Linitis plastica

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Linitis plastica
Other namesLeather bottle stomach
Linitis plastica.jpg
Endoscopic image of linitis plastica, where the entire stomach is invaded with stomach cancer, leading to a leather bottle like appearance
Specialty Oncology   OOjs UI icon edit-ltr-progressive.svg
total gastrectomy, splenectomy for infiltrative gastric cancer Infiltrative (poorly cohesive) gastric cancer.jpg
total gastrectomy, splenectomy for infiltrative gastric cancer

Linitis plastica (sometimes referred to as leather bottle stomach) is a morphological variant of diffuse stomach cancer in which the stomach wall becomes thick and rigid. [1]

Contents

Linitis plastica is a type of adenocarcinoma and accounts for 3–19% of gastric adenocarcinomas. [1] 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. [2] It is not associated with H. pylori infection or chronic gastritis. The risk factors are undefined, except for rare inherited mutations in E-cadherin. The hereditary form of this cancer, hereditary diffuse gastric cancer, accounts for only 1–3% of gastric adenocarcinomas. Somatic mutations in this gene are found in about 50% of diffuse-type gastric carcinomas. [2]

Signs and symptoms

close-up, total gastrectomy, infiltrative gastric cancer Infiltrative gastric cancer showing thickened stomach wall.jpg
close-up, total gastrectomy, infiltrative gastric cancer
Endoscopic image of linitis plastica, a diffuse type of stomach cancer characterized by a thickening and rigidity of the lining of the stomach, leading to a leather bottle-like appearance with blood coming out of it Linitis plastica 2.jpg
Endoscopic image of linitis plastica, a diffuse type of stomach cancer characterized by a thickening and rigidity of the lining of the stomach, leading to a leather bottle-like appearance with blood coming out of it

Diffuse stomach cancer is characterized by the presence of poorly differentiated tumor cells. Under a microscope, these appear as signet ring cells, 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. [3]

History

The condition was defined by William Brinton in 1859. [4]

Notable cases

Napoleon Bonaparte 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. [5]

Related Research Articles

<span class="mw-page-title-main">Barrett's esophagus</span> Precancerous tissue formation in the esophagus

Barrett's esophagus is a condition in which there is an abnormal (metaplastic) change in the mucosal cells lining the lower portion of the esophagus, from stratified squamous epithelium to simple columnar epithelium with interspersed goblet cells that are normally present only in the small intestine and large intestine. This change is considered to be a premalignant condition because of its potential to further transition to esophageal adenocarcinoma, an often-deadly cancer.

<i>Helicobacter pylori</i> Species of bacteria

Helicobacter pylori, previously known as Campylobacter pylori, is a gram-negative, flagellated, helical bacterium. Mutants can have a rod or curved rod shape that exhibits less virulence. Its helical body is thought to have evolved to penetrate the mucous lining of the stomach, helped by its flagella, and thereby establish infection. The bacterium was first identified as the causal agent of gastric ulcers in 1983 by Australian physician-scientists Barry Marshall and Robin Warren. In 2005, they were awarded the Nobel Prize in Physiology or Medicine for their discovery.

<span class="mw-page-title-main">Adenocarcinoma</span> Cancer of epithelial cells with glandular origin or characteristics

Adenocarcinoma 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 both. Adenocarcinomas are part of the larger grouping of carcinomas, but are also sometimes called by more precise terms omitting the word, where these exist. Thus invasive ductal carcinoma, the most common form of breast cancer, is adenocarcinoma but does not use the term in its name—however, esophageal adenocarcinoma does to distinguish it from the other common type of esophageal cancer, esophageal squamous cell carcinoma. Several of the most common forms of cancer are adenocarcinomas, and the various sorts of adenocarcinoma vary greatly in all their aspects, so that few useful generalizations can be made about them.

<span class="mw-page-title-main">Esophageal cancer</span> Gastrointestinal system cancer that is located in the esophagus

Esophageal cancer is cancer arising from the esophagus—the food pipe that runs between the throat and the stomach. Symptoms often include difficulty in swallowing and weight loss. Other symptoms may include pain when swallowing, a hoarse voice, enlarged lymph nodes ("glands") around the collarbone, a dry cough, and possibly coughing up or vomiting blood.

