03-06 Tumors of the Bowel + PATH Flashcards

Question 1

Q

OBJECTIVE: define hamartoma

Answer

A

<ul>
<li>
  mass  of  mature  tissues  normally  found  at  that  site,  but  present  in  an  abnormal   arrangement. </li>
</ul>

Question 2

Q

Name the benign neoplasm and malignant neoplasm version of normal: glandular epithelium

Answer

A

<ul>
<li>Adenoma</li>
<li>Adenocarcinoma</li>
</ul>

Question 3

Q

Name the benign neoplasm and malignant neoplasm version of normal: Adipose tissue

Answer

A

lipoma = benign

liposarcoma = malignant

Question 4

Q

Name the benign neoplasm and malignant neoplasm version of normal: vessels

Answer

A

angioma, angiosarcoma

Question 5

Q

Name the benign neoplasm and malignant neoplasm version of normal: neuroendocrine cells

Answer

A

<ul>
<li>
benign = carcinoid*</li>
<li>
malignant = Neuroendocrine carcinoma</li>
</ul>

1  There  is  no  reliable  histopathologic  feature  to  distinguish  which  of  these  tumors  will  behave  in  a  benign   vs. malignant fashion. Best "predictors" are size and mural invasion.

Question 6

Q

Name the benign and malignant: Smooth muscle neoplasms

Answer

A

leiomyoma, leiomyosarcoma

Question 7

Q

Name the benign and malig Peripheral  nerve  sheath neoplasms

Answer

A

<ul>
<li>
benign = Schwannoma</li>
<li>
Malignant  Peripheral  Nerve  Sheath  Tumor  (MPNST) </li>
</ul>

Question 8

Q

Interstital cells of Cajal neoplasms

Answer

A

<ul>
<li>
benign = GIST*</li>
<li>
malig = GIST*</li>
</ul>

Gastrointestinal  Stromal  Tumor,  a  unique  mesenchymal  tumor  (formerly  lumped  in  with   leiomyoma/leiomyosarcomas  or  schwannoma/MPNSTs)  that  demonstrate  expression  and  often  mutation  of  the   c-kit  receptor  tyrosine  kinase  proto-oncogene  (Stem  cell  factor-receptor;  CD117).

Question 9

Q

Only \_\_ % of GI tumors are in the small intestine.  In fact, most are actually \_\_\_\_\_\_.

Answer

A

Only 1 % of GI tumors are in the small intestine.  In fact, most are actually mets from other cancers.

Question 10

Q

Top 3 most common types of malignant small bowel cancer

Answer

A

<ol>
<li>
adenocarcinoma</li>
</ol>

followed by carcinoid and lymphoma

Question 11

Q

Estimated that the time interval required for progression from adenoma to carcinoma in individuals with sporadic colon cancers ranges from \_\_\_\_ yrs.

Answer

A

Estimated that the time interval required for progression from adenoma to carcinoma in individuals with sporadic colon cancers ranges from 5-12 yrs.

Question 12

Q

CRC is the \_\_\_\_ most common cancer

Answer

A

CRC is the 3rd most common cancer

Question 13

Q

adenoma of the small intestine

<ul>
<li>
Benign or malignant?</li>
<li>a
Found in \_\_\_\_\_\_\_\_</li>
<li>
Clinical Presentation</li>
<li>
Path Features</li>
<li>
Prognosis</li>
<li>
Treatment options</li>
</ul>

Answer

A

<ul>
<li>
benign (makes up 25% of all benign small bowel tumors), but neoplastic</li>
<li>
found in small intesting (most often by the ampulla of vader)</li>
<li>
Presentation:
<ul>
<li>
♀ = ♂; Age = 50-85</li>
<li>
Sx: often vague & nonspecific, bleeding: active or occult,  pain (colicky), n/v, weight loss, (rarely perf)</li>
<li>
Incidence  6200 cases/yr</li>
<li>
25-30%  of symptomatic patients have palpable mass.</li>
</ul>
</li>
<li>
Path features
<ul>
<li>
low-grade dysplasia</li>
</ul>
</li>
<li>
Prognosis: may progress to adenocarcinoma</li>
</ul>

Question 14

Q

What are other some benign small bowel tumors besides adenoma?

