Halling A C, Wollan P C, Pritchard D J, Vlasak R, Nascimento A G
Division of Anatomic Pathology, Mayo Clinic Rochester, MN 55905, USA.
Mayo Clin Proc. 1996 Jul;71(7):636-42. doi: 10.1016/S0025-6196(11)63000-0.
To identify any clinical and pathologic features of treatment modalities that are predictive of outcome in patients with epithelioid sarcoma, a rare slow-growing soft tissue tumor most commonly occurring in the distal extremities of young adults.
We reviewed the institutional files for cases of epithelioid sarcoma for the period 1956 to 1991 and analyzed the effect of various factors on survival.
Fifty-five cases of epithelioid sarcoma were found, and the relevant clinical, pathologic, treatment, follow-up, and outcome features were assessed.
All tumors were treated initially by operative resection. The recurrence rate progressively decreased with increasing aggressiveness of the initial operation; however, no difference was noted in metastatic rate. Overall, the recurrence rate was 38% and the metastatic rate was 47%. At the end of a mean follow-up of 102 months, 69% of patients had no evidence of disease, 27% had died of the disease, and 4% were alive with disease. Increasing tumor size, necrosis of more than 30%, and vascular invasion correlated significantly with a worse prognosis.
Epithelioid sarcoma should be considered a malignant neoplasm with a significant potential for aggressive behavior, and close follow-up of affected patients should be maintained for many years. Initial treatment should be aggressive in an attempt to prevent recurrence.
确定上皮样肉瘤患者治疗方式的临床和病理特征，上皮样肉瘤是一种罕见的生长缓慢的软组织肿瘤，最常见于年轻成人的四肢远端，这些特征可预测其预后。
我们回顾了1956年至1991年期间上皮样肉瘤病例的机构档案，并分析了各种因素对生存的影响。
共发现55例上皮样肉瘤病例，并对相关的临床、病理、治疗、随访及预后特征进行评估。
所有肿瘤最初均采用手术切除治疗。随着初次手术侵袭性的增加，复发率逐渐降低；然而，转移率未见差异。总体而言，复发率为38%，转移率为47%。在平均随访102个月结束时，69%的患者无疾病证据，27%死于该疾病，4%带瘤生存。肿瘤大小增加、坏死超过30%以及血管侵犯与预后较差显著相关。
上皮样肉瘤应被视为具有显著侵袭性行为潜能的恶性肿瘤，应对受影响患者进行多年的密切随访。初始治疗应积极，以试图预防复发。
