Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2018, Vol 34, Num, 3     (Pages: 255-258)

Cloacogenic Adenocarcinoma of the Vulva: A Case Report and Review of the Literature

Merih TEPEOĞLU 1, Halit ÜNER 2, A.Nihan HABERAL 1, Özlem ÖZEN 1, Esra KUŞÇU 3

1 Department of Pathology, Başkent University School of Medicine, Ankara, Turkey
2 Hacettepe University School of Medicine, Ankara, Turkey
3 Department of Obstetrics and Gynecology, Başkent University School of Medicine, Ankara, Turkey

DOI: 10.5146/tjpath.2015.01359
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Primary adenocarcinoma of the vulva, unrelated to the native glands of perineum is an extremely rare neoplasm. Despite awareness of this lesion for over 40 years, the origin is not beyond speculation. The most reasonable hypothesis is based on the remnants of cloacal differentiation during early days of life. Here we report the case of a 60-year-old patient with a vulvar mass, who underwent partial vulvectomy and bilateral regional lymph node dissection. The tumor was composed of papillary and complex glandular structures and exhibited diffuse positivity for cytokeratin 20 and polyclonal CEA, CDX2, and focal positivity with cytokeratin 7. Unlike the indolent behavior of this malignant neoplasm according to the literature, we found two metastatic inguinal lymph nodes. She did not receive adjuvant therapy and is still alive, free of disease 38 months after surgery. We present different aspects of vulvar adenocarcinomas with a case report.

Keywords : Vulvar neoplasms, Adenocarcinoma, Cloacogenic, Metastasis