Linitis plastica is a widely used term for Brinton's disease (also known as leather bottle stomach), a morphological variant of diffuse (or infiltrating)

stomach cancer Stomach cancer, also known as gastric cancer, is a cancer that develops from the lining of the stomach. Most cases of stomach cancers are gastric carcinomas, which can be divided into a number of subtypes, including gastric adenocarcinomas. Lymph ...

. In some texts, the term is also used to describe the condition of a rigid, non-distensible stomach which may be caused by a non-malignant condition such as a caustic injury to the stomach. Linitis plastica is a type of

adenocarcinoma Adenocarcinoma (; plural adenocarcinomas or adenocarcinomata ) (AC) is a type of cancerous tumor that can occur in several parts of the body. It is defined as neoplasia of epithelial tissue that has glandular origin, glandular characteristics, or ...

and accounts for 3–19% of gastric adenocarcinomas. Causes of cancerous linitis plastica are commonly primary gastric cancer, but in rarer cases could be metastatic infiltration of the stomach, particularly breast and lung carcinoma.Robbins Basic Pathology, 8th Edition It is not associated with ''

H. pylori ''Helicobacter pylori'', previously known as ''Campylobacter pylori'', is a gram-negative, microaerophilic, spiral (helical) bacterium usually found in the stomach. Its helical shape (from which the genus name, helicobacter, derives) is thoug ...

'' infection or chronic gastritis. The risk factors are undefined, except for rare inherited mutations in

E-cadherin Cadherin-1 or Epithelial cadherin (E-cadherin), (not to be confused with the APC/C activator protein CDH1) is a protein that in humans is encoded by the ''CDH1'' gene. Mutations are correlated with gastric, breast, colorectal, thyroid, and ovarian ...

. The hereditary form of this cancer,

hereditary diffuse gastric cancer Hereditary diffuse gastric cancer (HDGC) is an inherited genetic syndrome most often caused by an inactivating mutation in the E-cadherin gene (CDH1) located on chromosome 16. Individuals who inherit an inactive copy of the CDH1 gene are at signi ...

, accounts for only 1–3% of gastric adenocarcinomas. Somatic mutations in this gene are found in about 50% of diffuse-type gastric carcinomas.

Signs and symptoms

Diffuse stomach cancer is characterized by the presence of poorly differentiated tumor cells. Under a microscope, these appear as

signet ring cells In histology, a signet ring cell is a cell with a large vacuole. The malignant type is seen predominantly in carcinomas. Signet ring cells are most frequently associated with stomach cancer, but can arise from any number of tissues including the ...

, meaning that mucin droplets are visible that displace the nucleus to one side. Symptoms of linitis plastica do not usually present until the disease is in an advanced stage, making early diagnosis difficult. Symptoms are similar to those of stomach cancer including: difficulty swallowing, weight loss, indigestion, and vomiting.

Notable cases

Napoleon Bonaparte Napoleon Bonaparte ; it, Napoleone Bonaparte, ; co, Napulione Buonaparte. (born Napoleone Buonaparte; 15 August 1769 – 5 May 1821), later known by his regnal name Napoleon I, was a French military commander and political leader who ...

and many members of his family are thought to have died from this type of cancer, although it is believed by others that he may have died from arsenic poisoning.

References

External links

"Carcinoma of the Stomach."
at patient.info {{Digestive system neoplasia Gastrointestinal cancer