Kennedy J C, Majmudar B
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia.
J Reprod Med. 1993 Feb;38(2):113-6.
Primary adenocarcinoma of the vulva, arising in continuity with the epidermis and unrelated to the underlying mucus glands, is a distinctly uncommon neoplasm. The origin of such an unusual tumor, although attributed to cloacal remnants, remains speculative. We describe two patients with this rare lesion. No other primary tumor was found in either of the two after a thorough investigation. One patient was treated with radical vulvectomy and was free of disease for 10 years, until her death from unrelated causes. The second patient was treated with wide local excision and was free of disease four years postoperatively. Our experience indicates the need to recognize this tumor as a primary and not metastatic neoplasm of the vulva. We also noticed that in both cases, despite its histologic malignancy and ulceration, the tumor was superficial in its extent and without any tendency for deeper invasion. It can therefore be treated effectively with wide local excision, thus eliminating the need for radical vulvectomy.
原发性外阴腺癌起源于表皮且与下方黏液腺无关，是一种极为罕见的肿瘤。尽管这种不寻常肿瘤的起源被归因于泄殖腔残余，但仍存在推测性。我们描述了两名患有这种罕见病变的患者。经过全面检查，这两名患者均未发现其他原发性肿瘤。一名患者接受了根治性外阴切除术，术后无病生存10年，直至因无关原因死亡。第二名患者接受了广泛局部切除术，术后四年无病生存。我们的经验表明，需要将这种肿瘤识别为外阴原发性而非转移性肿瘤。我们还注意到，在这两个病例中，尽管肿瘤具有组织学恶性和溃疡表现，但其范围较浅，没有任何深部浸润的倾向。因此，通过广泛局部切除术可以有效治疗，从而无需进行根治性外阴切除术。
