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睾丸旁横纹肌肉瘤:多模态治疗的延迟效应及对当前管理的影响

Paratesticular rhabdomyosarcoma: delayed effects of multimodality therapy and implications for current management.

作者信息

Hughes L L, Baruzzi M J, Ribeiro R C, Ayers G D, Rao B, Parham D M, Pratt C B, Kun L E

机构信息

Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38101.

出版信息

Cancer. 1994 Jan 15;73(2):476-82. doi: 10.1002/1097-0142(19940115)73:2<476::aid-cncr2820730237>3.0.co;2-n.

Abstract

BACKGROUND

The combined modalities of surgery, chemotherapy, and radiation therapy have greatly improved the survival rate in childhood paratesticular rhabdomyosarcoma, but the incidence of complications and late side effects is a cause for concern.

METHODS

We reviewed the records of 18 patients treated for paratesticular rhabdomyosarcoma at St. Jude Children's Research Hospital between 1962 and 1989. Patients with Group I disease were treated with orchiectomy, retroperitoneal lymph node dissection, and multi-agent chemotherapy; more advanced cases also received radiation therapy with concurrent chemotherapy.

RESULTS

Sequelae included esophageal and common bile duct stricture, inguinal nerve entrapment syndrome, and small bowel obstruction. Short stature was found in all children whose spines were irradiated via para-aortic fields (34-37 Gy) prior to puberty. Two of 18 patients died from treatment complications and one from progressive disease.

CONCLUSIONS

Multimodality treatment offers an excellent prognosis in paratesticular rhabdomyosarcoma, but is associated with significant morbidity and mortality rates. A discussion of therapy components and their application to disease stages suggests possible approaches to optimizing treatment for this therapy-sensitive malignancy.

摘要

背景

手术、化疗和放疗相结合的治疗方式极大地提高了儿童睾丸旁横纹肌肉瘤的生存率,但并发症和晚期副作用的发生率令人担忧。

方法

我们回顾了1962年至1989年间在圣裘德儿童研究医院接受睾丸旁横纹肌肉瘤治疗的18例患者的记录。I组疾病患者接受睾丸切除术、腹膜后淋巴结清扫术和多药化疗;病情更严重的患者还接受了放疗并同步化疗。

结果

后遗症包括食管和胆总管狭窄、腹股沟神经卡压综合征和小肠梗阻。所有在青春期前通过腹主动脉旁野(34 - 37 Gy)接受脊柱照射的儿童均出现身材矮小。18例患者中有2例死于治疗并发症,1例死于疾病进展。

结论

多模式治疗对睾丸旁横纹肌肉瘤的预后良好,但与显著的发病率和死亡率相关。对治疗组成部分及其在疾病阶段的应用进行讨论,为优化这种对治疗敏感的恶性肿瘤的治疗提供了可能的方法。

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