Daniel S Rhee1, David A Rodeberg2, Reto M Baertschiger3, Jennifer H Aldrink4, Timothy B Lautz5, Christa Grant6, Rebecka L Meyers7, Elisabeth T Tracy8, Emily R Christison-Lagay9, Richard D Glick10, Peter Mattei11, Roshni Dasgupta12. 1. Division of Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address: drhee1@jhmi.edu. 2. Division of Pediatric Surgery, Department of Surgery, East Carolina University, Greenville, NC. 3. Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON. 4. Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH. 5. Division of Pediatric Surgery, Ann & Robert H Lurie Children's Hospital of Chicago, Northwestern University, Chicago, IL. 6. Division of Pediatric Surgery, Penn State Children's Hospital, Hershey, PA. 7. Division of Pediatric Surgery, University of Utah, Primary Children's Hospital, Salt Lake City, UT. 8. Division of Pediatric Surgery, Department of Surgery, Duke University Medical Center, Durham, NC. 9. Division of Pediatric Surgery, Department of Surgery, Yale University School of Medicine, Yale New Haven Children's Hospital, New Haven, CT. 10. Division of Pediatric Surgery, Zucker School of Medicine at Hofstra/Northwell, Cohen Children's Medical Center, New Hyde Park, NY. 11. Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA. 12. Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Abstract
BACKGROUND/ PURPOSE: Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults and requires multimodality treatment. The purpose of this review is to present an update on risk stratification as well as surgical and medical management strategies in pediatric rhabdomyosarcoma. METHODS: A comprehensive review of the current literature on pediatric rhabdomyosarcoma, including the most recent Children's Oncology Group studies and several international collaboratives, was performed by the authors and key findings were summarized in the manuscript. RESULTS: FOXO1 fusion status is a stronger prognostic factor than histology and is now used for risk stratification in treatment protocols. For assessment of regional nodal involvement, FDG-PET-CT shows poor sensitivity and specificity to detect histologically confirmed nodal metastasis. Thus, surgical assessment of regional lymph nodes is required for rhabdomyosarcoma of the extremities or trunk as well as paratesticular rhabdomyosarcoma in patients ≥10 years of age, although adherence to surgical guidelines remains poor. Hemiscrotectomy performed for scrotal violation in paratesticular rhabdomyosarcoma has not shown an improvement in event free survival and is not recommended. CONCLUSIONS: Surgical and medical treatment strategies for rhabdomyosarcoma in children continue to evolve. This review provides current evidence-based treatment standards with an emphasis on surgical care. TYPE OF STUDY: Review. LEVEL OF EVIDENCE: Level IV.
BACKGROUND/ PURPOSE:Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults and requires multimodality treatment. The purpose of this review is to present an update on risk stratification as well as surgical and medical management strategies in pediatric rhabdomyosarcoma. METHODS: A comprehensive review of the current literature on pediatric rhabdomyosarcoma, including the most recent Children's Oncology Group studies and several international collaboratives, was performed by the authors and key findings were summarized in the manuscript. RESULTS:FOXO1 fusion status is a stronger prognostic factor than histology and is now used for risk stratification in treatment protocols. For assessment of regional nodal involvement, FDG-PET-CT shows poor sensitivity and specificity to detect histologically confirmed nodal metastasis. Thus, surgical assessment of regional lymph nodes is required for rhabdomyosarcoma of the extremities or trunk as well as paratesticular rhabdomyosarcoma in patients ≥10 years of age, although adherence to surgical guidelines remains poor. Hemiscrotectomy performed for scrotal violation in paratesticular rhabdomyosarcoma has not shown an improvement in event free survival and is not recommended. CONCLUSIONS: Surgical and medical treatment strategies for rhabdomyosarcoma in children continue to evolve. This review provides current evidence-based treatment standards with an emphasis on surgical care. TYPE OF STUDY: Review. LEVEL OF EVIDENCE: Level IV.
Authors: Bin Xu; Albert J H Suurmeijer; Narasimhan P Agaram; Lei Zhang; Cristina R Antonescu Journal: Histopathology Date: 2021-05-19 Impact factor: 7.778