Kato M A, Flamant F, Terrier-Lacombe M J, Habrand J L, Schwaab G, Luboinski B, Valteau-Couanet D, Lemerle J
Department of Pediatrics, Hospital Erasto Gaertner Curitiba, Parana, Brazil.
Med Pediatr Oncol. 1991;19(2):110-4. doi: 10.1002/mpo.2950190208.
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.
喉横纹肌肉瘤是一种在儿童中罕见的恶性疾病。我们报告了1955年至1981年间在古斯塔夫 - 鲁西研究所接受治疗的126例头颈部（不包括眼眶）横纹肌肉瘤患者中观察到的5例（3例男性，2例女性）。诊断时患者年龄在5岁半至13岁半之间。主要症状为声音嘶哑和/或呼吸困难；2例患者需要进行气管切开术。所有5例患者均接受了由长春新碱、环磷酰胺、放线菌素D + 丙卡巴肼或阿霉素组成的化疗。4例患者还接受了放疗（5周内45戈瑞）。未进行喉切除术。治疗后遗症包括局部发育不全、声音沙哑和亚临床甲状腺功能不全；2例患者发生了良性甲状腺腺瘤。1例患者在对“大野”照射野进行放疗后出现双侧颈动脉狭窄，该照射野旨在对未受累淋巴结进行预防性治疗。所有患者均存活且无复发，随访时间为13至17年。这一小系列喉横纹肌肉瘤病例表明，尽管观察到与放疗相关的长期后遗症，但患者无需进行喉切除术即可治愈。
