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Malignant rhabdoid tumor. A study with two established cell lines

S Ota1, D C Crabbe, T N Tran

  • 1Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, CA 90027.

Cancer
|May 1, 1993
PubMed
Summary

Malignant rhabdoid tumor (MRT) cells show diverse differentiation potential, suggesting a primitive origin. This research indicates MRT may be a type of primitive neuroectodermal tumor.

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Area of Science:

  • Oncology
  • Cell Biology
  • Developmental Biology

Background:

  • Malignant rhabdoid tumor (MRT) is a rare pediatric sarcoma with known phenotypic diversity.
  • This study investigates unique characteristics of MRT cells using established cell lines.

Observation:

  • Two MRT cell lines (renal and extrarenal) were analyzed using immunocytochemistry, ultrastructure, cytogenetics, and PCR.
  • Cells were treated with 12-O-tetradecanoyl phorbol-13-acetate (TPA) or transretinoic acid (RA) to induce differentiation.

Findings:

  • MRT cells exhibited neural, epithelial, and mesenchymal markers and karyotypic abnormalities (chromosome 22q11.2).
  • Extrarenal MRT cells (Tm87-16) showed neuroblastic differentiation with TPA/RA treatment, while renal MRT cells (STM91-01) suggested schwannian differentiation.

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  • Both lines expressed c-myc but not N-myc, MyoD1, tyrosine hydroxylase, or N-CAM. Chromogranin expression was induced in Tm87-16 cells by TPA.

    • Implications:

    • MRT cells display a diverse neuro-ecto-mesenchymal differentiation phenotype.
    • Results suggest MRT may originate from primitive pluripotential cells, potentially neural crest-like.
    • MRT could be classified as a subset of primitive neuroectodermal tumors.