title: Rhabdomyosarcoma of the larynx in children: a series of five patients treated in the Institut Gustave Roussy (Villejuif, France).
abstract: Rhabdomyosarcoma of the larynx is a malignant disease rarely seen in children. We present five cases (3 males, 2 females) observed among 126 patients with rhabdomyosarcoma of the head and neck (excluding the orbit) treated in the Institut Gustave-Roussy between 1955 and 1981. The patients were aged between 5 1/2 years and 13 1/2 years at the time of diagnosis. The presenting symptoms were dysphonia and/or dyspnea; tracheotomy was required in two cases. All five patients received chemotherapy consisting of vincristine, cyclophosphamide, actinomycin D + procarbazine or doxorubicin. Four patients also received radiotherapy (45 Gy during 5 weeks). Laryngectomy was not performed. Treatment sequelae included regional hypoplasia, huskiness, and subclinical thyroid insufficiency; two patients developed a benign thyroid adenoma. One patient presented a bilateral carotid stenosis subsequent to radiotherapy performed on "wide fields" portal designed to provided prophylactic treatment on uninvolved lymph nodes. All the patients are alive and relapse-free with a follow-up of between 13 and 17 years. This small series of RMS of the larynx shows that patients can be cured without laryngectomy, although long-term sequelae associated with radiotherapy are observed.
authors.affiliation: Department of Pediatrics, Hospital Erasto Gaertner Curitiba, Parana, Brazil.
authors.forename: M A
authors.initials: MA
authors.lastname: Kato
authors.forename: F
authors.initials: F
authors.lastname: Flamant
authors.forename: M J
authors.initials: MJ
authors.lastname: Terrier-Lacombe
authors.forename: J L
authors.initials: JL
authors.lastname: Habrand
authors.forename: G
authors.initials: G
authors.lastname: Schwaab
authors.forename: B
authors.initials: B
authors.lastname: Luboinski
authors.forename: D
authors.initials: D
authors.lastname: Valteau-Couanet
authors.forename: J
authors.initials: J
authors.lastname: Lemerle
mesh_terms: D000293:Adolescent; 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; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D007822:Laryngeal Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D008297:Male; D011879:Radiotherapy Dosage; D012208:Rhabdomyosarcoma / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide