Introduction/Background Clear cell adenocarcinoma of the cervix (CCCC) is a rare form of cervical cancer. Historically, it affected women of reproductive age who were exposed to Diethylboestrol (DES), the major risk factor. However, since the prohibition on DES, the majority of CCCC cases have occurred in older women who were not exposed to DES, suggesting that additional risk factors are involved in the carcinogenesis of CCCC.

Methodology We retrospectively analyzed clinical data of 17 patients with CCCC who were treated from January 2012 to december 2020 in our institute.

Results The median age was 57.82 years. Twelve patients were menopausal. The mean age of first sexual intercourse was 24 years. The most common symptom was vaginal bleeding. In all cases, there was no evidence of DES exposure. The tumor was ulcerating in ten cases, budding in five cases, and destroying the cervix in one case. On average, clinical tumor size was 3.73 cm. 41.17% patients were stage I, 52.9% were stage II, 52.9% were stage III. Neoadjuvant treatment including concomitant radio-chemotherapy was performed in7 cases, external pelvic radiation combined with utero-vaginal-brachytherapy in3 cases, and exclusive vaginal-brachytherapy in5 cases. Radical-hysterectomy was performed on 12 patients (83% PIVER III, 16% PIVER II). Pelvic-lymphadenectomy was performed in all cases. Only 2 cases had a lumbo-aortic-lymphadenectomy. The mean histological size was 0.9 cm (0–3 cm). Lymph-node involvement was noted in2 patients. Four patients had adjuvant treatment: pelvic radiation (1/4), chemotherapy (1/4), vaginal-brachytherapy (1/4) and combination of chemotherapy and brachytherapy (1/4). after a median follow-up of 55 months, 4 patients were alive and in remission, 11 were still evolving and 3 were lost to follow-up.

Conclusion In the absence of traditional risk factors, CCCC does not have a poorer prognosis than squamous cell carcinoma. Treatment is based on concomitant radiochemotherapy followed by radical surgery.