Atypical Teratoid/Rhabdoid Tumor

**Semantic type:** Neoplastic Process

**Definition:** An aggressive malignant embryonal neoplasm arising from the central nervous system. It is composed of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. Mutations of the SMARCB1 gene or very rarely SMARCA4 (BRG1) gene are present. The vast majority of cases occur in childhood. Symptoms include lethargy, vomiting, cranial nerve palsy, headache, and hemiplegia.

**Synonyms:** - AT/RT - AT/RT - ATRT - ATT/RHT - Atypical Teratoid/Rhabdoid Tumor (WHO Grade 4) - Atypical Teratoid/Rhabdoid Tumor (WHO Grade IV) - Atypical teratoid/rhabdoid tumor - Atypical teratoid/rhabdoid tumor - CNS Rhabdoid Neoplasm - CNS Rhabdoid Tumor - Central Nervous System Rhabdoid Neoplasm - Central Nervous System Rhabdoid Tumor - Malignant Brain Rhabdoid Neoplasm - Malignant Brain Rhabdoid Tumor - Malignant Rhabdoid Neoplasm of Brain - Malignant Rhabdoid Neoplasm of the Brain - Malignant Rhabdoid Tumor of Brain - Malignant Rhabdoid Tumor of the Brain - Primary Malignant Brain Rhabdoid Neoplasm - Primary Malignant Brain Rhabdoid Tumor - Primary Malignant Rhabdoid Neoplasm of Brain - Primary Malignant Rhabdoid Neoplasm of the Brain - Primary Malignant Rhabdoid Tumor of Brain - Primary Malignant Rhabdoid Tumor of the Brain - Rhabdoid Neoplasm of CNS - Rhabdoid Neoplasm of Central Nervous System - Rhabdoid Neoplasm of the CNS - Rhabdoid Neoplasm of the Central Nervous System - Rhabdoid Tumor of CNS - Rhabdoid Tumor of Central Nervous System - Rhabdoid Tumor of the CNS - Rhabdoid Tumor of the Central Nervous System - atypical teratoid/rhabdoid tumor