<span class="mw-page-title-main">Stomach cancer</span> Cancerous tumor originating in the stomach lining

Stomach cancer, also known as gastric cancer, is a malignant tumor of the stomach. It's 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. Lymphomas and mesenchymal tumors may also develop in the stomach. Early symptoms may include heartburn, upper abdominal pain, nausea, and loss of appetite. Later signs and symptoms may include weight loss, yellowing of the skin and whites of the eyes, vomiting, difficulty swallowing, and blood in the stool, among others. The cancer may spread from the stomach to other parts of the body, particularly the liver, lungs, bones, lining of the abdomen, and lymph nodes.

<span class="mw-page-title-main">Carcinoma</span> Malignancy that develops from epithelial cells

Carcinoma is a malignancy that develops from epithelial cells. Specifically, a carcinoma is a cancer that begins in a tissue that lines the inner or outer surfaces of the body, and that arises from cells originating in the endodermal, mesodermal or ectodermal germ layer during embryogenesis.

<span class="mw-page-title-main">Pancreatic cancer</span> Type of endocrine gland cancer

Pancreatic cancer arises when cells in the pancreas, a glandular organ behind the stomach, begin to multiply out of control and form a mass. These cancerous cells have the ability to invade other parts of the body. A number of types of pancreatic cancer are known.

<span class="mw-page-title-main">Atrophic gastritis</span> Chronic inflammation and degradation of the stomach lining

Atrophic gastritis is a process of chronic inflammation of the gastric mucosa of the stomach, leading to a loss of gastric glandular cells and their eventual replacement by intestinal and fibrous tissues. As a result, the stomach's secretion of essential substances such as hydrochloric acid, pepsin, and intrinsic factor is impaired, leading to digestive problems. The most common are pernicious anemia possibly leading to vitamin B12 deficiency; and malabsorption of iron, leading to iron deficiency anaemia. It can be caused by persistent infection with Helicobacter pylori, or can be autoimmune in origin. Those with autoimmune atrophic gastritis (Type A gastritis) are statistically more likely to develop gastric carcinoma, Hashimoto's thyroiditis, and achlorhydria.

<span class="mw-page-title-main">Gastrointestinal cancer</span> Organ system cancer located in gastrointestinal tract

Gastrointestinal cancer refers to malignant conditions of the gastrointestinal tract and accessory organs of digestion, including the esophagus, stomach, biliary system, pancreas, small intestine, large intestine, rectum and anus. The symptoms relate to the organ affected and can include obstruction, abnormal bleeding or other associated problems. The diagnosis often requires endoscopy, followed by biopsy of suspicious tissue. The treatment depends on the location of the tumor, as well as the type of cancer cell and whether it has invaded other tissues or spread elsewhere. These factors also determine the prognosis.

Hepatoid tumor or hepatoid [adeno]carcinoma are terms for a number of uncommon or rare neoplasms in humans, named for a visual resemblance of the cells under the microscope to those of hepatocellular carcinoma, the most common form of liver cancer. They can arise in several parts of the body, and thus form sub-types of diseases such as stomach cancer and pancreatic cancer. The WHO defines "Hepatoid carcinoma" as "An adenocarcinoma with morphologic characteristics similar to hepatocellular carcinoma, arising from an anatomic site other than the liver".

<span class="mw-page-title-main">Small intestine cancer</span> Medical condition

Small intestine cancer is a cancer of the small intestine. It is relatively rare compared to other gastrointestinal malignancies such as gastric cancer and colorectal cancer.

<span class="mw-page-title-main">Fundic gland polyposis</span> Medical condition

Fundic gland polyposis is a medical syndrome where the fundus and the body of the stomach develop many fundic gland polyps. The condition has been described both in patients with familial adenomatous polyposis (FAP) and attenuated variants (AFAP), and in patients in whom it occurs sporadically.

<span class="mw-page-title-main">Non-small-cell lung cancer</span> Any type of epithelial lung cancer other than small-cell lung carcinoma

Non-small-cell lung cancer (NSCLC), or non-small-cell lung carcinoma, is any type of epithelial lung cancer other than small-cell lung cancer (SCLC). NSCLC accounts for about 85% of all lung cancers. As a class, NSCLCs are relatively insensitive to chemotherapy, compared to small-cell carcinoma. When possible, they are primarily treated by surgical resection with curative intent, although chemotherapy has been used increasingly both preoperatively and postoperatively.

