Pediatric vaginal rhabdomyosarcoma: Report of 2 cases

Abstract

Introduction: Rhabdomyosarcoma (RMS) arising in the female genital tract is rare accounting for 3.5% of all RMS cases. Approximately half these occur in the vagina, a site that has been associated with a favorable prognosis. Optimal loco-regional treatment for patients with vaginal RMS remains controversial since wide local excision is mutilating and often not done. Two cases of vaginal RMS are reported who underwent chemotherapy and local control with brachytherapy. Methods: Retrospective chart review was done between 2011 and 2015. During this interval, out of 31 cases of pediatric RMS managed at our institution, 2 had vaginal RMS. Their management and outcome is detailed below. Results - Case Materials: Two patients, both aged 2 years at the time of diagnosis, presented with grape-like mass protruding from vaginal orifice and bleeding for 1-2 months. Characteristic MRI features were of a heterogeneously enhancing polypoidal soft tissue mass filling vaginal lumen and protruding out of introitus confirming Botryoidal RMS. Biopsy and histopathology was suggestive of embryonal RMS (IHC positive for desmin, myogenin and focally for myo-D1). Tumor in both the patients was staged as Stage1 Group 3 (low risk). They were started on neo-adjuvant chemotherapy as per IRS-? Protocol with 3 weekly cycles of vincristine, dactinomycin and cyclophosphamide for 33 weeks. They had near complete regression of tumor and received brachytherapy for residual thickening of the vaginal wall. They have been followed up for 24 months and 57 months respectively from presentation, and are disease free. They are on close surveillance with periodic examination under anesthesia and imaging. One patient developed post radiation vaginal synechiae requiring vaginal dilatation. Conclusion: In patients with non-resected vaginal RMS, good outcome can be achieved by the use of brachytherapy for local control.