Term
| what are the different kinds of non neoplastic polyps? |
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Definition
| inflammatory polyps, hamartomatous polyps, and hyperplastic polyps |
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Term
| what is solitary rectal ulcer syndrome? |
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Definition
| a kind of *inflammatory polyp caused by impaired relaxation of the anorectal sphincter, leading to a *sharp angle at the rectal shelf which then causes an inflammatory lesion on the anterior rectal wall with recurrent abrasion/ulceration of the mucosa/mucus discharge. *these polyps are usually due to injury/repair cycles.* |
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Term
| how might an inflammatory polyp lead to mucosal prolapse? |
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Definition
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Term
| what can an inflammatory polyp cause chronically? |
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Definition
| fibromuscular hyperplasia, mixed inflammatory infiltrates, erosion, and epithelial hyperplasia |
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Term
| what are hamartomatous polyps? |
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Definition
| tumor-like (not true neoplasms) growths composed of mature tissues that are normally present in the organ that occur sporadically and in some genetic and acquired syndromes. |
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Term
| what are some syndromes associated with hamartomatous polyps? |
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Definition
| peutz-jeghers syndrome, cowden syndrome, bannayan-ruvalcaba-riley syndrome, and cronkhite-canada syndrome |
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Term
| what is a juvenile polyp? |
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Definition
| one example of a *hamartomatous polyp that occurs in children < 5 yrs old. they consist of *focal malformations of the mucosal epithelium and lamina propria and can be sporadic (usually solitary) or syndromic. these may be passed with a bowel movement. |
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Term
| where do most juvenile polyps occur? how do they present? |
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Definition
| in the rectum, and present as rectal bleeding. they may protrude through the anal sphincter. |
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Term
| what is an example of a syndrome associated with juvenile polyps? what locations of the body can be affected? are there extraintestinal manifestations? |
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Definition
| juvenile polyposis - an autosomal dominant disease where pts present w/3-100 polyps that may require colectomy due to the potential for severe hemorrhage. polyps may develop in the *stomach and small bowel*. extraintestinal manifestations include *pulmonary arteriovenous malformations and *marked protein loss |
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Term
| how do juvenile polyps appear morphologically? histologically? |
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Definition
| morphologically, juvenile polyps are usually less than 3 cm in diameter, have a pedunculated, smooth surface, appear reddish w/cystic spaces. histologically, there are *cysts (dilated glands filled with mucin/inflammatory debris), the muscularis mucosa may be normal though the *lamina propria has a mixed inflammatory infiltrate |
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Term
| are there any genetic associations with juvenile polyposis? |
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Definition
| the mutated genes SMAD4 and BMPPR1A which regulate cell growth |
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Term
| is there an increased risk for colonic adenocarcinoma for pts with juvenile polyposis? |
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Definition
| yes (even though these are hamartomatous polyps) |
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Term
| what is peutz-jeghers syndrome? |
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Definition
| a rare/autosomal dominant condition involving multiple hamartomatous polyps and *mucocutaneous hyperpigmentation (like around the mouth)*. the median age of presentation is 11 yrs |
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Term
| what characterizes peutz-jeghers syndrome? |
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Definition
| macules form around the mouth, buccal mucosa, palms, genitalia, and perianal areas. this can initiate intussusception (obstructive telescoping of the bowel) |
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Term
| is there an increased risk of malignancy with peutz-jeghers syndrome? |
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Definition
| yes, there is an association of increased risk of *malignancies in the breast/colon/lung/ovaries/uterus/testicles*. there is an association with *SCAT (sex cord tumor with annular tubules - know for boards). adenocarcinomas arise independently from the hamartomatous polyps (spatially speaking). |
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Term
| is there a genetic association with peutz-jeghers syndrome? |
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Definition
| mutations in the gene *LKB1/STK11, which encodes a kinase that regulates cell polarization, growth, and metabolism have been associated with peutz jeghers syndrome |
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Term
| where are peutz jeghers polyps most common? |
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Definition
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Term
| how do peutz-jeghers polyps appear morphologically? |
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Definition
| morphologically: large, pedunculated and lobular. histologically: there is an *arborizing network of connective tissue, smooth muscle, lamina propria and glands *lined by normal intestinal epithelium |
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Term
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Definition
| an autosomal dominant hamartomatous polyp syndrome which leads to macroencephaly, intestinal harmatomatous polyps, benign skin lesions, trichilemmomas, papillomatous papules, and acral keratoses |
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Term
| what is the genetic cause of cowden syndrome? |
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Definition
| there is a loss of function mutation in the *PTEN gene, which encodes a lipid phosphatase that normally inhibits signaling through the P13K/AKT pathway (therefore predisposed to neoplastic growth) |
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Term
| does having cowden syndrome increase the risk of malignancies? |
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Definition
| yes, w/cowdens there is increased risks of GI malignancy, breast CA, follicular CA, thyroid and endometrial CA |
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Term
| what is mcdonald's mnemonic for remembering cowdens? |
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Definition
| “cow has big head: macroencephaly” / “cow has many stomachs “GI symptoms” / “cow has utters = “breast tissue” / “cow has hair = trichelomommas” / cow has large neck = thyroidomas”/ “cows are female “endometrial” |
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Term
| what is bannayan-ruvalcaba-riley syndrome? |
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Definition
| similar to cowdens with increased mental/developmental delays and a lower incidence of neoplasm. |
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Term
| what is cronkite-canada syndrome? |
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Definition
| a *nonherediatary hamartomatous polyposis syndrome which usually presents in pts over 50 years |
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Term
| how do pts with cronkite-canada syndrome present? extraintestinally? |
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Definition
| in cronkite-canada syndrome, hamartomatous polyps that resemble juvenile polyps histologically appear in the stomach, small intestine, colon, and rectum. pts present with non specific findings: diarrhea, weight loss, abdominal pain, and weakness - *extraintestinally: nail splitting, hair loss, cutaneous hyperpigmentation, and hypopigmentation |
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Term
| what are hyperplastic polyps? |
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Definition
| a common epithelial proliferation seen usually in the L colon of pts 50-60 yrs old and are ~5 mm in diameter. they are smooth nodular projections of mucosa, usually multiple, and composed of *goblet cells *w/serrated surface architecture. they can become malignant, but unsignificantly and may be *associated with a reaction adjacent to a more ominous lesion or inflammatory disorder |
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