Association for Academic SurgeryRhabdomyosarcoma in Children: A SEER Population Based Study
Introduction
Soft-tissue sarcomas (STS) are a heterogeneous group of mesenchymal extra-skeletal malignancies that account for 8% of all neoplasms in children and adolescents [1]. Rhabdomyosarcoma (RMS) is the most common STS of childhood, accounting for over 50% of all cases and with the overwhelming majority of patients presenting by 14 y of age 2, 3.
RMS originates from the embryonal mesenchyme that ultimately gives rise to striated skeletal muscle and is further classified as embryonal, alveolar, and pleomorphic histologic subtypes. These tumor types can develop anywhere in the body as an expanding mass usually associated with pain and accompanied by specific symptoms depending on their anatomical locations. Embryonal RMS, the most frequent histologic subtype, commonly occurs in the orbit, head and neck, and genitourinary system of infants and young children. In contrast, alveolar and pleomorphic subtypes commonly arise in the extremities and trunk, and typically affect adolescents and adults [4].
In Europe and the United States, the cure rate of children with local or regional RMS has increased from 25% in 1970 to over 70% in 2001 5, 6. This improvement in outcome is largely due to multidisciplinary cooperative group trials and multimodal treatment protocols. Outcome in patients with RMS is directly influenced by such prognostic indicators as patient age, histology subtype, tumor site, tumor size, and stage of disease.
We performed a population-based analysis of data from a national cancer registry in an effort to more extensively define the incidence, outcomes, and predictors of survival for pediatric patients with RMS.
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Methods
The Surveillance, Epidemiology, and End Results (SEER) April 2008 release was used to identify all incident pediatric cases of RMS diagnosed from 1973 to 2005. Tumor location and histology was based upon topography and morphology codes according to the International Classification of Disease for Oncology, 3rd edition code. A total of 1544 cases were extracted from the database by selecting only patients < 20 y of age. There were no duplicate cases in the sample.
Patients with missing data were
Patient Demographics and Tumor Characteristics
A total of 1544 pediatric patients with RMS were identified during the 32-y study period. The incidence for RMS was 0.4414 cases per 100,000 children per year (Table 1) and highest for embryonal type. Overall incidence remained stable through the study period with an annual percent change (APC) of 0.58% (Fig. 1). Analysis by histologic type revealed that although incidence rates were stable for embryonal and pleomorphic subtypes, there has been an increase in incidence per year in alveolar RMS
Discussion
The Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute (NCI) is the largest registry of information on cancer incidence and survival in the United States [7]. The data is compiled from 17 population-based cancer registries, and encompasses approximately 26% of the U.S. population. The SEER program registers information on patient demographics, primary tumor site, tumor morphology, stage at diagnosis, first course of treatment, and follow-up for survival
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