Miyake Makito, Tanaka Nobumichi, Matsushita Chie, Tanaka Masahiro, Tanaka Motoyoshi, Fujimoto Kiyohide, Hirao Yoshihiko, Konishi Noboru
Department of Urology, Nara Medical University.
Nihon Hinyokika Gakkai Zasshi. 2006 Mar;97(3):602-6. doi: 10.5980/jpnjurol1989.97.602.
A 31-year-old man was referred to our department for investigation and treatment of a rapidly-growing mass in the perineal region. MRI showed a periurethral tumor in the perineal region measuring 3.5cm in maximum diameter and bilateral inguinal lymph nodes enlargement. Therefore, the tumor was extirpated immediately with bilateral inguinal lymphadenectomy. The pathological examination with immunohistochemistry revealed that the tumor was proximal-type epithelioid sarcoma. Multidisciplinary therapy with irradiation, chemotherapy, and tumor ablation with a microwave tissue coagulator, was not effective for local recurrence and metastasis, and finally he died of progressive disease 14 months after the operation. Epithelioid sarcoma originating from the thigh or trunk is classified into the proximal-type, which shows significantly poorer prognosis than the distal-type that develops in the distal extremities. The proximal-type epithelioid sarcoma of the perineum is rare and, to our knowledge, the present report is the fourth case in the Japanese literatures.
一名31岁男性因会阴部迅速生长的肿物被转诊至我科进行检查和治疗。磁共振成像（MRI）显示会阴部尿道周围有一肿物，最大直径为3.5厘米，双侧腹股沟淋巴结肿大。因此，立即对肿瘤进行了切除并同时行双侧腹股沟淋巴结清扫术。免疫组织化学病理检查显示该肿瘤为近端型上皮样肉瘤。采用放疗、化疗以及微波组织凝固器进行肿瘤消融的多学科治疗对局部复发和转移均无效，最终患者在术后14个月因疾病进展死亡。起源于大腿或躯干的上皮样肉瘤被归类为近端型，其预后明显比发生于四肢远端的远端型更差。会阴部近端型上皮样肉瘤较为罕见，据我们所知，本报告是日本文献中的第四例。