Answer

A

leiomyoma, lipoma, hamartomatous polyps, hemangioma, neurofibroma and ~carcinoid

Question 15

Q

SB adenocarcinoma

<ul>
<li>Found in \_\_\_\_\_\_\_\_</li>
<li>Clinical Presentation</li>
<li>Risk Factors</li>
<li>Path Features</li>
<li>Prognosis</li>
<li>Treatment options</li>
</ul>

Answer

A

malignant SB neoplasms

<ul>
<li>Found in: Most  occur  in  the  duodenum,  near  ampulla  of  Vater. </li>
<li>Clinical Presentation:
<ul>
<li>pain</li>
<li>bleeding/anemia</li>
<li>biliary obstruction (b/c so often near ampulla of Vater)</li>
</ul>
</li>
<li>Risk Factors:
<ul>
<li>h/o IBD --> adenocarcinoma</li>
<li>Celiac dz --> lymphoma</li>
<li>Herid cancer syndromes like Fam. polyposis, HNPCC, Peutz-Jeghers</li>
<li>enviro exposures</li>
</ul>
</li>
<li>Path Features</li>
<li>Prognosis: generally poor
<ul>
<li>50-60%  have advanced ds at time of dx</li>
<li>20-50%  5 yr survival for non-lymphoma </li>
</ul>
</li>
<li>Treatment options
<ul>
<li>Segmental resection :
<ul>
<li>small bowel carcinoids</li>
<li>confirmed adenomas/adenocarcinomas</li>
<li>symptomatic lesions & those of uncertain</li>
<li>histology</li>
</ul>
</li>
<li>Whipple resection for duodenal adenocarcinomas</li>
<li>Chemo for SB lymphomas</li>
<li>Endoscopic resection for benign lesions of duodenum or TI</li>
<li>Endoscopic surveillance for benign villous adenomas of duodenum in pts w/ FAP</li>
</ul>
</li>
</ul>

Question 16

Q

SB carcinoid tumor

<ul>
<li>Found in \_\_\_\_\_\_\_\_</li>
<li>Clinical Presentation</li>
<li>Path Features</li>
<li>Prognosis</li>
</ul>

Answer

A

SB carcinoid tumor

<ul>
<li>Found in: appendix #1 place; followed by followed by the small intestine (primarily ileum), rectum, colon, and stomach.
<ul>
<li>also:bronchial tree, GU & GYN tracts</li>
</ul>
</li>
<li>Clinical Presentation: MOST COMMON SB MALIG
<ul>
<li>pain/obstruction most common</li>
<li>rare: carcinoid syndrome
<ul>
<li>Occurs in <10% w/ malig. carcinoids, usu with extensive liver metastases</li>
<li>Release of serotonin into systemic circ. leads to</li>
<li>skin flushing/cyanosis</li>
<li>diarrhea & cramps</li>
<li>bronchospasm</li>
<li>R ventricular subendocardial fibrosis -></li>
<li>Pulmonic & tricuspid valve stenosis/regurge</li>
</ul>
</li>
</ul>
</li>
<li>Path Features
<ul>
<li>yellow submucosa (pic on reverse)</li>
<li>tumor invades muscularis propria, a characteristic muscle fiber stranding</li>
<li>fibrosis often severe enough to kink →obstruction. </li>
<li>organoid (seen here) or gyrating histo</li>
</ul>
</li>
<li>Prognosis:Site, depth of invasion, and size are prognostic indicators:
<ul>
<li>Having symptoms is bad: 90% of symptomatic pts have mets
<ul>
<li>often found incidentally</li>
</ul>
</li>
<li>appendicial and rectal: likely benign</li>
<li>< 2cm: likely benign</li>
<li>hasn't invaded muscularis mucosa: likely benign</li>
</ul>
</li>
</ul>

Question 17

Q

Dx of carcinoid cancers

Answer

A

<ul>
<li>
can use octreotide test: nearly all carcinoid tumors have somatostatin receptors</li>
<li>
CT, biopsy, blah blah</li>
</ul>

