Neoplastic intestinal disorders

Question 1

Intraluminal projections of mucosa that are formed of a non-neoplastic mixture of inflammatory cells, stromal and epithelial components

Answer 1

Inflammatory polyp

Question 2

Microscopy of a polyp shows inflamed granulation tissue with mixed inflammatory infiltrates, erosion, and epithelial hyperplasia.

Answer 2

Inflammatory polyp

Question 3

Polyp formed by piling up of goblet and absorptive cells from decreased epithelial turnover and delayed shedding of surface epithelial cells

Answer 3

Hyperplastic polyp

Question 4

Polyp formed by piling up of goblet and absorptive cells from being adjacent to or overlying any mass or inflammatory lesion

Answer 4

Hyperplastic polyp

Question 5

Chief significance of hyperplastic polyps

Answer 5

Must be distinguished from sessile serrated adenomas

Question 6

Smooth, nodular protrusion of the L colonic mucosa, often on the crests of mucosal folds. Microscopy shows mature goblet and absorptive cells with serrated surface.

Answer 6

Hyperplastic polyp

Question 7

Premalignant polyp caused by germline mutations in tumor suppressor genes or proto-oncogenes. Associated with extraintestinal manifestations

Answer 7

Hamartomatous polyp

Question 8

Polyp type of juvenile polyposis

Answer 8

Hamatomatous polyp

Question 9

Inheritance of juvenile polyposis

Answer 9

Autosomal dominant

Question 10

Treatment of juvenile polyposis

Answer 10

Colectomy –> limit hemorrhage

Question 11

Extraintestinal manifestations related to hamartomatous polyps

Answer 11

Pulmonary AVMs
Other congenital manifestations

Question 12

Mutations seen <50% of pts with hamartomatous polyps

Answer 12

SMAD4
BMPR1A

Question 13

Gene mutation associated with both juvenile polyposis and hereditary hemorrhagic telangiectasia

Answer 13

SMAD4

Question 14

<3 cm reddish polyp, typically pedunculated with a smooth surface. Cystic spaces are characteristic.

Answer 14

Hamartomatous polyp

Question 15

Microscopy of colon polyp shows normal or attenuated muscularis mucosae, lamina propria expanded by mixed inflammatory infiltrates, and cystically dilated crypts filled with mucin and inflammatory debris.

Answer 15

Hamartomatous polyp

Question 16

Precursors to colorectal adenocarcinomas

Answer 16

Colonic adenomas

Question 17

Small, pedunculated polyps composed of rounded glands. Mucosal surface is smooth, appearing white or red.

Answer 17

Tubular adenoma

Question 18

White granular mucosa distinct form the normal mucosa on a small sessile polyp with mixed architecture.

Answer 18

Serpentine appearance of tubulovillous adenoma

Question 19

Larger and sessile colonic polyp covered by slender villi. Diffuse nodular appearance.

Answer 19

Villous adenoma

Question 20

Conditions associated with villous adenomatous polyp

Answer 20

Hypoproteinemia
Secretory diarrhea
Hypokalemia

Question 21

Most important characteristic of adenomatous polyps that correlates with malignancy

Answer 21

Size

Question 22

Microscopy of polyp found in the R colon shows serrated architecture through the full length of the glands, including the crypt base, crypt dilation, and lateral crypt growth

Answer 22

Sessile serrated adenoma

Question 23

Inheritance of Peutz-Jeghers syndrome

Answer 23

Autosomal dominant

Question 24

Hereditary syndrome characterized by multiple GI hamartomatous polyps and mucocutaneous hyperpigmentation. Associated with a markedly increased risk of several malignancies throughout childhood and adulthood. Commonly presents between 10-15 yo.

Answer 24

Peutz-Jeghers syndrome

Question 25

Tumor that can be present at birth in Peutz-Jeghers syndromes

Answer 25

Sex cord tumors of the testes

Question 26

Mutation found in most cases of Peutz-Jeghers syndrome

Answer 26

Loss of function mutation in STK11

Question 27

Most common location of polyps in Peutz-Jeghers syndromes

Answer 27

Small intestine

Question 28

Syndrome associated with arborizing network microscopy of polyp

Answer 28

Peutz-Jeghers

Question 29

Mutation in Cowden syndrome

Answer 29

PTEN --> PI3K/AKT pathway

Question 30

Hereditary syndrome associated with hamartomatous and inflammatory intestinal polyps, lipomas, and ganglioneuromas. Mean age of presentation <15 yo

Answer 30

Cowden syndrome

Question 31

Standard of care for familial adenomatous polyposis

Answer 31

Prophylactic colectomy

Question 32

Common extracolonic sites for neoplasia in familial adenomatous polyposis

Answer 32

Ampulla of Vater Stomach

Question 33

Congenital hypertrophy of the retinal pigment epithelium is associated with what hereditary syndrome

Answer 33

Familial adenomatous polyposis

Question 34

Gene mutations associated with familial adenomatous polyposis

Answer 34

APC MUTYH

Question 35

Mean age of presentation in classic FAP

Answer 35

10-15 yo

Question 36

Mean age of presentation in attenuated FAP

Answer 36

40-50 yo

Question 37

Mean age of presentation in Gardner syndrome and Turcot syndrome

Answer 37

10-15 yo

Question 38

Mean age of presentation in MUTYH associated polyposis

Answer 38

30-50 yo

Question 39

Extra-GI manifestations in Gardner syndrome

Answer 39

Osteomas Thyroid and desmoid tumors Skin cysts

Question 40

Extra-GI manifestations in Turcot syndrome

Answer 40

Medulloblastoma Glioblastoma

Question 41

Gene associated with autosomal dominant FAP

Answer 41

APC

Question 42

Most common mutations in hereditary non-polyposis colorectal carcinoma

Answer 42

MSH2 MLH1

Question 43

Inheritance of hereditary non-polyposis colorectal carcinoma

Answer 43

Autosomal dominant

Question 44

Hereditary cancer syndrome associated with consequences in mismatch repair deficiency and resulting microsatellite insufficiency.

Answer 44

Lynch syndrome

Question 45

Pharmacologic chemoprevention of colorectal adenocarcinoma

Answer 45

COX-2 inhibitors

Question 46

Most common maligancy of GI tract

Answer 46

Colorectal adenocarcinoma

Question 47

Stepwise accumulation of multiple mutations in colorectal adenocarcinoma

Answer 47

APC --> beta-catenin --> KRAS --> TP53, LOH, and SMAD

Question 48

Mutation in TGFBR2 results in what?

Answer 48

Uncontrolled cell growth

Question 49

How to identify microsatellite instability?

Answer 49

Absence of immunohistochemical staining for mismatch repair proteins Molecular genetic analysis of microsatellite sequences

Question 50

Colorectal adenocarcinoma that produces abundant mucin is associated with what prognosis?

Answer 50

Poor

Question 51

Dysplastic epithelial cells breach the basement membrane to invade the lamina propria. May extend into, but not through the muscularis mucosa. Complete polypectomy is generally curative and there is little metastatic potential.

Answer 51

Intramucosal carcinoma