Vulvo-vaginal intestinal/enteric heterotopia – report of two cases
The occurrence of ectopic intestinal/enteric type epithelium at the vulva is a rare entity sometimes mimicking intraepithelial neoplasia or malignant disease.
Here we report two cases.
The first consisted of an 82-year old woman with a long standing (10 years) white papillary lesion with some reddish areas at her left labium, extending into the vaginal introitus. The second case occurred in an 30-year old women at the vaginal introitus. Biopsies represented colonic-type glandular epithelium with positive immunostaining against CDX-2, p53, CK 7 and CEA, whereas staining against estrogen and progesterone receptor, mammoglobin, GCDFP-15 and CK 20 was negative.
The occurrence of coelomic-type glandular epithelium at the vulva may represent the result of dysontogenetic replacement of embryologic stem cells which undergo mucinous differentiation. So, the proper diagnostic term may glandular heterotopia. Although, some lesions especially in the proximity to orthotopic vulval glands, may be of metaplastic origin. Immunohistochemical staining patterns (CEA, CK 7 positive, CK 20 negative) indicate an intestinal/enteric phenotype (i.e. intestinal/enteric heterotopia). Because of the reported increased risk of malignant transformation of glandular vulval lesions, close clinical follow is recommended.