Clear Cell Carcinoma In 13 Year-Old Girl With No History Of Distilben Exposure

Introduction Clear cell carcinoma of the vagina (CCV) is a rare entity in the pediatric population. It is usually reported to have been associated with an intrauterine exposure to Distilben (DES). CCV with no exposure to DES arise in older women. Our case is unique because it reports a CCV in young girl with no history of DES exposure. Case Report We report a case of 13-year-old girl with no history of intrauterine exposure to DES. She was reported to our department for a vaginal bleeding evolving since 8 months and wrongly mistaken for the menarche. Clinical examination showed a vaginal mass invading the lower third of the vagina, the hymen was intact. Body scan found suspect pulmonary nodes. Biopsy concluded to CCV. She first had chemotherapy with taxoter and carboplatin regimen then concomitant chemotherapy and radiotherapy. The clinical and radiological response was partial. She underwent a pelvic exenteration and pulmonary lobectomy. Histology showed a CCV in both surgical specimen with free margins and one metastatic lymph node to lomboaortic chain. Actually the patient is free of disease after 3 months of follow-up. Conclusion No DES-exposure CCV shows a bimodal age distribution with a first peak in latest twenties and the second in 7th decade. Our case is unique due to the early age of our patient. The treatment was not standard due to the young age of the patient too. Further cases should be studied to elaborate adequate guidelines.