Clear Cell Carcinoma of the Vagina in an 11-Year-Old Girl: A Case Report on Clinical, Imaging, and Immunophenotypic Features

Abstract

Vaginal clear cell carcinoma (CCC) is a rare malignant tumor in the pediatric population and may occur even in the absence of in utero exposure to diethylstilbestrol (DES). We report the case of an 11-year-old girl without DES exposure who presented with persistent foul-smelling vaginal discharge, abdominal pain, and weight loss. Imaging revealed a large vaginal mass with regional lymph node involvement. Histopathology confirmed CCC with Müllerian immunophenotype. The disease was classified as advanced and unresectable, and the patient was treated with concurrent cisplatin-based chemoradiation followed by high-dose-rate interstitial brachytherapy. She achieved significant tumor reduction and remains clinically disease-free to date. This case underscores the diagnostic complexity of pediatric vaginal CCC and supports chemoradiation with brachytherapy as an effective strategy for unresectable disease.