Plexiform architecture in gastrointestinal stromal tumors is not restricted to succinate dehydrogenase-deficient cases

Maria Cristina Giustiniani, Valerio Papa, Maurizio Martini, Federica Castri, Tonia Cenci, Sergio Alfieri, Riccardo Ricci

Research output: Contribution to journalArticle

Abstract

Accumulating evidence reveals the heterogeneous features of gastrointestinal stromal tumors (GISTs), primarily distinguished by their various molecular triggers defining well characterized subgroups. The identification of the pathogenetic group a given GIST belongs to, in combination with the currently adopted GIST prognosticators, is pivotal for the correct management of GIST patients. Epidemiological, anatomical and morphological features are more or less strictly associated with the various possible GIST molecular pathogenesis; therefore, they can concur to addressing molecular analysis or even influence the identification of GIST subsets by themselves. This is particularly true in a cost/benefit perspective aimed at cutting the expenses of pathology labs. Under these circumstances, a correct classical pathological analysis still appears a fundamental step to achieve an optimal GIST characterization. We herein report a gastric epithelioid PDGFRA-mutant GIST displaying the multinodular/plexiform architecture distinctive of succinate dehydrogenase (SDH)-deficient GISTs. Immunohistochemistry and molecular analysis led to the correct tumor characterization. The reported case constitutes a valuable contribution to GIST pathology in that it demonstrates that multinodular/plexiform architecture is not restricted to SDH-deficient GISTs, but can be found also in PDGFRA-mutant ones; this is an event to be aware of, given the predilection for gastric location and epithelioid morphology shared by these two GIST subgroups, only the latter of which includes imatinib-sensitive cases.

Original languageEnglish
Pages (from-to)69-72
Number of pages4
JournalHuman Pathology: Case Reports
Volume13
DOIs
Publication statusPublished - Sep 1 2018

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Gastrointestinal Stromal Tumors
Succinate Dehydrogenase
Stomach
Pathology
Social Identification
Cost-Benefit Analysis
Immunohistochemistry

Keywords

  • Gastrointestinal stromal tumor
  • Multinodular architecture
  • Platelet-derived growth factor receptor alpha
  • Plexiform architecture
  • Succinate dehydrogenase

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

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title = "Plexiform architecture in gastrointestinal stromal tumors is not restricted to succinate dehydrogenase-deficient cases",
abstract = "Accumulating evidence reveals the heterogeneous features of gastrointestinal stromal tumors (GISTs), primarily distinguished by their various molecular triggers defining well characterized subgroups. The identification of the pathogenetic group a given GIST belongs to, in combination with the currently adopted GIST prognosticators, is pivotal for the correct management of GIST patients. Epidemiological, anatomical and morphological features are more or less strictly associated with the various possible GIST molecular pathogenesis; therefore, they can concur to addressing molecular analysis or even influence the identification of GIST subsets by themselves. This is particularly true in a cost/benefit perspective aimed at cutting the expenses of pathology labs. Under these circumstances, a correct classical pathological analysis still appears a fundamental step to achieve an optimal GIST characterization. We herein report a gastric epithelioid PDGFRA-mutant GIST displaying the multinodular/plexiform architecture distinctive of succinate dehydrogenase (SDH)-deficient GISTs. Immunohistochemistry and molecular analysis led to the correct tumor characterization. The reported case constitutes a valuable contribution to GIST pathology in that it demonstrates that multinodular/plexiform architecture is not restricted to SDH-deficient GISTs, but can be found also in PDGFRA-mutant ones; this is an event to be aware of, given the predilection for gastric location and epithelioid morphology shared by these two GIST subgroups, only the latter of which includes imatinib-sensitive cases.",
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AU - Giustiniani, Maria Cristina

AU - Papa, Valerio

AU - Martini, Maurizio

AU - Castri, Federica

AU - Cenci, Tonia

AU - Alfieri, Sergio

AU - Ricci, Riccardo

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