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Published on: September 13, 2019
Rhabdomyosarcoma in children: a SEER population based study
Eduardo A Perez1, Noor Kassira, Michael C Cheung
1Division of Pediatric Surgery and Division of Surgical Oncology, DeWitt Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, Miami, Florida, USA.
Insights
Pediatric rhabdomyosarcoma (RMS) incidence is 0.44/100,000 annually. Embryonal RMS, common in young children, shows better survival rates than other types, especially with early diagnosis and surgical resection.
Area of Science:
- Pediatric Oncology
- Cancer Epidemiology
- Tumor Biology
Background:
- Rhabdomyosarcoma (RMS) is a rare pediatric soft tissue sarcoma.
- Understanding its incidence and outcomes is crucial for improving patient care.
- Histological subtypes and patient demographics significantly influence prognosis.
Purpose of the Study:
- To determine the incidence of pediatric rhabdomyosarcoma (RMS).
- To analyze the outcomes and survival rates associated with pediatric RMS.
- To identify prognostic factors influencing mortality in pediatric RMS patients.
Main Methods:
- Utilized the Surveillance, Epidemiology, and End Results (SEER) registry.
- Included patients diagnosed with RMS under 20 years of age.
- Conducted univariate and multivariate analyses to identify predictors of mortality.
Main Results:
- Identified 1544 pediatric RMS cases with an incidence of 0.44/100,000 annually.
- Embryonal RMS (67%) was more common in younger children, while alveolar and pleomorphic types were more frequent in adolescents.
- 5- and 10-year survival rates were 60% and 57%, respectively; factors like age < 10, localized disease, and embryonal type improved survival.
Conclusions:
- Embryonal rhabdomyosarcoma (RMS) is the most common pediatric subtype, presenting in younger children with a better prognosis.
- Favorable outcomes are associated with embryonal histology, favorable tumor location, age under 10, localized disease, and surgical resection.
- Non-embryonal type, unfavorable sites, lack of surgery, older age, and distant disease independently predict higher mortality.
Objective:
To examine incidence and outcomes for pediatric rhabdomyosarcoma (RMS).
Methods:
The SEER registry was examined for patients with RMS < 20 y old.
Results:
Overall, 1544 patients were identified for an incidence of 0.4414/100,000 per year. Males outnumbered females 3:2. Tumors were classified as embryonal (67%), alveolar (32%), and pleomorphic (1%). Alveolar and pleomorphic RMS were more common in adolescents, whereas embryonal type was more common in younger children (P = 0.0001). Pleomorphic (47%) and alveolar (39%) RMS commonly presented with distant disease, in contrast to embryonal (25%). Most patients had surgical resection (81%) and radiotherapy (63%). Overall, 5- and 10-y survival was 60% and 57%, respectively. Univariate analysis identified higher survival for age < 10 y, local stage, favorable site, embryonal type, <5 cm tumor size, and surgical resection. Multivariate analysis identified non-embryonal type (HR 1.451), non-favorable site (HR 1.570), no surgery (HR 1.726), age ≥ 10 y (HR 1.734), 1973-1978 diagnosis year (HR 1.730), and distant disease (HR 3.456) as independent predictors of mortality.
Conclusions:
Embryonal histology, the most common type of pediatric RMS, presents in young children and has better prognosis than alveolar or pleomorphic types. Patients with embryonal tumors, favorable tumor location, age < 10 y, localized disease, and surgical resection have improved survival.
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