Vaginal clear cell adenocarcinoma with associated Müllerian duct anomalies, renal agenesis and situs inversus: report of a case with no known in-utero exposure with diethyl stilboestrol.
A 27 year old female presented with two months history of continuous vaginal bleeding. On evaluation, she was diagnosed as a case of vaginal clear cell adenocarcinoma (VCCA) along with associated Müllerian duct anomalies including didelphys uterus with double vagina; left sided renal agenesis and situs inversus. Although VCCA is strongly linked to in-utero exposure with Diethyl stillboestrol (DES), this patient had no known history regarding such exposure. Also, only a few cases have been described in the literature in which this tumour was seen in association with Müllerian duct anomalies and renal agenesis; in the absence of known DES exposure.
Zeeshan-ud-din
Section of Histopathology, Department of Pathology & Microbiology, The Aga Khan University Hospital, Karachi.
Ahsan
Aamir
A
D018262:Adenocarcinoma, Clear Cell / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D000328:Adult; D002273:Carcinogens / Q000633:toxicity; D004054:Diethylstilbestrol / Q000633:toxicity; D005260:Female; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D009095:Mullerian Ducts / Q000473:pathology*; D011247:Pregnancy; D011297:Prenatal Exposure Delayed Effects; D012857:Situs Inversus / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D014599:Uterus / Q000002:abnormalities; D014621:Vagina / Q000002:abnormalities; D014625:Vaginal Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery
D002273:Carcinogens; D004054:Diethylstilbestrol