Case for website

6-month-old male child who presented with an abdominal mass and hematuria for 2 months in th surgical OPD.CT abdomen suggested Neoplastic lesion of the kidney.-Rhabdoid tumor Kidney

Gross specimen show grey black variegated renal mass destroying the renal parenchyma

H&E stained sections show atypical rhabdoid cells on 40X

The most aggressive renal tumor of young age.
Highly malignant undifferentiated tumor composed of noncohesive cells with large, eccentric nuclei, prominent nucleoli and intracytoplasmic inclusions; characterized by loss of INI1 immunostaining and SMARCB1genetic abnormalities (in ~95% of cases).
The majority (~95%) of rhabdoid tumors of kidney have genetic changes resulting in the loss of function of SMARCB1, a tumor suppressor gene encoding BAF47 (INI1), which is a core member of the BAF chromatin remodeling complex
No established standard treatment.
Most patients are treated with intensive multimodal regimens; surgical resection, intensive multidrug chemotherapy regimens and local radiotherapy or high dose chemotherapy followed by autologous stem cell rescue