A Review of the Heterogeneity of the Subgroups of Atypical Teratoid/Rhabdoid Tumors and its Role in Slowing Therapeutic Advances

Abstract

One of the most common brain tumors found in infants is the Atypical Teratoid/Rhabdoid Tumor (AT/RT). This tumor is responsible for up to 20% of brain cancer cases in children less than three years old. AT/RT is typically summarized by the loss of part of chromosome 22 resulting in the loss of the SMARCB1/INI-1 gene. The tumor has shown large clinical heterogeneity suggesting possible molecular heterogeneity which has played a large role in slowing therapeutic advances.  Recent clinicopathological analysis and bisulfite sequencing studies have found that there are three subgroups of AT/RT that present different clinicopathologic and epigenetic interactions. These studies may give insight into future therapeutic targets and treatment results for patients with AT/RT. Further studies are needed to help identify these subgroup-specific targets before finding potential treatments for this fatal tumor.