Abdelhafeez H. Abdelhafeez, Tea Reljic, Farina Klocksieben, Ambuj Kumar, Sharon Cox, Andrew M. Davidoff, Kudzayi Munanzvi, C. E. J. (Sheila) Terwisscha van Scheltinga, Ahmed Elgendy, J. Ted Gerstle, Bilal Qureshi, Abdulrasheed Nasir, Timothy B. Lautz, Amabelle A. Moreno, Amos Loh, Sajid Qureshi, Gordan M. Vujanić, Pablo Lobos, Sheena Mukkada, Simone Abib
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Abstract
Background
Surgery remains the cornerstone of treatment for rhabdomyosarcoma (RMS) in children. However, there is considerable variation in surgical management practices worldwide, highlighting the need for standardized Clinical Practice Guidelines (CPG).
Methods
The CPG development involved assembling a multidisciplinary group, prioritizing 10 key topic areas, conducting evidence searches, and synthesizing findings. Recommendations were voted on using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluations) methodology.
Recommendations
The panel recommended regional lymph node evaluation for patients with paratesticular RMS who are more than 10 years old and extremity RMS. Other suggestions included pretreatment re-excision for incompletely resected RMS, preoperative radiation therapy for unresectable tumors, maintaining a 0.5 cm resection margin, and tumor bed marking with surgical clips. The panel also suggests resection of residual metastatic disease following chemotherapy, resection of relapsed disease, and the least invasive approach for managing patients presenting with obstruction.
Conclusion
This CPG provides evidence-based surgical management recommendations for RMS that can be adapted to diverse resource settings.
期刊介绍:
Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.