Rhabdoid Tumour of the Kidney
A highly malignant renal neoplasm of infancy characterised by cells with vesicular nuclei, prominent nucleoli, and eosinophilic cytoplasmic inclusions, typically associated with SMARCB1 (INI1) loss.
Full Definition
Rhabdoid tumour of the kidney (RTK) is one of the most aggressive paediatric renal tumours, occurring predominantly in infants under two years of age. Histologically, the tumour cells display eccentric nuclei, prominent 'owl-eye' nucleoli, and glassy cytoplasmic inclusions formed by whorled intermediate filaments. Loss of SMARCB1/INI1 protein expression by immunohistochemistry is a defining diagnostic feature. RTK is genetically and morphologically related to atypical teratoid/rhabdoid tumour of the central nervous system. Editors working with paediatric oncology reports must distinguish it from Wilms tumour and other small round blue cell tumours.
Usage
Usage note: Do not abbreviate to 'rhabdoid tumour' without qualification, as the term also applies to CNS and extrarenal soft-tissue sites.
In Context
- "The synoptic report confirmed rhabdoid tumour of the kidney with loss of INI1 nuclear expression on immunohistochemistry." — Surgical pathology report
- "Editors should note that rhabdoid tumour of the kidney is classified separately from Wilms tumour in all current paediatric oncology protocols." — Editorial style guide