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手部和足部横纹肌肉瘤的局部治疗:是否需要截肢?来自儿童肿瘤学组的报告。

Local therapy for rhabdomyosarcoma of the hands and feet: is amputation necessary? A report from the Children's Oncology Group.

机构信息

Department of Radiation Oncology, Stanford University, Stanford, CA 94305, USA.

出版信息

Int J Radiat Oncol Biol Phys. 2011 May 1;80(1):206-12. doi: 10.1016/j.ijrobp.2010.01.053. Epub 2010 Jun 18.

Abstract

PURPOSE

To evaluate the outcome of children with rhabdomyosarcoma (RMS) of the hand or foot treated with surgery and/or local radiotherapy (RT).

METHODS AND MATERIALS

Forty-eight patients with nonmetastatic RMS of the hand or foot were enrolled on Intergroup Rhabdomyosarcoma Study III, IV-Pilot, and IV. Patients received multiagent chemotherapy with surgery and/or RT. Twenty-four patients (50%) underwent surgery without local RT, of whom 4 had complete resection and 20 had an amputation. The remaining 24 patients (50%) underwent local RT, of whom 2 required RT for microscopic residual disease after prior amputation. Median follow-up for surviving patients was 9.7 years.

RESULTS

Actuarial 10-year local control was 100%; 10-year event-free survival and overall survival rates were 62% and 63%, respectively. Poor prognostic factors for recurrence included gross residual (Group III) disease and nodal involvement (p = 0.01 and 0.05, respectively). More patients in the RT group had alveolar histology, Group III disease, and nodal involvement, as compared with the surgery group. There was no difference in 10-year event-free survival (57% vs. 66%) or overall survival (63% vs. 63%) between patients who underwent surgery or local RT. Among relapsing patients, there were no long-term survivors. No secondary malignancies have been observed.

CONCLUSIONS

Despite having high-risk features, patients treated with local RT achieved excellent local control. Complete surgical resection without amputation is difficult to achieve in the hand or foot. Therefore, we recommend either definitive RT or surgical resection that maintains form and function as primary local therapy rather than amputation in patients with hand or foot RMS.

摘要

目的

评估手部或足部横纹肌肉瘤(RMS)患儿经手术和/或局部放疗(RT)治疗的结果。

方法和材料

共有 48 例手部或足部非转移性 RMS 患儿入组 Intergroup Rhabdomyosarcoma Study III、IV-Pilot 和 IV。患儿接受多药化疗联合手术和/或 RT。24 例患者(50%)未行局部 RT 而行手术治疗,其中 4 例为完全切除,20 例行截肢。其余 24 例患者(50%)行局部 RT,其中 2 例因先前截肢后存在显微镜下残留疾病而需要 RT。存活患者的中位随访时间为 9.7 年。

结果

10 年局部无复发生存率为 100%;10 年无事件生存率和总生存率分别为 62%和 63%。与复发相关的不良预后因素包括大体残留(III 组)疾病和淋巴结受累(p=0.01 和 0.05)。与手术组相比,RT 组更多患者具有肺泡组织学、III 组疾病和淋巴结受累。行手术或局部 RT 治疗的患者在 10 年无事件生存率(57%比 66%)或总生存率(63%比 63%)方面无差异。在复发患者中,无长期生存者。未观察到继发性恶性肿瘤。

结论

尽管存在高危特征,但接受局部 RT 治疗的患者局部控制效果良好。在手或足部完全切除而不截肢难以实现。因此,我们建议在手或足部 RMS 患者中,将明确的 RT 或保留形态和功能的手术切除作为主要局部治疗手段,而不是截肢。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5be4/3075377/e513aabd43b4/nihms-179324-f0001.jpg

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