Rhabdoid tumor of the kidney (RTK) is extremely rare accounting for 1.5 to 3.7% of pediatric renal tumors. RTK is extremely aggressive leading to the worst outcomes seen in this tumor group with an overall survival ranging from 22 to 33% at four to five years. RTK was first described as a distinct tumor from Wilms tumor by Haas and colleagues in 1981 [1]. They called it rhabdoid tumor because histologically the cells resemble rhabdomyoblasts. Rhabdoid tumors can occur in multiple locations including the central nervous system and extracranial sites. Among extracranial sites, the kidney accounts for 48% of cases, the liver for 13% and the rest occurring in the soft tissues of the head, neck, trunk and extremities [2]. Several studies have shown that survival is uniformly poor and is not site dependent [3][4].
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