Cheng H, et al. - In this retrospective medical record review, researchers examined 53 patients with pathologically confirmed malignant rhabdoid tumor (MRT) in order to gain a better understanding of the extracranial MRT, which represents a rare and highly aggressive tumor that occurs most commonly in infants and young children. Overall 53 patients received a diagnosis of MRT at a median age of 16 months. Among these, malignant rhabdoid tumor of the kidney (MRTK) was diagnosed in 32 cases and extrarenal extracranial rhabdoid tumor (EERT) in 21 cases. For the overall cohort, 23.71% and 18.44% were the estimated overall survival rates of 3 years and 5 years, respectively. Among patients managed with standard chemotherapy, total resection, and radiotherapy, statistically significant disparities in survival were evident. Overall, the highly aggressive nature of extracranial MRT was still evident in children. A poor outcome due to rapid progression or recurrence of the tumor was most likely to be seen among younger patients and those suffering from metastatic disease.