Kasamatsu T, Hasegawa T, Tsuda H, Okada S, Sawada M, Yamada T, Tsunematsu R, Ohmi K, Mizuguchi K, Kawana T
Division of Gynecology, National Cancer Center Hospital, Tokyo, Japan.
Int J Gynecol Cancer. 2001 Jul-Aug;11(4):316-20. doi: 10.1046/j.1525-1438.2001.011004316.x.
A case of a 31-year-old woman with epithelioid sarcoma of the vulva which metastasized to the regional lymph node 8 years after onset of the disease is reported here. The patient first noticed a painless subcutaneous mass of 5 mm in diameter in the right labium majus at age of 21. This was excised locally at age 23, but recurred 17 months later. Although local excision was again performed, the tumor recurred and continued to enlarge very slowly. At this stage, based on the pathology of both the initial and second tumors, the diagnosis was of a benign inflammatory process. However, local recurrence and inguinal lymph node swelling occurred at age 29, and biopsy was taken. The pathology report indicated benign granulomatous changes. The slides were reconsidered and re-interpreted as epithelioid sarcoma, whereupon radical vulvectomy was performed at age 31. Vulvar epithelioid sarcoma with inguinal lymph node metastasis was first diagnosed at that time. Epithelioid sarcoma of the vulva is an exceedingly rare tumor, and only 15 cases have been reported thus far in the literature. Early diagnosis and curative treatment of this tumor may be problematic for gynecologists because of its rarity and therefore little-known characteristic clinical behavior and histology. Radical vulvectomy or extensive local excision with inguinal lymphadenectomy at the time of diagnosis is recommended as the treatment of choice.
本文报道了一例31岁女性外阴上皮样肉瘤患者，该病发病8年后转移至区域淋巴结。患者21岁时首次发现右侧大阴唇有一个直径5毫米的无痛性皮下肿物。23岁时进行了局部切除，但17个月后复发。尽管再次进行了局部切除，但肿瘤仍复发并继续非常缓慢地增大。在此阶段，根据最初和第二次肿瘤的病理结果，诊断为良性炎症过程。然而，29岁时出现局部复发和腹股沟淋巴结肿大，并进行了活检。病理报告显示为良性肉芽肿性改变。对切片进行重新评估和重新解读后诊断为上皮样肉瘤，于是患者31岁时接受了根治性外阴切除术。当时首次诊断出伴有腹股沟淋巴结转移的外阴上皮样肉瘤。外阴上皮样肉瘤是一种极其罕见的肿瘤，迄今为止文献中仅报道了15例。由于其罕见性以及鲜为人知的特征性临床行为和组织学表现，妇科医生对该肿瘤进行早期诊断和根治性治疗可能存在困难。建议在诊断时采用根治性外阴切除术或广泛局部切除加腹股沟淋巴结清扫术作为首选治疗方法。
