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

J Pediatr Surg. 2018 Jul;53(7):1374-1380. doi: 10.1016/j.jpedsurg.2017.11.044. Epub 2017 Nov 21.


Purpose: 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.

Materials and methods: 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.

Results: 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.

Conclusions: 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.

Type of study: Case series.

Level of evidence: Level IV.

Keywords: Buccal mucosa; Children; Local control; Radiation; Vaginal rhabdomyosarcoma; Vaginectomy.

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Gynecologic Surgical Procedures / methods*
  • Humans
  • Mouth Mucosa / transplantation*
  • Neoadjuvant Therapy / methods
  • Rhabdomyosarcoma / surgery*
  • Treatment Outcome
  • Vagina / surgery*
  • Vaginal Neoplasms / surgery*