Gastrointestinal stromal tumor pathophysiology

Gastrointestinal stromal tumor Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

On microscopic histopathological analysis, spindle cells or plump epithelioid cells are characteristic findings of gastrointestinal stromal tumor.

Pathophysiology

• GIST can occur in any part of the gastrointestinal tract but the most common location is stomach with the second most common location as small intestine. Less frequent sites of occurrence include the colon, rectum and esophagus. Rare sites include pancreas, omentum, or mesentery.
• GIST can arises from the submucosal layer or the smooth muscle cells of the GI tract.
• GIST tumors can either be benign or malignant. They can be any size. GIST can grow as an endophytic or exophytic lesions.
  • Endophytic lesions are linear lesions that grow along the lumen of the affected organ.
  • Exophytic lesions can present as a protruding outgrowth outside the lumen of GI tract.
• GISTs are thought to arise from interstitial cells of Cajal (ICC), that are normally part of the autonomic nervous system of the intestine. They serve a pacemaker function in controlling motility.

• GISTs are believed to arise from the interstitial cells of Cajal, with 95% staining positive for CD117 (c-KIT) and 70% for CD34. The former is a tyrosine kinase growth factor receptor and the target of ST-571 (Imatinib; Glivec).

• Macroscopically these tumours are rounded with frequent hemorrhagic change. Larger tumours also may demonstrate necrosis and cystic change. Size is variable ranging form 1 to 30cm.

• Histology demonstrates a relatively cellular tumor comprised of spindle cells (70-80%) and or plump epithelioid cells (20-30%). They appear to arise from the muscularis propria layer.

Gastrointestinal stromal tumor of stomach. Courtesy of Ed Uthman, MD.

Endoscopic image of GIST in fundus of stomach, seen on retroflexion.

Same GIST seen on forward view of the endoscope showing overlying clot.

Overview

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR

[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Pathophysiology

Pathogenesis

• The exact pathogenesis of [disease name] is not fully understood.

OR

• It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
• [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
• Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
• [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
• The progression to [disease name] usually involves the [molecular pathway].
• The pathophysiology of [disease/malignancy] depends on the histological subtype.

Genetics

• [Disease name] is transmitted in [mode of genetic transmission] pattern.
• Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
• The development of [disease name] is the result of multiple genetic mutations.

Gross Pathology

• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

• On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

References

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