Linitis plastica pathophysiology

Linitis plastica Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Linitis plastica from Other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Staging
Diagnostic study of choice
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Linitis plastica pathophysiology On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Linitis plastica pathophysiology All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Linitis plastica pathophysiology
CDC on Linitis plastica pathophysiology
Linitis plastica pathophysiology in the news
Blogs on Linitis plastica pathophysiology
Directions to Hospitals Treating Linitis plastica
Risk calculators and risk factors for Linitis plastica pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]

Overview

On gross pathology, thick, rigid, leather bottle-like stomach from diffuse infiltration of the stomach wall by the tumor cells are characteristic findings of linitis plastica. On microscopic histopathological analysis, atypical signet ring cells diffusely infiltrating the stomach wall, submucosal fibrosis and thickening, with minimal mucosal involvement are characteristic findings of linitis plastica. Development of linitis plastica is the result of genetic mutation in the CDH1 (E-cadherin) gene, that is responsible for intercellular adhesions.

Pathophysiology

• Linitis plastica is a morphological variant of diffuse (or infiltrating) gastric adenocarcinoma (stomach cancer).
• Adenocarcinoma is the most common type of gastric cancer accounting to almost 90% of the same, of which about 10% constitute linitis plastica.

Genetics

• Germline mutations in the CDH1 (E-cadherin) gene, resulting in altered intercellular adhesions, may be involved in the pathogenesis of diffuse gastric adenocarcinoma.
• Hereditary form of diffuse gastric adenocarcinoma is transmitted in an autosomal dominant fashion, with a 70% disease penetrance.

Gross pathology

• On gross pathology, thick, rigid, leather bottle-like stomach from diffuse infiltration of the stomach wall by the tumor cells are characteristic findings of linitis plastica.
• As a result of the tumor cell infiltration and fibrosis, there is diffuse thickening and rigidity of the gastric wall, making the process of digestion difficult.
• Metastasis to the peritoneum, lymph nodes and/or other organs usually occurs by the time linitis plastica is diagnosed. Linitis plastica may also occur secondary to metastasis from other primary cancers, particularly infiltrating lobular carcinoma of breast.

• 

  Endoscopic image of linitis plastica, a type of stomach cancer where the entire stomach is invaded, leading to a leather bottle-like appearance with blood coming out of it.

Microscopic pathology

• In diffuse type adenocarcinoma (mucinous, colloid), tumour cells are discohesive and secrete mucus, which is delivered in the interstitium, producing large pools of mucus or colloid (optically "empty" spaces). If the mucus remains inside the tumour cell, it pushes the nucleus to the periphery, leaving a "signet-ring cell" appearance.
• On microscopic histopathological analysis, poorly differentiated, atypical signet ring cells diffusely infiltrating the stomach wall, submucosal fibrosis and thickening, with minimal mucosal involvement are characteristic findings of linitis plastica.
• Superficial mucosal layers are usually spared and rarely show superficial ulcers on endoscopy, that has a wide differential diagnosis, making the diagnosis even more difficult. The biopsies are falsely negative, as the disease remains mostly in the submucosa and muscularis propria.
• Although the lower mucosal and submucosal layers are mostly involved, the muscular and subserosal layers may also be infiltrated with the neoplastic cells.

References

Template:WikiDoc Sources