Path Winter 8 Gi Tumors

Front 1

where are most GI tumors
is it deadly
what kind of cancer

Back 1

large intestine, LOTS of primary tumors
2 killer (lung is 1)
most are adenocarcinoma

sm intestine is uncommon for cancer

Front 2

what is the frequency of sm intestine tumors in relation to ALL GI tumors

what are 3 benign sm intestine lesions
what is the 1 malignant

Risk factos for malignant sm intestine tumor

Back 2

1. <5%, uncommon

Benign
1. ademona
2. GI stromal tumor (GIST)
3. Peutz Jaghers polyp

Malignant: Adenocarcinoma
Risk: chrons, FAP, HNPCC, Celiac

Front 3

what is the dif btwn adenomas and adenocarcinoma

Back 3

both are sm intestine tumor

adeonom is benign
adenocarcinoma is malignant: risk- chrons, FAP, HNPCC, celiac

Front 4

what tumor is this

40-70 yo w/napkin ring encurcling pattern of duodenum. Pt has cramping, pain, weight loss, NV and occult blood loss and fatigue

Back 4

adenocarcinoma, only malignant tumor of sm intestine

**can cause intussucption, at dx most are stage 4

Front 5

what are 3 things to classify polyps

Back 5

1. attachment to wall- pedunculated or sessile
2. histology- hyperplastic or adenomatous
3. Neoplastic Potential- benign or malignant

Front 6

adenoma
GIST
Peutz Jeghers

Back 6

all are benign tumor of small intestine

Front 7

what are the 4 types of non neoplastic polyps

Back 7

1. inflammatory: solitary rectal ulcer syndrome

2. harmartomatous- sporadic or syndrome. Juvenile, peutx jegher, cowden, babbayab ruvalcabe, Cronkhite-Canada syndrome

3. lymphoid

5. hyperplastic

Front 8

what are the 5 GI polyosis with hamartomatous polyp

Back 8

1. Juveline
2. Peutz Jeghers
3. Cowden
4. Cronkite

Front 9

tell me about Juveline polyps

Back 9

sm intestine, hamartomatous polyp

<5yo
rectum, rectal bleed
risk of adenocarcinoma
associated with pulm AV malformation, clubbing
mutations: SMAD4, BMPR1A, autosomal dominant

On sectioning have cystic spaces = dilated
glands filled with mucin and inflammatory
debris

Front 10

SMAD4, BMPR1A

Back 10

juveline polyps

kids under 5, rectal bleed,AD disorder, 3-100 polyps, pulm AV malformations--> clubbing, On sectioning have cystic spaces = dilated
glands filled with mucin and inflammatory debris

increased risk of other GI adenocarcinomas (the majority)

Front 11

Peutz Jeghers

Back 11

hamartomous polyp

AD, 11yo kids
blue/black macules around eye, mouth, nose, buccal mucosa, palmar surface of hands, genitals, perianal

can initiate intussucption, increased risk of colon, pancreas, breast, lung etc cancer

LKB1/STK11 gene mutation

Front 12

what hamartomous polyp is ossociated with blue/bwron/block hyperpigmentation around mucosa

Back 12

Peutz Jeghers syndrome, 11 yo

AD- LKB1/STK11 gene
increased risk of other malignancy: colon, pancreas, breast, lung, ovary, uterus, testicle

common in SI, large pedunculated
SM present

Front 13

what is the large pedunculated polyp in the sm intestine with sm muscle that is associated with increase in LOTS of cancers and LKB1/STK11

Back 13

Peutz-Jeghers Syndrome- hamartomous polyp

Front 14

tell me about the hemartomatous polyps syndrome Cowden and Babbayab-Ruvalcabe syndromes

Back 14

LOF mutation in PTEN

Front 15

what is associated with:

