytoma, Benign Fibrous / Q000532:radiotherapy; D006801:Humans; D007223:Infant; D008297:Male; D009455:Neurofibroma / Q000532:radiotherapy; D010414:Pennsylvania; D012208:Rhabdomyosarcoma / Q000532:radiotherapy; D012509:Sarcoma / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*