Association for Academic Surgery
Rhabdomyosarcoma in Children: A SEER Population Based Study

https://doi.org/10.1016/j.jss.2011.03.001Get rights and content

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.

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.

Section snippets

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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