Wevers A C, Kroon B B, Albus-Lutter C E, Gortzak E
Department of Surgery, Netherlands Cancer Institute, Amsterdam.
Eur J Surg Oncol. 1989 Aug;15(4):345-9.
In a 9-year period we treated 11 patients with an epithelioid sarcoma. Most patients were young adults. The tumour affected mainly the distal upper extremity (hand and fingers: four patients; wrist and forearm: three patients); one patient had an epithelioid sarcoma of the knee. Trunk localizations were seen in two patients and one patient presented with a vulva localization. Treatment consisted of surgery, regional isolation perfusion with doxorubicin (Adriamycin) or melphalan, radiation therapy, systemic chemotherapy or a combination of these modalities. Radical surgery in eight patients resulted in only two local recurrences. Locoregional metastases occurred in five patients. In five patients a therapeutic lymph node dissection was performed, in all instances followed by extensive recurrent disease. Distant metastases were seen in seven patients and mainly affected the skeleton (6x) and the lungs (4x); in the majority of cases these metastases occurred within a year after excision of the primary. Remissions following regional or systemic chemotherapy were not observed. At the time of analysis seven patients had died as a result of their epithelioid sarcoma; two patients were still alive with tumour 18 and 27 months after diagnosis. Only two patients remained tumour free for 17 and 65 months respectively following radical surgery, postoperative radiation therapy and (in one case) adjuvant chemotherapy. It is concluded that epithelioid sarcoma is a rare but exceedingly aggressive tumour. Since early diagnosis can only be auspicious, familiarity with the clinical features is of great importance.
在9年的时间里，我们治疗了11例上皮样肉瘤患者。大多数患者为年轻成年人。肿瘤主要累及上肢远端（手部和手指：4例；腕部和前臂：3例）；1例患者患有膝关节上皮样肉瘤。2例患者肿瘤位于躯干，1例患者肿瘤位于外阴。治疗方法包括手术、多柔比星（阿霉素）或美法仑区域隔离灌注、放射治疗、全身化疗或这些方法的联合应用。8例患者接受根治性手术后仅2例局部复发。5例患者发生局部区域转移。5例患者进行了治疗性淋巴结清扫，所有病例随后均出现广泛的复发性疾病。7例患者出现远处转移，主要累及骨骼（6例）和肺部（4例）；在大多数情况下，这些转移发生在原发肿瘤切除后一年内。未观察到区域或全身化疗后的缓解情况。在分析时，7例患者因上皮样肉瘤死亡；2例患者在诊断后18个月和27个月仍带瘤存活。仅2例患者在接受根治性手术、术后放射治疗和（1例）辅助化疗后分别无瘤生存17个月和65个月。结论是上皮样肉瘤是一种罕见但极具侵袭性的肿瘤。由于早期诊断可能才是有利的，熟悉其临床特征非常重要。
