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上皮样肉瘤。诊断、预后指标及治疗。

Epithelioid sarcoma. Diagnosis, prognostic indicators, and treatment.

作者信息

Chase D R, Enzinger F M

出版信息

Am J Surg Pathol. 1985 Apr;9(4):241-63.

PMID:4014539
Abstract

A retrospective review of 241 cases of epithelioid sarcoma reaffirmed the propensity of this tumor to occur in the distal extremities of young adults. The tumor was generally firm and nontender, and involved the dermis, subcutis or deeper soft tissues, particularly fascial planes, aponeuroses, and tendon sheaths. Follow-up data, available in 202 cases (84%), showed a 77% recurrence and a 45% metastatic rate. The most common initial sites of metastasis were lymph nodes (48%) and lungs (25%). A more aggressive course was associated with a proximal or axial tumor location, increased size and depth, hemorrhage, mitotic figures, necrosis, or the presence of vascular invasion. More favorable behavior was observed when the tumor arose in younger individuals, in distal extremities, or in females between the ages of 10 and 49. (The last observation calls for further work with steroid receptors.) Radical excision or amputation still appears to be the initial treatment of choice, though adjunctive high-dose radiotherapy to the excision site may prove to be of additional value.

摘要

一项对241例上皮样肉瘤病例的回顾性研究再次证实,这种肿瘤倾向于发生在年轻成年人的四肢远端。肿瘤通常质地坚硬且无压痛,累及真皮、皮下组织或更深层的软组织,尤其是筋膜平面、腱膜和腱鞘。202例(84%)有随访数据,显示复发率为77%,转移率为45%。最常见的初始转移部位是淋巴结(48%)和肺(25%)。肿瘤位于近端或中轴线、体积增大、深度增加、出血、有丝分裂象、坏死或存在血管侵犯与病程更具侵袭性相关。当肿瘤发生在较年轻个体、四肢远端或10至49岁的女性时,观察到其行为更有利。(最后这一观察结果需要对类固醇受体做进一步研究。)根治性切除或截肢似乎仍是首选的初始治疗方法,不过对切除部位辅助进行高剂量放疗可能会有额外价值。

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