Difficulties of clinical and histopathological diagnosis in advanced vulvar clear cell carcinoma.

Clear cell carcinoma (CCC) of the female genital tract usually arises in the ovary, endometrium, cervix and vagina. A rare site for CCC is the vulva, and moreover even rarer are the cases involving the Bartholin gland. A 54-year-old female was admitted for a 1.5×2 cm tumor at the level of the right Bartholin gland. The magnetic resonance imaging (MRI) exam revealed enlarged inguinal, pelvic and para-aortic lymph nodes but no other primary tumor. Microscopic examination revealed CCC. The tumor was positive for cytokeratin 7 (CK7), paired-box 8 (Pax8), napsin A and vimentin, negative for estrogen receptor (ER), progesterone receptor (PR), calretinin, cluster of differentiation 10 (CD10), carcinoembryonic antigen (CEA), p16 and p63. Also, p53 was expressed in 30-40% and Ki67 in 70% of the malignant cells. Given the clinical, imagistic, histological and immunohistochemical features of the tumor, we concluded that the tumor is a CCC of the Bartholin gland. Aim of the study is to signal a rare case of CCC of Bartholin gland. Since there are only two other cases reported in literature, the natural history and prognosis of the disease is not known, also there are no therapeutic guidelines regarding this rare tumor so appropriate treatment is uncertain. Therefore, it is important that new cases are reported for a better understanding of this rare condition. Bartholin gland carcinoma is a pathology quite rarely encountered in practice. The positive diagnosis is eminently histological and immunohistochemistry. Bartholin gland CCC is an extremely rare diagnosis with, to our knowledge, only two other cases reported in literature, but with a potential aggressive clinical behavior and poor outcome.