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Related Experiment Videos

[Inflammatory myofibroblastic tumors].

Nicolas Sirvent1, Jean-Michel Coindre, Florence Pedeutour

  • 1Service de Pédiatrie, Hôpital de l'Archet, CHU de Nice, 06202 Nice Cedex 3.

Annales De Pathologie
|February 21, 2003
PubMed
Summary

Inflammatory myofibroblastic tumors (IMT) are characterized by specific ALK gene rearrangements, indicating a neoplastic origin. Fluorescence in situ hybridization (FISH) aids in diagnosing these tumors by detecting ALK alterations.

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Area of Science:

  • Oncology
  • Genetics
  • Pathology

Background:

  • Inflammatory myofibroblastic tumors (IMT) are mesenchymal neoplasms often affecting children and young adults.
  • IMT are characterized by myofibroblastic cell proliferation with inflammatory cell infiltration.
  • Recent studies suggest a neoplastic basis for IMT, moving away from a purely reactive inflammatory model.

Purpose of the Study:

  • To investigate the role of specific gene alterations in IMT tumorigenesis.
  • To determine if IMT development involves neoplastic processes.
  • To explore the significance of ALK gene rearrangements in IMT.

Main Methods:

  • Cytogenetic and molecular analyses to identify chromosomal abnormalities.
  • Investigated rearrangements of the ALK gene at chromosomal band 2p23.
  • Utilized immunohistochemistry on paraffin-embedded tissues to detect ALK protein expression.
  • Employed fluorescence in situ hybridization (FISH) to search for ALK gene rearrangements.

Main Results:

  • Identified non-random abnormalities of chromosomal band 2p23, specifically rearrangements of the ALK gene in IMT.
  • Found similarities in ALK breakpoints and fusion partners between IMT and anaplastic large-cell lymphomas (ALCL).
  • Observed positive cytoplasmic ALK expression in approximately half of IMT cases with molecular ALK rearrangements.
  • Demonstrated that ALK gene rearrangements occur in both hematopoietic (ALCL) and mesenchymal (IMT) cell lines.

Conclusions:

  • Novel molecular data define a subset of IMT as neoplastic, characterized by ALK alterations.
  • Constitutive activation of ALK due to gene rearrangements plays a central role in IMT development.
  • FISH analysis for ALK rearrangements is a valuable diagnostic tool for IMT.

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