Question 18

Q

SB lymphoma

<ul>
<li>Found in \_\_\_\_\_\_\_\_</li>
<li>Clinical Presentation</li>
<li>Risk Factors</li>
<li>Path Features</li>
<li>Prognosis</li>
<li>Treatment options</li>
</ul>

Answer

A

SB lymphoma

<ul>
<li>Found in: GIT = commonest  site  for  1°  extranodal  lymphomas.
<ul>
<li>most often  stomach followed  by  SB, ileocecal  region and colon </li>
</ul>
</li>
<li>Clinical Presentation</li>
<li>Risk Factors: 1°  GI  lymphomas  are  commonly  associated with:</li>
<li>
<ul>
<li>chronic  infection  (H. pylori  gastritis)</li>
<li>celiac  disease</li>
<li>immunodeficiency  (AIDS,  immunosuppression),  and</li>
<li>Crohn's  disease</li>
<li>Can  be  sporadic  also.   </li>
</ul>
</li>
<li>Path Features
<ul>
<li>2 types in both stomach and intestines: DLBCL and Marginal zone types</li>
</ul>
</li>
<li>Prognosis:
<ul>
<li>Lymphomas  have  a  much  better  prognosis  than  carcinomas.</li>
<li>5 yr survival  rate depends  on  the  stage  and  histologic  type,  but,  overall,  it  is  >50%.</li>
</ul>
</li>
</ul>

Question 19

Q

SB GIST tumor basics

Answer

A

<ul>
<li>
Mesenchymal tumor resembling modified smooth muscle and neural tissue
<ul>
<li>
Hypothesized origin = Interstitial cells of Cajal</li>
</ul>
</li>
<li>
Resides in muscularis mucosa (so deep-seated tumor)</li>
<li>
CD117 positive (c-kit tyrosine kinase)</li>
<li>
Spectrum from benign to malignant</li>
</ul>

Question 20

Q

Cancers that met to SB

Answer

A

Melanoma,  breast  carcinoma,  lung  carcinoma,  renal  cell  carcinoma.

Question 21

Q

#1 gene mutation in colon ca

Answer

A

APC  gene  mutations  are  usually  the  earliest  and,  possibly,  the  initiating  event  in  about  80%  of  sporadic  colon  cancers.  With  clonal  progression,  additional  mutations  accumulate,  such  as  in  K-ras  and  p53  genes.

Question 22

Q

When do most CRCs occur?

Answer

A

90% occur in pts > 50 y/o

Question 23

Q

If you see CRC in younger pts you should think

Answer

A

think  FAP,  HNPCC, MUTYH, PJS, or IBD

Question 24

Q

FAP

<ul>
<li>
caused by?</li>
<li>
present at what age? how?</li>
<li>
natural hx?</li>
<li>
tx?</li>
</ul>

Answer

A

Familial Adenomatous Polyposis

<ul>
<li>
-Autosomal dominant disorder
<ul>
<li>
however, 25% of pts have no family hx</li>
</ul>
</li>
<li>
-mutation in the APC (Adenomatous polyposis coli) gene → > 300 mutation ID's</li>
<li>
-present in the 2nd to 3rd decades w/  100's or 1000's of adenomatous polyps</li>
<li>
-rate of progression ~variable, but all patients will eventually develop colonic carcinoma(s) in early adult life (age 39)
<ul>
<li>
assoc'd w/ lots of other tumors</li>
</ul>
</li>
<li>
-the treatment is total colectomy.
<ul>
<li>
different options: some with more sparing of fxn than others</li>
</ul>
</li>
</ul>

Question 25

Q

MYH Polyps

<ul>
<li>
caused by?</li>
<li>
present at what age? how?</li>
<li>
natural hx?</li>
<li>
tx?</li>
</ul>

Answer

A

<ul>
<li>
caused by: you have to have bi-allelic mutation in MutY Homolog (base excision repair gene)
<ul>
<li>
can lead to mutation of APC which is esp susceptible to these lesions</li>
</ul>
</li>
<li>
presentation can be the same as FAP but usually < 100 polyps
<ul>
<li>
Mean age at cancer dx = 45 yrs</li>
</ul>
</li>
<li>
natural hx?</li>
<li>
tx?</li>
</ul>

