Abstract
Clear cell adenocarcinoma of the cervix is a rare neoplasm that accounts for about 4-9% of all uterine cervical adenocarcinoma. It was first reported in young women with in-utero exposure of diethylstilbesterol (DES). However, many cases have been reported post-ban of DES that are not associated with diethylstilbesterol (DES). Here, we are reporting a case of clear cell adenocarcinoma in an 18-year-old woman without any exposure history of DES. An exophytic mass arising from the anterior lip was found in MRI. Biopsy from the mass was diagnosed as Clear cell adenocarcinoma of the cervix based on histopathological and immunohistochemical findings. The patient then underwent a total hysterectomy and bilateral salpingo-oophorectomy. One each of the right pelvic lymph nodes and external iliac lymph nodes showed metastatic deposits. The patient was staged as FIGO stage I [AJCC TNM staging (8th Edition): pT2b pN1]. Post 6 months follow up there is no evidence of any recurrence.