Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data.

OBJECTIVE

To evaluate trends in incidence, survival, and treatment of rhabdomyosarcoma (RMS) of the head and neck.

STUDY DESIGN

Retrospective review of a national database.

SETTING

Tertiary medical center.

SUBJECT AND METHODS

Incidence and survival trends were examined for head and neck RMS diagnosed between 1973 and 2007 using the Surveillance, Epidemiology, and End Results Program. Frequencies, incidence rates, and relative survival curves were calculated for various RMS subtypes and primary sites.

RESULTS

Between 1973 and 2007, the incidence of RMS of the head and neck increased significantly, with an annual percentage change of 1.16%. Relative 5-year survival was statistically unchanged during the study period at 62.8% ± 2.3%. When analyzed by univariate analysis, overall survival was found to be dependent on sex, age, primary site, extent of disease, and histology. When evaluated by stage, most orbital tumors (60.6%) presented with localized disease, while most parameningeal tumors presented with either regional (53.2%) or distant (28.1%) spread. Multivariate analysis found that age less than 10 years at diagnosis and tumors with localized or regional spread were associated with improved overall survival. Relative survival was found to be largely dependent on extent of disease rather than primary site.

CONCLUSIONS

Despite reported advances in overall and disease-free survival for patients with RMS, population-based analysis shows no substantial improvement during the past 30+ years. The prognosis of these patients is largely dependent on extent of disease at diagnosis.