Vaginal Rhabdomyosarcome: Case Report

Abstract

Introduction: Rhabdomyosarcoma is a soft tissue sarcoma, most common in children, localised in the female genital tract and therefore rare in adults. Among its subtypes, it is most often distinguished as embryonal. It is an aggressive tumour, difficult to diagnose, is usually detected by CT or MRI and the diagnosis is confirmed by biopsy and treatment involving surgical excision, radiotherapy and chemotherapy. The authors present a case of embryonal rhabdomyosarcoma localised in the vagina, highlighting relevant aspects of the subject. Observation: We report the observation of a girl aged 2 years and 8 months, with no notable antecedents, from a 2nd degree consanguineous marriage. For 1 month she presented with a rapidly progressive vaginal mass associated with bleeding. Examination revealed a reddish, grape-like mass in the vulva. Abdomino-pelvic ultrasound revealed a vaginal tumour, abdomino-pelvic MRI revealed an endovaginal mass delivered through the vulva, suggesting a botryoid rhabdomyosarcoma, and biopsy of the vaginal mass with morphological appearance and immunohistochemical profile of an embryonal rhabdomyosarcoma. The extension work-up showed a vulvovaginal lesional process, with no secondary location on the thoraco-abdominal CT scan. The patient was classified as group C according to the RMS 2005 protocol and received 6 courses of IVA + vaginal brachytherapy + 3 courses of IVA with a good clinical and biological evolution. Conclusion: In view of the diagnostic difficulties and the risk of progression and rapid worsening of rhabdomyosarcoma, early positive diagnosis means that treatment can be started at an early stage with chemotherapy, surgery and radiotherapy.