Professional
Technical
IVT
rhabdoid tumor
Pronunciation: RAB-doyd
A rare, aggressive malignant tumor characterized by loss of SMARCB1/INI1 expression, occurring predominantly in infants and young children.
Full Definition
Rhabdoid tumors are rare, highly aggressive malignant neoplasms that occur predominantly in infants and very young children. These tumors are characterized by the loss of SMARCB1 (INI1) tumor suppressor gene expression and can arise in various anatomical locations including the kidney (malignant rhabdoid tumor), brain (atypical teratoid rhabdoid tumor), and soft tissues. Despite their name, rhabdoid tumors are not related to rhabdomyosarcoma and have a generally poor prognosis.
Usage
Usage note: Distinguish from rhabdomyosarcoma, which is an entirely different entity.
In Context
- "Immunohistochemistry confirmed loss of INI1 expression consistent with rhabdoid tumor." — Pathology report
- "Rhabdoid tumors require intensive multimodal therapy due to their aggressive nature." — Treatment guidelines
Also known as
malignant rhabdoid tumor
MRT
Don't confuse with
rhabdomyosarcoma
atypical teratoid rhabdoid tumor