Botryoid rhabdomyosarcoma of vagina

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents under 15 years of age. Due to the young age of the affected patients, this malignancy poses a certain challenge for patient management, as the preservation of hormonal, sexual and reproductive functions is essential at this age. It is therefore necessary to make a correct diagnosis as quickly as possible, in order to perform the most considerate resection while maintaining fertility. Imaging methods such as cystography, ultrasonography, computed tomography and magnetic resonance imaging help us to achieve this goal. As a part of the differential diagnosis lesion like yolk sack tumor and tumorous cystitis should be considered. Neuroblastoma, Burkitt›s lymphoma, and sacrococygeal teratoma should be ruled out in the broader differential diagnosis when the localization of tumor lesion is in pelvic region. The case report deals with a tumor that occurred in the vagina of a 2-year-old female patient. The patient was treated with fertility sparing surgery, adjuvant chemotherapy and, because of repeated relapses received brachytherapy. Until the last follow-up, the patient remained in remission and no local or distant metastases were documented. It is important to raise awareness of such an uncommon lesion, its differential diagnosis, and clinical implications to avoid misdiagnosis. (author)