Acute teratoid rhabdoid tumor

A rhabdoid tumor is a rare and highly malignant tumor of childhood, first described in 1978. These tumors were initially considered an aggressive variant of Wilms' tumor of the kidney, however, with newer diagnostic techniques, these tumors are believed to represent a distinct entity. Since that time, there have been fewer than 50 cases reported, although it is likely that some cases previously identified as medulloblastoma or as primitive neuroectodermal tumors (PNET) are in fact rhabdoid tumors.

These tumors occur in young children, mean age at diagnosis of 3.5 years, with a range of 2 to 13 years. There are no reported cases in adults. Rhabdoid tumors occur equally in males and females. The location can be supratentorial, intraventicular, and infratentorial.

As you read further, you will find general information about rhabdoid tumors. If you would like to view summary information about brain tumors first, see the overview on brain tumors.