Large intestine - polyps

Question 1

Characteristics of advanced adenomas?

Answer 1

• over 1 cm
  – Villous or tubulovillous histology
  – high-grade dysplasia

Question 2

Microvesicular and “goblet cells rich” Are subtypes of?

Answer 2

Hyperplastic polyps

Question 3

Main precursor of hyper methylated colorectal cancer?

Pathologic diagnosis requires?

Answer 3

Sessile serrated lesions

At least one feature of crypt distortion

Question 4

Histology of this polyp shows filiform projections lined with tall eosinophilic cells? Characteristic growth pattern?

Answer 4

Traditional serrated adenoma

Ectopic crypts perpendicular to the long axis of the villi

Question 5

Hamartomatous malformation of the mucosa consisting of lamina propria and circling dilated cystic ducts?

Answer 5

Juvenile polyps

Question 6

Hamartomatous polyps in Peutz-Jeghers vs juvenile polyps?

Answer 6

Hamartomatous polyps in Peutz-Jeghers have Smooth muscle hypertrophy

Question 7

Morphologic classification system of superficial colonic polyps?

Answer 7

Paris classification

Question 8

Inherited hamartomatous polyposis syndromes?

Answer 8

Peutz-Jeghers, juvenile polyposis, Cowdens

Question 9

Non-inherited polyposis syndromes?

Answer 9

Cronkhite-Canada syndrome, Serrated polyposis Syndrome

Question 10

Adenomatous polyposis syndrome associated with mandibular osteomas and dental anomalies?

Mutation of?

Answer 10

Familial adenomatous polyposis

APC gene on chromosome five

Question 11

Adenomatous polyposis syndrome associated with Congenital hypertrophy of the retinal pigment epithelium and desmoid tumor?

Mutation of?

Answer 11

Gardner syndrome

APC Gene

Question 12

Adenomatous polyposis syndrome associated with Medulloblastoma and glioblastoma?

Mutation of?

Answer 12

Turcot syndrome

APC gene

Question 13

Adenomatous polyposis syndrome associated with endometrial cancer?

Mutation of?

Answer 13

Polymerase proofreading-associated polyposis?

POLE and POLD1

Question 14

Only hereditary adenomatous polyposis syndrome that is not autosomal dominant?

Mutation of?

Associated with?

Answer 14

MutYH Associated polyposis

Biallelic mutation of the base excision repair gene MutYH

Ovarian, Bladder, skin cancer

Question 15

Patient with APC mutation - start screening At this age?

Most common cause of death after prophylactic colectomy? Start screening for this at age?

Answer 15

10-12

Duodenal adenocarcinoma; 25

Question 16

Drug combination that lowers duodenal polyp burden in patients with FAP?

Answer 16

Sulindac (NSAID) and erlotinib (kinase inhibitor)

Question 17

Scoring system for duodenal polyposis?

Answer 17

Spigelman scoring system

Question 18

Soft tissue fibrous tumors that are associated with Gardner syndrome? Treatment?

Answer 18

Desmoid tumor

NSAIDs, tamoxifen, imatinib

Question 19

Location if the Mutation that leads to attenuated FAP? Comparative risk of colon cancer?

Answer 19

3’ or 5’ end of the gene

70% (as opposed to 100%)

Question 20

Polyps of this syndrome show (on histology) proliferations of the smooth muscle in the shape of tree branches (arborization)? Gene?

Start EGD when?

Associated with these other cancers?

Answer 20

Peutz-Jeghers; STK11

Age 8

Ovarian, testicular (Sertoli) cancer, breast cancer

Question 21

Juvenile polyposis syndrome is associated with mutations in these genes?

Answer 21

SMAD4
BMPR1
ENG

Question 22

Cowden syndrome - associated gene?

Answer 22

PTEN
SDH
Killin gene

Question 23

The syndrome is characterized by hamartomatous polyps, pigmented macule of the penis, and developmental delay?

Answer 23

Bannayan-Riley-Ruvalcaba syndrome