Gastrointestinal stromal tumors: recent advances in understanding of their biology.

Abstract:

:Gastrointestinal stromal tumor (GIST) is the preferred term for mesenchymal tumors specific for the gastrointestinal tract (60% in stomach, 30% small intestine, 10% elsewhere). GISTs include most tumors previously designated as leiomyoma, cellular leiomyoma, leiomyoblastoma, and leiomyosarcoma. However, in the esophagus, leiomyoma is the most common mesenchymal tumor. GISTs are composed of spindle (70%) or epithelioid (30%) cells, and 10%-30% are malignant showing intra-abdominal spread or liver metastases. They are immunohistochemically positive for c-kit (CD117), CD34, and sometimes for actin but are almost always negative for desmin and S100-protein. The malignant GISTs especially show activating mutations in the c-kit gene. GISTs and gastrointestinal autonomic nerve tumors (GANT) overlap. The cell of origin is not fully understood, but resemblance to the interstitial cells of Cajal, expression of some smooth muscle markers, and occurrence outside of the GI-tract suggest origin from multipotential cells that can differentiate into Cajal and smooth muscle cells.

journal_name

Hum Pathol

journal_title

Human pathology

authors

Miettinen M,Sarlomo-Rikala M,Lasota J

doi

10.1016/s0046-8177(99)90040-0

subject

Has Abstract

pub_date

1999-10-01 00:00:00

pages

1213-20

issue

10

eissn

0046-8177

issn

1532-8392

pii

S0046-8177(99)90040-0

journal_volume

30

pub_type

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