Linitis plastica pathophysiology

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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-Cadgerin) gene, that is responsible for intercellular adhesions.

Pathophysiology

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.

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 ulcerations on endoscopy, that has a wide differential diagnosis, making the diagnosis even more difficult. The biopsies are falsely negative, as the disease remains mostly submucosal.
• Although the lower mucosal and submucosal layers are mostly involved, the muscular and subserosal layers may also be infiltrated with the neoplastic cells.

References

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