title: Vulval intestinal/enteric heterotopia with a 10-yr follow-up: a case report and review of the literature.
abstract: 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 a case of an 82-yr-old woman with a long-standing (10 yr) white papillary lesion with some reddish areas at her left labium, extending into the vaginal introitus. Biopsy reports revealed 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 reported to be negative. A follow-up of 10 yr appeared uneventful. The occurrence of celomic-type glandular epithelium at the vulva may represent the result of dysontogenetic replacement of embryonic stem cells, which undergo mucinous differentiation. Thus, the proper diagnostic term may be 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, and 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-up is recommended.
authors.affiliation: Division of Breast, Gynecologic & Perinatal Pathology, Institute of Pathology, University of Leipzig, Leipzig (L.-C.H.) Department of Obstetrics and Gynecology, Martin-Luther-University, Halle/Saale (U.K.B., H.-G.S, I.K.), Germany.
authors.forename: Lars-Christian
authors.initials: LC
authors.lastname: Horn
authors.forename: Ursula Katharina
authors.initials: UK
authors.lastname: Bauerfeind
authors.forename: Hans-Georg
authors.initials: HG
authors.lastname: Strau
authors.forename: Ina
authors.initials: I
authors.lastname: Karbe
mesh_terms: D000369:Aged, 80 and over; D002828:Choristoma / Q000473:pathology*; D003937:Diagnosis, Differential; D004848:Epithelium; D004919:Erythroplasia / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007422:Intestines; D014845:Vulvar Diseases / Q000473:pathology*