International Journal of Radiation Oncology*Biology*Physics
Clinical InvestigationAdult Rhabdomyosarcoma Survival Improved With Treatment on Multimodality Protocols
Introduction
Rhabdomyosarcoma (RMS) is a rare malignancy, with an incidence of 4.5 per million people younger than 20 years in the United States. It is largely a disease of childhood; more than 50% of cases are diagnosed before the age of 10 (1). Whereas the 5-year overall survival (OS) rates of RMS in the pediatric population have improved in recent years to approximately 70%, the survival in adult populations with RMS is consistently lower, with 5-year OS rates ranging from 40% to 54% (2) In many pediatric studies, age itself is identified as a poor prognostic feature, with children older than 10 demonstrating lower rates of survival than children 1 to 9 years of age (3). Some of this difference in outcome between children and adults has been attributed to increased incidence of poor prognostic features in adults such as unfavorable primary site, unfavorable histologic diagnosis, and higher rates of regional and distant spread (4). The extent to which discrepancies in the treatment of the pediatric and adult populations with RMS plays a role in the lower survival rates among adults is unknown (5).
To better understand the role of demographic, clinical, and treatment variables in adult patients with RMS, we analyzed a large cohort of patients with RMS with a focus on both prognostic variables and treatment parameters.
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Patients
We analyzed data from all patients who presented to Memorial Sloan-Kettering Cancer Center (MSKCC) between 1990 and 2011 and received diagnoses of RMS at age 16 or older. Details regarding demographic features, tumor characteristics, surgery, radiation, and chemotherapy were collected from the electronic medical records and the patients' charts. The variables that were analyzed included age at diagnosis, histologic diagnosis, primary site distribution, Children's Oncology Group (COG) risk
Patients
Of the 148 patients who met the study criteria, 10 were excluded for lack of adequate data. The median follow-up time was 43 months (range, 1-254 months) for surviving patients. Demographic and clinical characteristics are shown in Table 1. The median age was 28 years (range, 16-86 years). Embryonal tumors were present in 74 patients (54%), alveolar in 45 patients (33%), pleomorphic in 16 patients (12%), and NOS in 3 patients (2%). The majority of patients had an unfavorable primary site: 24
Discussion
Rhabdomyosarcoma is the most common pediatric sarcoma, and large multi-institutional trials have detailed its biologic and clinical characteristics. In particular, the Intergroup Rhabdomyosarcoma Studies (IRS) provide long-term survival data on thousands of children (<21 years of age) 3, 6, 7, 8. With regard to adult RMS, much of the published literature is from single-institution series, which report on clinical parameters and survival data for adults with RMS.
The data from these large
Conclusions
The results of the current study indicate that adults with RMS have increased rates of poor prognostic features such as unfavorable primary site. For the entire cohort, OS is poor in comparison with pediatric survival rates, as demonstrated in multiple large-scale studies. However, for a subset of patients with nonmetastatic disease treated on prospective RMS protocols, survival is significantly improved and approaches the survival rates seen in the pediatric population.
Acknowledgment
The authors thank Lawrence A. Herman for editorial assistance.
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Conflict of interest: none.