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手足部肉瘤:肢体保留综合治疗的局部控制及功能结果分析

Sarcomas of the hand and foot: analysis of local control and functional result with combined modality therapy in extremity preservation.

作者信息

Johnstone P A, Wexler L H, Venzon D J, Jacobson J, Yang J C, Horowitz M E, DeLaney T F

机构信息

Radiation Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892.

出版信息

Int J Radiat Oncol Biol Phys. 1994 Jul 1;29(4):735-45. doi: 10.1016/0360-3016(94)90561-4.

Abstract

PURPOSE

The records of 28 patients with sarcomas of the hand and foot treated at the National Cancer Institute (NCI) between 1977 and 1992 were reviewed to assess local control and functional results.

METHODS AND MATERIALS

Histologic types included 15 cases of the Ewing's sarcoma family of tumors, 7 cases of alveolar rhabdomyosarcoma, and 6 cases of nonrhabdomyosarcoma soft tissue sarcomas. Median age of all patients was 18 years (range 4-61), with a median potential follow-up of 114 months following diagnosis. Surgery varied from incisional biopsies for Ewing's Sarcoma and rhabdomyosarcoma lesions to complete excision when possible for nonrhabdomyosarcoma soft tissue sarcoma lesions. Amputation was not primarily performed, except in two patients who underwent ray resections of hand lesions (patients 13 and 24). Radiotherapy generally consisted of 50 Gy/25 fractions (fx)/5 weeks for Ewing's Sarcoma, 54 Gy/30 fx/6 weeks for rhabdomyosarcoma, and 63 Gy/35 fx/7 weeks for nonrhabdomyosarcoma soft tissue sarcomas. Chemotherapy was administered on various NCI protocols.

RESULTS

Actuarial local control for Ewing's Sarcoma was 84% at 5 and 10 years. All but one survivor are capable of hand/foot function for routine activities without orthotic requirements. Five of six patients (83%) who died of metastatic disease had functional distal extremities. Actuarial local control for rhabdomyosarcomas was 100%, with equivalent function. No patient developed a second malignancy in the treatment field.

CONCLUSIONS

Although equivalent local control may be achieved in these lesions with either amputation or radiotherapy, a prudent management course would be to defer amputation for management of local recurrences. Many patients with these lesions fail in distant sites only and die without local failure. For these patients and for those who remain long-term survivors, we believe a functional hand and foot provides a better quality of life than a prosthesis.

摘要

目的

回顾1977年至1992年间在美国国立癌症研究所(NCI)接受治疗的28例手足肉瘤患者的记录,以评估局部控制情况和功能结果。

方法和材料

组织学类型包括15例尤因肉瘤家族性肿瘤、7例肺泡横纹肌肉瘤和6例非横纹肌肉瘤软组织肉瘤。所有患者的中位年龄为18岁(范围4 - 61岁),诊断后的中位潜在随访时间为114个月。手术方式因病情而异,尤因肉瘤和横纹肌肉瘤病变采用切开活检,非横纹肌肉瘤软组织肉瘤病变尽可能进行完整切除。除两名接受手部病变射线切除的患者(患者13和24)外,未主要进行截肢手术。放疗一般为尤因肉瘤50 Gy分25次/5周,横纹肌肉瘤54 Gy分30次/6周,非横纹肌肉瘤软组织肉瘤63 Gy分35次/7周。化疗按照各种NCI方案进行。

结果

尤因肉瘤5年和10年的精算局部控制率为84%。除一名幸存者外,所有幸存者均能够进行手足功能的日常活动,无需矫形器。死于转移性疾病的6名患者中有5名(83%)远端肢体功能正常。横纹肌肉瘤的精算局部控制率为100%,功能相当。治疗区域内无患者发生第二原发恶性肿瘤。

结论

虽然截肢或放疗在这些病变中均可实现相当的局部控制,但谨慎的处理方法是推迟截肢以处理局部复发。许多患有这些病变的患者仅在远处部位出现失败并死于无局部失败。对于这些患者以及那些长期存活的患者,我们认为功能正常的手足比假肢能提供更好的生活质量。

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