Resumen
In this article, we review the clinical features of advanced PEComas and show the diversity of reported data among authors, emphasizing the heterogeneity of molecular characterization and treatment strategy. Based on clinical data collected from 124 case reports, metastatic disease at diagnosis and a grouped version of the Bleeker?s risk category were the only factors significantly associated with death. Due to a significant number of missing data or short follow-ups, results regarding prognostic factors should, however, be interpreted with caution.