Rhabdomyosarcoma in children: a SEER population based study

Abstract

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.