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Purpose:  Given the poor prognosis and the relative rarity of patients diagnosed with limb rhabdomyosarcoma (LRMS) and metastasis at diagnosis, we performed this study to reveal distinctive clinical features and evaluated prognostic factors of this special population in order to provide appropriate treatment.   Patients and Methods:  We carried out retrospective research of patients diagnosed with LRMS and metastasis from 1975 to 2016 using the Surveillance, Epidemiology, and End Results (SEER) program database. Survival curves were generated by applying the Kaplan–Meier method. In terms of evaluating and determining independent predictors of survival, we conducted univariate and multivariate survival analyses using the Cox proportional hazard regression model.   Results:  This retrospective analysis contained a series of 245 patients with metastatic LRMS, with male predominance (male vs. female, 1.6:1). Nearly half of the patients were diagnosed with alveolar rhabdomyosarcoma (44.9%). According to the results of the univariate and multivariate analyses, younger age, tumor subtype, and radiotherapy were found to be significantly associated with improved overall survival (OS) and cause-specific survival (CSS).   Conclusions:  Patients with LRMS and metastasis at diagnosis experienced a quite poor prognosis. Age at diagnosis, tumor subtype, and radiotherapy can help clinicians to better estimate the prognosis. This study indicated that local radiotherapy can provide a survival benefit.

Population-Based Survival Analysis of Patients With Limb Rhabdomyosarcoma and Metastasis at Diagnosis