Primary renal leiomyosarcoma in adult patients: a systematic review and individual patient data analysis

The optimal management of primary renal leiomyosarcomas is unknown owing to its rarity and minimal available information about their primary, adjuvant treatment and clinical outcomes. This study systematically reviews treatment evidence and effects in terms of survival for leiomyosarcomas arising primarily from kidney, renal pelvis and renal vessels. PubMed and Embase databases were searched from inception to March 2023, with manual searches of reference lists. Two investigators independently reviewed the studies reporting management and survival outcomes of renal leiomyosarcomas. A total of 85 publications met inclusion criteria, reporting on 188 cases. The median age was 55.5 years, predominantly female [52.7