Publication: Biological and clinical review of stromal tumors in the gastrointestinal tract
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Date
2000
Authors
Nishida, T. ; Hirota, S.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Submucosal tumors of the gastrointestinal
tract (G1 tract) mainly consist of gastrointestinal
mesenchymal tumors (GIMTs) that are distributed in the
G1 tract from the esophagus through the rectum. GIMTs
include myogenic tumors, neurogenic tumors and
gastrointestinal stromal tumors (GISTs). The term
"GIST" is now preferentially used for the tumors that
express CD34 and KIT. GIMTs are composed of spindle
or epithelioid cells, and 20% to 30% show malignant
behavior, including peritonea1 dissemination and
hematogenous metastasis. KIT expression and mutations
in the c-kit gene are found only in GISTs, but not in
myogenic or neurogenic tumors. Mutation in the c-kit
gene is associated with aggressive features and poor
prognosis, and malignant GISTs frequently have
mutations in the c-kit gene. The clinicopathological
features of GISTs with or without c-kit mutations are
markedly different. Therefore, GIMTs may be divided
into four major categories based on histochemical and
genetic data: myogenic tumors; neurogenic tumors;
GISTs with c-kit mutation; and GISTs without c-kit
mutation. The origin of GISTs is not fully understood.
However, phenotypical resemblance to the interstitial
cells of Cajal (ICCs) and gain-of-function mutations in
the c-kit gene may suggest origin from ICCs andlor
multipotential mesenchymal cells that differentiate into
ICCs.
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