Question 26

Q

HNPCC

<ul>
<li>
caused by?</li>
<li>
present at what age? how?</li>
<li>
natural hx?</li>
<li>
tx?</li>
</ul>

Answer

A

Hereditary Non-Polyposis Colon Cancer

<ul>
<li>
Caused by A.D. inherited germline mutation in mismatch repair genes (MSH2, MLH1, MSH6, PMS2)
<ul>
<li>
correct microsatellite repeats that increas r/o mutation</li>
<li>
can do IHC to see qualitatively if these are missing</li>
</ul>
</li>
<li>
Present with two types
<ul>
<li>
Lynch I = CRC ca's only</li>
<li>
Lynch II = CRC ca's + Endometrial, ovarian, pancreatic, gastric, SB, transitional cell of kidney/ureter</li>
<li>
Have adenomatous polyps that are slightly more dysplastic
<ul>
<li>
Larger with a villous or dysplastic component</li>
<li>
Flat adenomas/serrated adenomas</li>
</ul>
</li>
<li>
More present in proximal colon</li>
<li>
present early avg = 45 y/o</li>
<li>
higher incidence of metachronous cancer</li>
<li>
however, no big phenotypic differences → difficult dx</li>
</ul>
</li>
<li>
natural hx:
<ul>
<li>
earlier dx (~45 y/o)</li>
<li>
tumors progress to cancer faster (~3.5 years) so need more screening</li>
<li>
BUT! Better cancer-specific survival!!</li>
</ul>
</li>
<li>
 </li>
</ul>

Question 27

Q

PJS

Answer

A

Peutz-Jeghers Syndrome: 
-Autosomal dominant 
-Multiple hamartomatous polyps of the entire GIT (esp SB) 
-Mucocutaneous melanosis (lips & buccal mucosa). 
-PJ polyps themselves have no malignant potential, but these patients have a higher incidence of carcinoma elsewhere in the colon 
-Increased frequency of extra-GI cancer

Question 28

Q

Potential lifestyle, diet, meds that protect against CRC

Answer

A

<ul>
<li>
COX2 inhibition</li>
<li>
fiber</li>
<li>
diet/exercise</li>
<li>
Vit b6</li>
<li>
Calcium and vitamin D
<ul>
<li>
Calcium believed to bind fatty and bile acids</li>
</ul>
</li>
<li>
Folic Acid - deficiency may -> DNA hypermethylation</li>
<li>
Omega 3s</li>
</ul>

Question 29

Q

Are most CRCs caused by inheritied syndromes or sporadic occurence?

Question 30

Q

Polyps

<ul>
<li>
Two types</li>
<li>
Causes of and percent that are neoplastic</li>
<li>
Cause of and percent that are non-neoplastic</li>
</ul>

Answer

A

<ul>
<li>
sessile and pedunculated</li>
<li>
90% are non-neoplastic caused by:
<ul>
<li>
Hyperplastic</li>
<li>
Hamartomatous polyps (Juvenile, PJS)</li>
<li>
Inflam. pseudopolyps</li>
</ul>
</li>
<li>
neoplastic (adenomas) are only 10%</li>
</ul>

Question 31

Q

Tubular adenomatous polyp

<ul>
<li>
appearance</li>
<li>
malig risk</li>
</ul>

Answer

A

Tubular

<ul>
<li>
LOWEST MALIG RISK</li>
<li>
small and pedunculated
<ul>
<li>
polyp only in head</li>
</ul>
</li>
<li>
most pedunculated polyps are TAs</li>
</ul>

Question 32

Q

Tubulovilous adenoma polyp

<ul>
<li>
apperance</li>
<li>
malig risk</li>
</ul>

Answer

A

mix of other two (Combined tubular and villous architecture)

Risk of harboring in situ or invasive carcinoma generally correlates with the proportion of the lesion that is villous (i.e. more villous = worse)

