Primary clear cell adenocarcinoma of the vagina in a young female with uterus didelphys

Abstract

Adenocarcinomas of clear cell type are uncommon tumors accounting for 5-10% of all vaginal tumours which in turn account for approximately 2% of all gynaecologic malignancies. Though Clear cell Adenocarcinomas arising in the setting of previous Di-Ethyl–Stilbesterol [DES] exposure have been reported quite often, only few cases of these not associated with DES have been documented. Amongst non-DES exposed patients, CCAs are said to be sporadic or in association with endometriosis in women of peri/postmenopausal age or in association with some congenital genitourinary anomalies mostly affecting young females. A 16 year old female presented with heavy bleeding per vaginum since 2 months. On per vaginal examination a polypoidal mass was felt on posterior wall of vagina. Ultrasonography revealed the presence of a uterus didelphys in addition to this mass. Polypectomy was done. On microscopy, a diagnosis of clear cell adenocarcinoma was made which was confirmed on immunohistochemistry. There was no history of any DES exposure in the mother. We present here a case of an adolescent female with uterus didelphys and no DES exposure who was diagnosed as a case of Vaginal Clear cell Adenocarcinoma. The rarity of this tumour among DES-exposed cases and its presence in non-exposed patients warrants a more thorough search for the underlying pathology.