Peutz-Jeghers syndrome
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Peutz-Jeghers syndrome
Pathology
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Gastrointestinal system pathology review
Appendicitis: Pathology review
Cirrhosis: Pathology review
Colorectal polyps and cancer: Pathology review
Congenital gastrointestinal disorders: Pathology review
Diverticular disease: Pathology review
Esophageal disorders: Pathology review
Gallbladder disorders: Pathology review
Gastrointestinal bleeding: Pathology review
GERD, peptic ulcers, gastritis, and stomach cancer: Pathology review
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Peutz-Jeghers syndrome
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Hyperpigmentation
Peutz-Jeghers syndrome as cause p. 396
Peutz-Jeghers syndrome p. 225, 396
Transcript
Content Reviewers
Peutz-Jeghers syndrome, named after Dr. Jan Peutz, who first described it, and Dr. Harold Joseph Jeghers, who later reported on it, is a rare autosomal dominant condition in which individuals develop polyps throughout their gastrointestinal tract, as well as dark spots called melanotic macules in their mouth, lips, genitalia, palms, and soles.
The large intestine is found in the abdominal cavity, which can be thought of as having two spaces - the intraperitoneal space and the retroperitoneal space.
The intraperitoneal space contains the first part of the duodenum, all of the small intestines, the transverse colon, sigmoid colon, and the rectum; the retroperitoneal space contains the distal duodenum, ascending colon, descending colon, and anal canal.
So the large intestines essentially weave back and forth between the intraperitoneal and retroperitoneal spaces.
Now, the walls of the gastrointestinal tract are composed of four layers.
The outermost layer is the called serosa for the intraperitoneal parts, and the adventitia for the retroperitoneal parts.
Next is the muscular layer, which contracts to move food through the bowel.
After that is the submucosa, which consists of a dense layer of tissue that contains blood vessels, lymphatics, and nerves.
Summary
Peutz-Jeghers syndrome (PJS), also known as hereditary intestinal polyposis syndrome, is an autosomal dominant disease characterized by polyps throughout the gastrointestinal tract, along with melanotic macules in the skin and mucosa, and a high risk of developing malignancy in various organs, including cancers of the GI tract, pancreas, breasts, lungs, ovaries, uterus, and testicles.
People with PJS have a higher risk of developing certain types of cancer, particularly in the colon, stomach, small intestine, pancreas, and breast. Regular surveillance and screening for these cancers are recommended for people with PJS. Treatment of PJS usually involves surgically removing the polyps, and regular surveillance with colonoscopies, upper endoscopies, and other imaging studies.
Sources
- "Peutz-Jeghers syndrome: a systematic review and recommendations for management" Gut (2010)
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "Regulation of LKB1 expression by sex hormones in adipocytes" International Journal of Obesity (2011)
- "Colorectal cancer screening and surveillance: Clinical guidelines and rationale?Update based on new evidence" Gastroenterology (2003)
- "Hamartomatous polyposis syndromes: A review" Orphanet Journal of Rare Diseases (2014)
- "Surface area of the digestive tract – revisited" Scandinavian Journal of Gastroenterology (2014)
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