Question 33

Q

vilous adenoma polyp

Question 34

Q

Hyperplastic polyp

<ul>
<li>
malig risk</li>
<li>
appearance</li>
<li>
other</li>
</ul>

Answer

A

<ul>
<li>
benign</li>
<li>
Small (usually <5 mm) sessile polyps
<ul>
<li>
Elongated and serrated, stellate crypts lined by “hypermature” goblet cells</li>
</ul>
</li>
<li>
Most common in the rectum/sigmoid</li>
</ul>

Question 35

Q

Juvenile or Retention Polyp

Answer

A

<ul>
<li>
Rare A.D. hamartomatous malformations</li>
<li>
Most frequent in the rectum</li>
<li>
Most often in children (<5yoa)</li>
<li>
Usually large (1 to 3 cm), round, smooth lesions with stalks</li>
<li>
Inflamed lamina propria with cystically dilated glands</li>
<li>
No malignant potential but slight incr risk of adenomas and adenocarcinoma</li>
</ul>

Question 36

Q

PJS

Answer

A

Peutz-Jeghers Polyp

<ul>
<li>
Hamartomatous</li>
<li>
Branching smooth muscle and glands lined by goblet cells</li>
<li>
Slight malignant potential</li>
<li>
(LOH at LKB1 locus)</li>
<li>
PJS pts at increased risk of developing CA of pancreas, breast, lung, ovary, and uterus.</li>
</ul>

Question 37

Q

OBJECTIVE: Outline the different screening options and recommendations for CRC.

Answer

A

Starting at age 50 USPSTF gives A-level evidence for:

<ul>
<li>FOBT - every year, if normal; (if abnormal order colo)</li>
<li>Sigmoidoscopy - Every five years if normal (more often and/or order full colo if polyp removed)</li>
<li>Colonoscopy - Every ten years if normal (more often if polyps removed)</li>
</ul>

Question 38

Q

Classic presentation and pathological apperance of left- vs. right-sided colon tumors?

Answer

A

Left - obstructs apple core

<ul>
<li>grow as annular,  circumferential ("napkin ring"), ulcerated masses</li>
</ul>

Right - bleeds cauliflower

<ul>
<li>grow as polypoid, exophytic masse
<ul>
<li>May still be deeply infiltrating</li>
</ul>
</li>
<li>Obstruction is uncommon</li>
<li>bleeding either occult or melena</li>
</ul>

Question 39

Q

Staging of tumors

Answer

A

Uses TNM (tumors, nodes, mets) rubric

pT is based on how far it invades, you just add one number for each layer of the colon it busts through (p stands for primary tumor)

N is number of nodes involved

M is number of distant mets

<ul>
<li>pTis =
<ul>
<li>Intraepithelial carcinoma(Severe dysplasia)

<ul>
<li>No met risk</li>
</ul>
</li>
<li>Intramucosal carcinoma: Ltd to lamina propria.
<ul>
<li>Very low met risk</li>
</ul>
</li>
</ul>
</li>
<li>pT1 = Invasion into submucosa
<ul>
<li>95% survival</li>
</ul>
</li>
<li>pT2 = Into muscularis propria</li>
<li>pT3 = Through muscularis propria</li>
<li>pT4 = Penetration of serosa or invasionof adjacent viscera
<ul>
<li>30% survival</li>
</ul>
</li>
</ul>

Question 40

Q

Barium enema in pt w/ ∆ in bowel habits shows the following. Thoughts? n/ame for sign?

Answer

A

This is the apple core sign in the sigmoid colon which is suggestive of the annular, napkin ring-type lesions typical of the L colon.

Question 41

Q

What are the main goals of surgery to resect CRC?

Answer

A

<ol>
<li>to stage dz</li>
<li>Resection of 1° lesion, w/ en-bloc excision of adjacent organ extension PRN</li>
<li>Remove regional LNs
<ul>
<li>They follow the regional blood supply</li>
</ul>
</li>
<li>Remove isolated mets</li>
</ol>

Question 42

Q

Lynch Syndrome

Answer

A

Hereditary nonpolyposis colorectal cancer (HNPCC)

—CRCs due to Lynch Syndrome are at higher risk for malignancy/seriousness

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03-06 Tumors of the Bowel + PATH Flashcards

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