Primitive Neuroectodermal Tumors and Other Sarcomas of the Kidney

Although extremely rare, renal sarcomas can cause high rates of morbidity and death. The diagnosis of these entities remains challenging and findings illustrate the importance of obtaining a preoperative biopsy given the various range of prognosis. Furthermore, there is no standardized treatment for renal sarcomas, and evidence from the literature to help clinicians manage renal sarcomas is limited. Here, we summarize the clinical knowledge about the major clinicopathological characteristics, outcomes, and management of primary renal primitive neuroectodermal tumors and other rare types of kidney sarcomas, including leiomyosarcoma, liposarcoma, hemangiopericytoma, fibrosarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma, and primary renal synovial sarcoma.