Primary vulval mucinous adenocarcinoma of intestinal type masquerading as Bartholin's cyst

Abstract

Vulval carcinomas are rare and account for 3%–5% of female genital tract malignancies. Primary vulval adenocarcinoma of intestinal type is an extremely rare tumor which is considered metastatic until otherwise proven with very few case reports available in the literature. A 58-year-old woman presented with recurrent swelling in the genital region associated with pain. She had a past history of surgery done for Bartholin's cyst which was reported as adenocarcinoma. She underwent radical vulvectomy for the recurrent tumor which showed features of adenocarcinoma with glandular and papillary pattern with abundant extracellular mucin production and immunohistochemical (IHC) features favoring a diagnosis of primary vulval mucinous adenocarcinoma of intestinal type after excluding metastasis from other sites. As morphology cannot differentiate it from metastasis, a thorough investigation to rule out any primary in the gastrointestinal tract with a minimum IHC panel of markers including cytokeratin (CK) 20, CK7, carcinoembryonic antigen, and caudal-related homeobox 2 can help in confirming the diagnosis.