1. PTEN
2. SMDA4, BMPR1A
3. KHB1/STK11

Back 15

1. PTEN- Cowden and Babbayab-Ruvalcabe (<15)

2. Juvelile polyps (<5)

3. Peutz Jeghers (10-15)

Front 16

tell me about hte hamartomatous polyp syndrime cronkhite canada syndrome

Back 16

>50
NOT hereditary
wt loss, abd pain, weakness.
1/2 are fatal

Front 17

what is teh hamartomatous polys that is NOT congenital

Back 17

crinkhite, polyps

>50, not hereditary, half are fatal
weight loss, abd pain, weakness

Front 18

what does a hyperplastic polyp look like
are they malignant

what do you need to be able to tell them apart from

Back 18

1. goblet cells pile up, left colon, <5mm, multiple

2. NO malignant potential

3. distingiush from sessile serrated ademoma which DOES have malignant potential

Front 19

what polyp is less than 5 mm and found in multiples in the L colon, it has NO malignant potential. what must it be distinguised from

Back 19

hyperplastic

distinguish from sessile serrated ademoma which DOES have malignant potential: located on R side, precursor of colorectal colon

Front 20

what is the most common/clinically important NEOPLASTIC poly in teh colon

what do they look like, who gets them

Back 20

1. colinic adenoma: precursor to colorectal cacner, do screening colonoscopy

2. ppl over 50, M=W. they range from small pedunculated ones to large sessile lesions

**they are the result of proliforation adn dysplasia, they are true neoplastic lesiosn

Front 21

tell me about colonic adenomas

Back 21

precancerous, take them out

small pedunculated to large sessile lesions, like M adn Women adn common in ppl over 50

Front 22

do most colonic adenomas progress to carconomas

Back 22

nope, most are silent. BUT they can turn into colorectal cancer

**they range from small pedunculatd ones to large sessile ones.

Front 23

ok so adenomas in the colon are precacner (proliforation and dysplasia)


we know they vary in shape, what shape is most and least linked to cancer

Back 23

1. Tubular adenoma- low risk

2. sessile villous- high risk

**maximum diameter is the cheif determinant in risk of adenoma --> carcinoma

Front 24

sessile villous
adenomas

Back 24

old ppl

Velvety, cauliflower -like masses, project above
surrounding mucus

surrounded with sysplastic colmunar epithelium

Front 25

contrast a sessile serrated adenoma with typical ademona

Back 25

Sessile serrated: overlap histo with hyperplastic, R colon, serrated throughout full length of glands, potential for malignancy

Typical:

Front 26

FAP

1. Pathway
2. # polyps for dx
3. cancer prevention
4. how common is cancer development

Back 26

1. APC/WNT path OR DNA mismatch w/MUTYH mutation
2. 100 (500-2500 typical)
3. Prophylactic colectomy
4. 100% get cancer

**get congenital hypertrophy of retinal pigment epithelium

Front 27

HNPCC (Hereditary nonpolypsosis colorectal cancer)

1. genetic defect
2. genetic transmission
3. other name
4. increased risk of...
3.

Back 27

1. genetic defect: MSH2, MLH1, mismatch defect. micrrosattelite instability

2. genetic transmission: germ line mutation

3. other name: lynch

4. increased risk of colorectal cancer AND extraintestinal CA

Front 28

what is APC/WNT associated with

Back 28

FAP, autosomal dom transmission. tubular villious typical adenocarcinoma

**less commonly FAP is associated with DNA mismatch repair. sessil eserrated adenoma

Front 29

which polyp disease is assoicated with 100% cancer progression, and 100's of polyps

Back 29

FAP- APC/WNT autosomal dom transmission

Front 30

whats gardner syndrom

what is turcot syndrome

Back 30

variant of FAP, you get lots of osteomas in skull, mandible, long bones. specific APC mutation

variant of APC with CNS tumors

Front 31

what is the variation of FAP that has lots of osteomas

what about hte FAP variant that has lots of CNS gliomas

Back 31

gardners

turcot

Front 32

whats attenuated FAP

Back 32

MUHY mutation, delayed polyp development
less than 100 adenomas
*delayed appearance of cancer

Front 33

what is the disease assoicated with defective mismatch repair genes (MSH2, MLH1) in the germ line. It is associated with increased CRC and also extraintestinal cacner

Back 33

lynch, Hereditary nonpolymosis CRC (HNCRC)

Front 34

what is the most common cancer of GI tract? whats it associated with

Back 34

adenocarcinoma of colon

causes 2 most cancer deaths (2 to lung)

Lots of things (diet- high fat high carb low fiber) ASA, NSAIDS are PROTECTIVE

Front 35

epidemiology of adenocarcinoma of colon

(MOST common GI tumor and 2 most common cancer killer)

