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Long-term outcomes of surgically resected pediatric rhabdomyosarcoma patients: A 10-year experience
Andres Guillen, MD, Candace Granberg, MD, Patricio Gargollo, MD.
Mayo Clinic, Rochester, MN, USA.

Background Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood and is responsible for approximately one-half of all soft tissue sarcomas in this age group. However, they are rare, representing only 3 to 4 percent of pediatric cancers overall. Approximately 350 new cases are diagnosed in the United States each year, and the annual incidence in children, adolescents, and young adults under the age of 20 is 4.3 cases per million. The objective of this study was to profile our institution's experience with surgically resected pediatric rhabdomyosarcoma patients. Herein, we describe the clinicopathologic features, management, and short- and long-term outcomes of 87 patients treated at our clinic in the last 10 years.
MethodsA retrospective review of medical records was performed for all pediatric patients (n = 87) who underwent surgical resection of an RMS tumor between 2011 and 2021. The sample was clustered by demographic and clinical variables. Discrete variables were compared using Fisher's exact test, and continuous variables were compared using Kruskal-Wallis Test. Patients were profiled based on RMS subtype, and Kaplan-Meier analysis was performed to evaluate overall survival/progression. Difference between groups was considered as statistically significant if p < 0.05.
ResultsOf 87 patients, 26 (30%) had a genitourinary primary site, and 35 (40.2%) were female. The most common subtype of RMS was embryonal (n=54; 62.8%) then alveolar (n=28; 32.6%) and spindle cell (n=4; 4.7%). 16 (18.4%) patients died and 29 (33.3%) developed progression during the study timeframe with an average of 38.9 months without death or progression. No statistical significance was found between subtypes and development of progression or survival (p=0.21).
ConclusionIn our pediatric RMS population, we have seen an average of 38.9 months of progression-free survival after surgical resection. The difference in survival between RMS subtypes was not statistically significant. Nonetheless, further prospective studies are warranted to explore underlying factors related to therapeutical response in pediatric RMS patients.


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