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

Jennifer G. Michlitsch, Rodrigo L.P. Romao, Joseph Gleason, Luis H. Braga, Lisa Allen, Abha Gupta, Armando J. Lorenzo

Research output: Contribution to journalArticle

Abstract

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 24 months. 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 35 months. 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.

Original languageEnglish (US)
Pages (from-to)1374-1380
Number of pages7
JournalJournal of Pediatric Surgery
Volume53
Issue number7
DOIs
StatePublished - Jul 1 2018

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Rhabdomyosarcoma
Cheek
Radiation
Transplants
Radiotherapy
Reconstructive Surgical Procedures
Esthetics
Disease-Free Survival
Referral and Consultation
Pediatrics

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

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. / Michlitsch, Jennifer G.; Romao, Rodrigo L.P.; Gleason, Joseph; Braga, Luis H.; Allen, Lisa; Gupta, Abha; Lorenzo, Armando J.

In: Journal of Pediatric Surgery, Vol. 53, No. 7, 01.07.2018, p. 1374-1380.

Research output: Contribution to journalArticle

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abstract = "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 24 months. 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 35 months. 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.",
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