A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma

Background: Perianal rhabdomyosarcoma ((P)RMS) are rare and have a poor prognosis. Data in young children are limited and local therapy is not well defined. Combined brachytherapy and surgery is routinely being used for RMS at other sites in children as it provides good oncologic outcomes and allows...

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Main Authors: Andreas Schmidt, David Baumann, Ulf Lamprecht, Benjamin Mayer, Cristian Urla, Benjamin Bender, Jürgen Schäfer, Frank Fideler, Maximilian Niyazi, Frank Paulsen, Jörg Fuchs
Format: Article
Language:English
Published: Elsevier 2024-11-01
Series:Clinical and Translational Radiation Oncology
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Online Access:http://www.sciencedirect.com/science/article/pii/S2405630824001393
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author Andreas Schmidt
David Baumann
Ulf Lamprecht
Benjamin Mayer
Cristian Urla
Benjamin Bender
Jürgen Schäfer
Frank Fideler
Maximilian Niyazi
Frank Paulsen
Jörg Fuchs
author_facet Andreas Schmidt
David Baumann
Ulf Lamprecht
Benjamin Mayer
Cristian Urla
Benjamin Bender
Jürgen Schäfer
Frank Fideler
Maximilian Niyazi
Frank Paulsen
Jörg Fuchs
author_sort Andreas Schmidt
collection DOAJ
description Background: Perianal rhabdomyosarcoma ((P)RMS) are rare and have a poor prognosis. Data in young children are limited and local therapy is not well defined. Combined brachytherapy and surgery is routinely being used for RMS at other sites in children as it provides good oncologic outcomes and allows for organ-sparing surgery. The objective of this study was to evaluate this combination treatment for local tumor control and organ-sparing surgery in young children with (P)RMS. Methods: A retrospective review of the medical records of all children who underwent surgery and brachytherapy for (P)RMS at our institution since 2009 was conducted. Results: Surgery for (P)RMS was performed in 6 patients at a median age of 19 months (range 8–50). Embryonal RMS was diagnosed in 4 patients and alveolar RMS in 2 patients, of which 1 patient had FOXO1 fusion-positive RMS. All patients underwent postoperative high-dose rate (HDR) brachytherapy. Organ-preserving surgery was achieved in 5 of 6 patients (83 %). In 1 patient, the entire sphincter was infiltrated, making organ-preserving resection impossible. 5 of 6 patients (83 %) exhibited an event-free and overall survival at a median follow-up of 26 months (range 8–107). One patient died due to locoregional recurrence. Complications were urethral leakage in 1 patient followed by urethral stenosis and delayed wound healing and vaginal stenosis in another patient. No patient reported fecal incontinence. Conclusions: Combined treatment with surgery and HDR brachytherapy is feasible in very young children with (P)RMS and leads to a favorable oncologic outcome. Preliminary data show a good functional preservation.
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spelling doaj-art-ac5b0fdcd13a4dc598692c06be82118e2025-08-20T02:18:20ZengElsevierClinical and Translational Radiation Oncology2405-63082024-11-014910086210.1016/j.ctro.2024.100862A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcomaAndreas Schmidt0David Baumann1Ulf Lamprecht2Benjamin Mayer3Cristian Urla4Benjamin Bender5Jürgen Schäfer6Frank Fideler7Maximilian Niyazi8Frank Paulsen9Jörg Fuchs10Department of Pediatric Surgery and Pediatric Urology, University Children’s Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany; Corresponding author at: Department of Pediatric Surgery and Pediatric Urology, University Children’s Hospital, Eberhard Karls University Tuebingen, Hopppe-Seyler-Strasse 3, 72076 Tuebingen, Germany.Department of Radiation Oncology, University Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Radiation Oncology, University Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Pediatric Surgery and Pediatric Urology, University Children’s Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Pediatric Surgery and Pediatric Urology, University Children’s Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Diagnostic and Interventional Neuroradiology, University Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Pediatric Radiology, University Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Anesthesiology and Intensive Care Medicine, University Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Radiation Oncology, University Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Radiation Oncology, University Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Pediatric Surgery and Pediatric Urology, University Children’s Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyBackground: Perianal rhabdomyosarcoma ((P)RMS) are rare and have a poor prognosis. Data in young children are limited and local therapy is not well defined. Combined brachytherapy and surgery is routinely being used for RMS at other sites in children as it provides good oncologic outcomes and allows for organ-sparing surgery. The objective of this study was to evaluate this combination treatment for local tumor control and organ-sparing surgery in young children with (P)RMS. Methods: A retrospective review of the medical records of all children who underwent surgery and brachytherapy for (P)RMS at our institution since 2009 was conducted. Results: Surgery for (P)RMS was performed in 6 patients at a median age of 19 months (range 8–50). Embryonal RMS was diagnosed in 4 patients and alveolar RMS in 2 patients, of which 1 patient had FOXO1 fusion-positive RMS. All patients underwent postoperative high-dose rate (HDR) brachytherapy. Organ-preserving surgery was achieved in 5 of 6 patients (83 %). In 1 patient, the entire sphincter was infiltrated, making organ-preserving resection impossible. 5 of 6 patients (83 %) exhibited an event-free and overall survival at a median follow-up of 26 months (range 8–107). One patient died due to locoregional recurrence. Complications were urethral leakage in 1 patient followed by urethral stenosis and delayed wound healing and vaginal stenosis in another patient. No patient reported fecal incontinence. Conclusions: Combined treatment with surgery and HDR brachytherapy is feasible in very young children with (P)RMS and leads to a favorable oncologic outcome. Preliminary data show a good functional preservation.http://www.sciencedirect.com/science/article/pii/S2405630824001393Perianal rhabdomyosarcomaBrachytherapySurgeryLocal therapyYoung children
spellingShingle Andreas Schmidt
David Baumann
Ulf Lamprecht
Benjamin Mayer
Cristian Urla
Benjamin Bender
Jürgen Schäfer
Frank Fideler
Maximilian Niyazi
Frank Paulsen
Jörg Fuchs
A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma
Clinical and Translational Radiation Oncology
Perianal rhabdomyosarcoma
Brachytherapy
Surgery
Local therapy
Young children
title A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma
title_full A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma
title_fullStr A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma
title_full_unstemmed A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma
title_short A pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma
title_sort pilot study to evaluate the combination of surgery and brachytherapy for local tumor control in young children with perianal rhabdomyosarcoma
topic Perianal rhabdomyosarcoma
Brachytherapy
Surgery
Local therapy
Young children
url http://www.sciencedirect.com/science/article/pii/S2405630824001393
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