Progress in the surgical management of vaginal rhabdomyosarcoma: A 25- year review from the Intergroup Rhabdomyosarcoma Study Group

Background/Purpose: During its 25 years of experience, the Intergroup Rhabdomyosarcoma Study Group (IRSG) has completed four sequential prospective clinical trials to improve survival and decrease morbidity rates in childhood rhabdomyosarcoma (RMS). Surgical management of vaginal RMS has changed dramatically. Methods: The records of 72 patients with localized vaginal RMS were reviewed to assess surgical therapy, chemotherapy, and radiotherapy and their relation to outcome. Results: Each successive IRSG trial resulted in decreased need for surgical resection (IRS-I, 100%; IRS-II, 70%; IRS-III, 30%; IRS-IV, 13%) and excellent disease-free survival using increasingly effective multiagent chemotherapy. Conclusions: Primary chemotherapy after initial biopsy provides excellent tumor control. Local resection may be appropriate, but removal of organs (ie, complete vaginectomy/hysterectomy) has no role except in persistent or recurrent disease. Mature, residual rhabdomyoblasts may be evidence of a chemotherapy response, and further surveillance and biopsy without surgical resection is adequate treatment.