Gastrointestinal stromal tumors: Recent advances in understanding of their biology

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. Howe...

Full description

Saved in:
Bibliographic Details
Published in:Human pathology 1999-10, Vol.30 (10), p.1213-1220
Main Authors: Miettinen, Markku, Sarlomo-Rikala, Maarit, Lasota, Jerzy
Format: Article
Language:English
Subjects:
Animals
Autonomic Nervous System - pathology
Biological and medical sciences
Biomarkers, Tumor - metabolism
c-kit
Esophageal Neoplasms - diagnosis
Esophageal Neoplasms - metabolism
Esophageal Neoplasms - pathology
Gastroenterology. Liver. Pancreas. Abdomen
Gastrointestinal Neoplasms - diagnosis
Gastrointestinal Neoplasms - genetics
Gastrointestinal Neoplasms - metabolism
Gastrointestinal Neoplasms - pathology
genetics
GIST
Humans
Immunohistochemistry
Leiomyoma - pathology
leiomyosarcoma
Leiomyosarcoma - pathology
Medical sciences
Neurilemmoma - pathology
pathology
Peripheral Nervous System Neoplasms - pathology
Prognosis
Proto-Oncogene Proteins c-kit - genetics
Stomach. Duodenum. Small intestine. Colon. Rectum. Anus
Stromal Cells - metabolism
Stromal Cells - pathology
Tumors
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0046-8177
1532-8392
DOI:10.1016/S0046-8177(99)90040-0