Establishment and characterization of malignant rhabdoid tumor of the kidney

Oncol Rep. 2001 Jan-Feb;8(1):43-8.

Abstract

Malignant rhabdoid tumor of the kidney (MRTK) is a highly aggressive tumor which occurs in childhood and which is histologically characterized by the existence of eosinophilic intracytoplasmic inclusions. We established and characterized a cell line from this tumor with histological, immunohistochemical and cytogenetical analysis. Histologically, the tumor cells demonstrate typical eosinophilic inclusions, while immunohistochemically the cells demonstrate common mesenchymal and epithelial differentiation. Although the conventional karyotyping of this tumor lacked the abnormalities of 22q chromosome, Southern blot analysis and microsatellite analysis verified abnormalities of the BCR gene and of the hSNF5/INI1 gene. Despite the variety of locations, these common genetic abnormalities appear to contribute to distinguish rhabdoid tumor from such other small round cell tumors as primitive neuroectodermal tumor, rhabdomyosarcoma, poorly differentiated synovial sarcoma and desmoplastic small round cell tumor.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Blotting, Southern
  • Cell Differentiation
  • Chromosomal Proteins, Non-Histone
  • DNA-Binding Proteins / genetics
  • Humans
  • Inclusion Bodies / ultrastructure
  • Infant
  • Karyotyping
  • Keratins / analysis
  • Kidney Neoplasms / genetics
  • Kidney Neoplasms / pathology*
  • Loss of Heterozygosity
  • Male
  • Membrane Proteins*
  • Mice
  • Mice, Nude
  • Microsatellite Repeats
  • Mucin-1 / analysis
  • Neoplasm Proteins / analysis
  • Neoplasm Proteins / genetics
  • Neoplasm Transplantation
  • Phosphopyruvate Hydratase / analysis
  • Polymerase Chain Reaction
  • Protozoan Proteins / analysis
  • Rhabdoid Tumor / genetics
  • Rhabdoid Tumor / pathology*
  • S100 Proteins / analysis
  • SMARCB1 Protein
  • Transcription Factors
  • Transplantation, Heterologous
  • Tumor Cells, Cultured* / chemistry
  • Tumor Cells, Cultured* / pathology
  • Tumor Cells, Cultured* / transplantation
  • Tumor Suppressor Protein p53 / analysis
  • Vimentin / analysis

Substances

  • Chromosomal Proteins, Non-Histone
  • DNA-Binding Proteins
  • MIC2 protein, Toxoplasma gondii
  • Membrane Proteins
  • Mucin-1
  • Neoplasm Proteins
  • Protozoan Proteins
  • S100 Proteins
  • SMARCB1 Protein
  • SMARCB1 protein, human
  • Smarcb1 protein, mouse
  • Transcription Factors
  • Tumor Suppressor Protein p53
  • Vimentin
  • Keratins
  • Phosphopyruvate Hydratase