<span class="mw-page-title-main">Adenocarcinoma in situ of the lung</span> Medical condition

Adenocarcinoma in situ (AIS) of the lung —previously included in the category of "bronchioloalveolar carcinoma" (BAC)—is a subtype of lung adenocarcinoma. It tends to arise in the distal bronchioles or alveoli and is defined by a non-invasive growth pattern. This small solitary tumor exhibits pure alveolar distribution and lacks any invasion of the surrounding normal lung. If completely removed by surgery, the prognosis is excellent with up to 100% 5-year survival.

<span class="mw-page-title-main">Signet ring cell carcinoma</span> Medical condition

Signet ring cell carcinoma (SRCC) is a rare form of highly malignant adenocarcinoma that produces mucin. It is an epithelial malignancy characterized by the histologic appearance of signet ring cells.

<span class="mw-page-title-main">CMTM2</span> Protein-coding gene in the species Homo sapiens

CKLF-like MARVEL transmembrane domain-containing protein 2, previously termed chemokine-like factor superfamily 2, is a member of the CKLF-like MARVEL transmembrane domain-containing family (CMTM) of proteins. In humans, it is encoded by the CMTM2 gene located in band 22 on the long arm of chromosome 16. CMTM2 protein is expressed in the bone marrow and various circulating blood cells. It is also highly expressed in testicular tissues: The CMTM2 gene and CMTM2 protein, it is suggested, may play an important role in testicular development.

<span class="mw-page-title-main">Hereditary cancer syndrome</span> Inherited genetic condition that predisposes a person to cancer

A hereditary cancer syndrome is a genetic disorder in which inherited genetic mutations in one or more genes predispose the affected individuals to the development of cancer and may also cause early onset of these cancers. Hereditary cancer syndromes often show not only a high lifetime risk of developing cancer, but also the development of multiple independent primary tumors.

<span class="mw-page-title-main">Hereditary diffuse gastric cancer</span> Medical condition

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 significantly elevated risk for developing stomach cancer. For this reason, individuals with these mutations will often elect to undergo prophylactic gastrectomy, or a complete removal of the stomach to prevent this cancer. Mutations in CDH1 are also associated with high risk of lobular breast cancers, and may be associated with a mildly elevated risk of colon cancer.

<span class="mw-page-title-main">Squamous-cell carcinoma</span> Carcinoma that derives from squamous epithelial cells

Squamous-cell carcinoma (SCC), also known as epidermoid carcinoma, comprises a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on the lining of the respiratory and digestive tracts.

Hereditary lobular breast cancer is a rare inherited cancer predisposition associated with pathogenic CDH1 (gene) germline mutations, and without apparent correlation with the hereditary diffuse gastric cancer syndrome. Research studies identified novel CDH1 germline variants in women with diagnosed lobular breast cancer and without any family history of gastric carcinoma. Firstly, in 2018 Giovanni Corso et al. defined this syndrome as a new cancer predisposition and the Authors suggested additional clinical criteria to testing CDH1 in lobular breast cancer patients. In 2020, the International Gastric Cancer Linkage Consortium recognized officially that the hereditary lobular breast cancer is a possible independent syndrome. To date, there are reported about 40 families clustering for lobular breast cancer and associated with CDH1 germline mutations but without association with diffuse gastric cancer. Other recent studies demonstrated a possible correlation between hereditary lobular breast cancer and gastric cancer risk.

References

  1. 1 2 Jafferbhoy S, Shiwani H, Rustum Q (2013). "Managing Gastric Linitis Plastica: Keep the scalpel sheathed". Sultan Qaboos Univ Med J. 13 (3): 451–3. doi:10.12816/0003269. PMC   3749031 . PMID   23984032.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. 1 2 Robbins Basic Pathology, 8th Edition
  3. "Linitis plastica of the stomach | Cancer Research UK".
  4. Agnes, Annamaria; Estrella, Jeannelyn S.; Badgwell, Brian (2017-07-05). "The significance of a nineteenth century definition in the era of genomics: linitis plastica". World Journal of Surgical Oncology. 15 (1): 123. doi: 10.1186/s12957-017-1187-3 . ISSN   1477-7819. PMC   5498981 . PMID   28679451.
  5. Bevan S, Houlston RS (1999). "Genetic predisposition to gastric cancer". QJM: Monthly Journal of the Association of Physicians. 92 (1): 5–10. doi: 10.1093/qjmed/92.1.5 . PMID   10209666.