Linitis plastica overview
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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
The term "linitis plastica" was first used by Dr. William Brinton, an English physician, in 1854.[1][2] Development of linitis plastica is the result of genetic mutation in the CDH1 (E-cadherin) gene, that is responsible for intercellular adhesions.[3] On gross pathology, a thick, rigid, and leather bottle-like stomach wall is a characteristic finding of linitis plastica.[4] On microscopic histopathological analysis, atypical signet ring cells that diffusely infiltrate the stomach wall, submucosal fibrosis and thickening, and a minimal mucosal involvement are characteristic findings of linitis plastica. Linitis plastica usually affects individuals of the Asian race.[4] The presence of metastasis is associated with a particularly poor prognosis among patients with linitis plastica. Linitis plastica is associated with a 5-year survival rate of approximately 10-20% in Japan.[3] Total gastrectomy is recommended for the management of local disease and as a prophylactic surgery in patients with hereditary form of linitis plastica.[3]
Historical Perspective
The term "Linitis plastica" was first used by Dr. William Brinton, an English physician, in 1854.[1]
Pathophysiology
On gross pathology, a thick, rigid, and leather bottle-like stomach wall is a characteristic finding of linitis plastica.[4] On microscopic histopathological analysis, atypical signet ring cells that diffusely infiltrate the stomach wall, submucosal fibrosis and thickening, and a 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.[3]
Causes
The most common cause of linitis plastica is scirrhous adenocarcinoma. Linitis plastica may be caused by germline mutations in the CDH1 (E-cadherin) gene.[5][1]
Differntiating Linitis Plastica from Other Diseases
Linitis plastica must be differentiated from other diseases that cause thickening of the gastric wall such as lymphoma, other forms of gastric adenocarcinoma, menetrier's disease, lymphoid hyperplasia, amyloidosis, and granulomatous diseases.[3][1]
Epidemiology and Demographics
Hereditary form of diffuse gastric adenocarcinoma (HDGC) is usually first diagnosed before 40 years of age, sometimes even before the age of 25 years.[3] Females are slightly more affected with linitis plastica than males.[3]Linitis plastica usually affects individuals of the Asian race, particularly the Japanese.[4]
Risk Factors
Common risk factors in the development of linitis plastica may include Asian race, female gender, and the presence of CDH1 gene mutations.[6][3]
Screening
There is insufficient evidence to recommend routine screening for linitis plastica.
Natural History, Complications and Prognosis
If left untreated, patients with linitis plastica may progress to develop symptoms from decreased peristalsis of the stomach that may include dyspepsia, dysphagia, nausea, vomiting, increased satiety, and regurgitation.[7][3] Depending upon the stage of linitis plastica at the time of diagnosis, the prognosis may vary. The presence of metastasis is associated with a particularly poor prognosis among patients with linitis plastica. Linitis plastica is associated with a 5-year survival rate of approximately 10-20% in Japan.[3]
Diagnosis
History and Symptoms
Symptoms of linitis plastica may include dyspepsia, nausea, vomiting, early satiety, loss of appetite, weight loss, abdominal pain/discomfort, and sometimes diarrhea.[3][4][7] A positive family history of diffuse gastric cancer may be present if it is the hereditary form of the disease.
Physical Examination
Common physical examination findings of linitis plastica may include lymphadenopathy, organomegaly, and signs of ascites.[8][9]
Laboratory Findings
Laboratory findings consistent with the diagnosis of linitis plastica may include abnormal complete blood picture, electrolyte abnormalities, and occult blood in stool.[9]
Electrocardiogram
There are no electrocardiogram findings associated with linitis plastica.
CT
On CT abdomen, linitis plastica is characterized by diffusely thickened stomach with a small lumen. CT of chest, abdomen and/or pelvis may be performed to detect metastases to lymph nodes or any other organs.[1]
MRI
MRI of chest, abdomen and/or pelvis may be performed to detect local invasion and metastasis from or of linitis plastica to breast, lungs and liver.[10]
Other Imaging Findings
Upper GI series may be helpful in the diagnosis of linitis plastica. Findings on upper GI series suggestive of linitis plastica include inadequately distended stomach with increased rigidity and a narrow stomach lumen, loss of normal mucosal fold pattern, and distorted, thickened or nodular mucosa.[1]
Other Diagnostic Studies
On upper GI endoscopy, linitis plastica may show superficial ulcers and/or inflammation. On endoscopic ultrasound, abnormal thickening of the stomach wall is a characteristic finding.[3]
Treatment
Medical Therapy
The predominant therapy for linitis plastica is surgical resection. Adjunctive chemotherapy and radiation may be required.[3]
Surgery
Total gastrectomy is recommended for the management of local disease. Surgical resection is not recommended among patients with metastatic linitis plastica.[3]
Primary Prevention
Effective measure for the primary prevention of hereditary diffuse gastric adenocarcinoma includes prophylactic gastrectomy in individuals who test positive for CDH1 gene mutations.[11]
Secondary Prevention
There are no secondary preventive measures available for linitis plastica.
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Linitis plastica. Radiopedia.org. http://radiopaedia.org/articles/linitis-plastica Accessed on November 17, 2015
- ↑ 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.
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 Gastric linitis plastica. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=36273 Accessed on December 8, 2015.
- ↑ 4.0 4.1 4.2 4.3 4.4 Linitis plastica. Wikipedia. https://en.wikipedia.org/wiki/Linitis_plastica Accessed on November 18, 2015.
- ↑ Robbins Basic Pathology, 8th Edition
- ↑ Wolf, E.-M.; Geigl, J.B.; Svrcek, M.; Vieth, M.; Langner, C. (2010). "Hereditäres Magenkarzinom". Der Pathologe. 31 (6): 423–429. doi:10.1007/s00292-010-1353-7. ISSN 0172-8113.
- ↑ 7.0 7.1 Jafferbhoy S, Shiwani H, Rustum Q (2013). "Managing Gastric Linitis Plastica: Keep the scalpel sheathed". Sultan Qaboos Univ Med J. 13 (3): 451–3. PMC 3749031. PMID 23984032.
- ↑ Ascites. Wikipedia. https://en.wikipedia.org/wiki/Ascites Accessed on December 10, 2015
- ↑ 9.0 9.1 Stomach cancer. Canadian cancer society. http://www.cancer.ca/en/cancer-information/cancer-type/stomach/diagnosis/?region=nb Accessed on December 10, 2015
- ↑ Stomach cancer. Canadian cancer society. http://www.cancer.ca/en/cancer-information/cancer-type/stomach/diagnosis/?region=nb Accessed on December 9, 2015
- ↑ Pandalai PK, Lauwers GY, Chung DC, Patel D, Yoon SS (2011). "Prophylactic total gastrectomy for individuals with germline CDH1 mutation". Surgery. 149 (3): 347–55. doi:10.1016/j.surg.2010.07.005. PMID 20719348.