Graf A H, Su H C, Tubbs R R, Hacker G W, Dietz O, Staudach A
Department of Obstetrics and Gynecology, Salzburg General Hospital, Austria.
Anticancer Res. 1998 May-Jun;18(3B):2041-5.
Only a few cases on mucinous adenocarcinomas of the vulva have been reported. In this study, we present a case of a 75-year-old woman with a tumor in the left major labium. Because biopsy had shown formations of squamous cell carcinoma, radical vulvectomy with bilateral inguinal and femoral lymph node dissection were performed. At that time, histology was interpreted as small-cell, anaplastic carcinoma, with focal epidermoid differentiation. Postoperative radiation therapy was performed. Sixteen months after surgery, the patient presented with bilateral breast carcinomas. Histology showed a scirrhous carcinoma of the left and a medullary carcinoma of the right breast, but no lymph node metastases. Histochemical and immunohistochemical re-examination of the vulvar carcinoma now revealed a mucinous adenocarcinoma with neuroendocrine differentiation. The tumor expressed neuroendocrine markers such as chromogranin A and protein gene-product (PGP) 9.5, as well as peptides of the vasoactive intestinal polypeptide (VIP) family, and serotonin. Histochemical silver stains demonstrated Grimelius argyrophilia and Masson argentaffinity. Because of positive estrogen and progesterone receptor status of both breast cancers, postoperative Tamoxifen therapy was performed. The patient is still alive four years after vulvectomy.
仅有少数外阴黏液腺癌的病例被报道。在本研究中，我们报告了一例75岁女性，其左侧大阴唇有一肿瘤。由于活检显示为鳞状细胞癌形成，遂行根治性外阴切除术及双侧腹股沟和股淋巴结清扫术。当时，组织学诊断为小细胞、间变性癌，伴有局灶性表皮样分化。术后进行了放射治疗。术后16个月，患者出现双侧乳腺癌。组织学显示左侧为硬癌，右侧为髓样癌，但无淋巴结转移。对外阴癌进行组织化学和免疫组织化学重新检查，现在发现是具有神经内分泌分化的黏液腺癌。肿瘤表达神经内分泌标志物，如嗜铬粒蛋白A和蛋白基因产物（PGP）9.5，以及血管活性肠多肽（VIP）家族的肽类和5-羟色胺。组织化学银染显示嗜银性和亲银性。由于双侧乳腺癌雌激素和孕激素受体状态均为阳性，术后进行了他莫昔芬治疗。该患者在外阴切除术后四年仍然存活。
