title: Rhabdomyosarcoma of the head and neck in children.
abstract: Twenty-two children, 9 male and 13 female, with a non-orbital rhabdomyosarcoma of the head and neck, treated between 1970 and 1988, have been reviewed. Since 1972, treatment has consisted of combination chemotherapy, and where necessary radiotherapy and/or surgery. Complete clinical remission after initial chemotherapy was observed in 21 children. Six children were cured after primary treatment but 15 developed recurrent disease. Thirteen children had a parameningeal localized tumour, with eventual meningeal involvement in 5. Survival in this group was worse than in the non-meningeal group. Lymph node metastasis at first presentation (5 patients) had no influence on prognosis, whereas development of lymph node metastases during follow-up resulted in 100% mortality. All patients were retrospectively classified according to both the IRS-classification and TNM-descriptive system. No correlation with either system could be established. Fourteen of 15 children with recurrent disease were treated, 4 of whom were cured. Thus, 10 out of 22 (45%) children were long-term survivors.
authors.affiliation: Department of ENT, The Netherlands Cancer Institute, Amsterdam.
authors.forename: I J
authors.initials: IJ
authors.lastname: Coene
authors.forename: P F
authors.initials: PF
authors.lastname: Schouwenburg
authors.forename: P A
authors.initials: PA
authors.lastname: Voûte
authors.forename: J
authors.initials: J
authors.lastname: Marion
authors.forename: V
authors.initials: V
authors.lastname: Burgers
authors.forename: F J
authors.initials: FJ
authors.lastname: Hilgers
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality* / Q000628:therapy; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000628:therapy; D009426:Netherlands / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000401:mortality* / Q000628:therapy; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide; D007069:Ifosfamide