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Local control for vaginal botryoid rhabdomyosarcoma with pre-rectal transperineal surgical resection and autologous buccal graft vaginal replacement: A novel, minimally invasive, radiation-sparing approach

2017; Elsevier BV; Volume: 53; Issue: 7 Linguagem: Inglês

10.1016/j.jpedsurg.2017.11.044

1531-5037

Jennifer Michlitsch, Rodrigo Romao, Joseph M. Gleason, Luis H. Braga, Lisa Allen, Abha A. Gupta, Armando J. Lorenzo,

Surgical site infection prevention

Localized vaginal rhabdomyosarcoma (RMS) is associated with a favorable prognosis, but strategies for local control remain controversial. The use of radiotherapy (RT) can have important long-term sequelae, while traditional resection involves major reconstructive surgery. We describe a new surgical approach employing a minimally-invasive resection and immediate reconstruction.Records from 4 consecutive patients with localized vaginal RMS managed in 4 major pediatric referral centers were reviewed. All cases were performed with a standardized technique.Patients were diagnosed at a median age of 24months. Each underwent a total/subtotal vaginectomy with autologous buccal graft vaginal replacement. Final margins were focally positive in one patient and negative in three. None received radiotherapy. To date, all patients have patent buccal neovaginas, enjoy a favorable aesthetic result, and remain disease-free at a median follow-up of 35months.We report 4 cases of localized vaginal RMS successfully treated with a minimally invasive surgical approach. All patients have avoided radiation and remain disease-free. Our initial data suggest that surgical local control and immediate reconstruction are feasible and can spare these patients the long-term complications of RT. Longer follow-up is critical to ensure disease-free survival with a functional, successfully reconstructed neovagina.Case series.Level IV.