Back 35

? old ppl

Front 36

what are the 3 molecular pathways for the development of colon cancer

Back 36

1. APB/b catenin path (stepwise mutations in oncogenes and tumor supressors, present in 80% of sporadic colon cancers)

2. Mismatch repair (microsatellite instability)

3. loss of p53, SMAD2/4

Front 37

tell me about APC/B catenin path

Back 37

leads to colon cancer, 80% of sporadic colon cancer have defect here

chromosome instability bc of stepwise accumulate in oncogenes ann tumor suppressor genes

1. loss of APC tumor suppressor at 5q21 (FAP, cardner,) this is the "first hit"
2. APC regulates b catenin, part of WNt pathway
3. K RAS mutation

Front 38

tell me about the 2 hit for HNPCC

Back 38

HNPCC patients inherit one mutant DNA repair
gene (first hit) and are susceptible to second
somatic mutation (second hit)

Front 39

how do cecal and R sided lesions present

what about L sided

Back 39

Right: polypoid, exophytic, obstruction uncommon, fatigue, weight loss, Fe dificit anemia, bulky lesions that bleed easily

Left: annular, encircling, constricting, (napkin rink) obstructing. occult bleed, change in bowel (melena, diarreha, constipation) constipation

**histo for R and L same: rage in appearnace, columnar to anaplastic, hard, mucin,

Front 40

what is Fe deficit in an old man mean

Back 40

Gi cancer until proven otherwise

Front 41

where does colon cancer mets to

Back 41

regional LN
liver
lung
bone

Front 42

what is the most important prognostic marker for CRC,

Back 42

mucin,

aids in dissemination through
wall, worsens prognosis

Front 43

staging for CRC

Back 43

TNM classiciation

Tumor
insitu, mucosa only
T1into submucosa
T2 into muscularis
T3 entire wall

Front 44

where is the cancer

Fatigue, weaknes, Fe deficit anemia- lots of bleeding, bulky lesion

occult bleed, cramping, changes in poo

Back 44

right


left, obstructive

Front 45

where do carcinoids come from
where do they arise often
what do they look like

Back 45

1. they are neuroendocrine tumors and secrete stuff!
2. GI, appendix most, good bc wont spread (appey-Sm intest, rectum, stomach, colon)

3. in the appendix its a bulbous swelling of tip- lumen is obliterated. Elsewhere- intramural, submucoasal mass, yellow/tan

*all are potentially malignant

Front 46

aggressive tumors correlate with what

Back 46

1. site
2. depth/penetration
3. size of tumor

**most ileal, gastric and colon spread. the rectal and appendix ones dont spread

Front 47

what does mucin in a colon cancer mean


what is THE bad prognostic marker of carcinoid tumor

Back 47

BAD news

location location location (appendix/rectum least likely to spread)

Front 48

carcinoids where dont usually meds,

Back 48

in the appendix, rectal

Front 49

what is carcinoid syndrome likely due to

what are 3 common sx of carcinoid syndrome

Back 49

excess erotonin, urine 5HiAA is dx'

**needs tumor to secrete directly into non portal veins, strong association with liver mets

Front 50

what is the overall survival rate for carcinoids

Back 50

all are potentially malignant, LOCATION, depth of penetration, adn the size all determine aggressiveness. appendix and rectal is a good location

seems good

Front 51

what tumor in the GI is common in appendix, tan/yellow, and has scant ppink granular cytoplasm

Back 51

carcinoids- the neuroendocrine tumor

really granular

Front 52

what is the clinical for carcinoids

Back 52

1. sx based on secretory products

gastric/pancreatic release secretory products directly into circulation

zollinger ellison- gastrin carcinoid

Front 53

where are GI lymphomas common

Back 53

**2 most common SI cancer in industrualized places)

Front 54

what are the 3 types of GI lymphoma

Back 54

1. enteropathy type intestinal T cell lymphoma: CD130, not CD4/8. complicates celiac

2. medeterranean lymphoma: poor men, a heavy chain disease

3. western intestinal lymphoma: >40 & <10. arise from malt and cause obstruction, intussusception, and perforation. occult blood

Front 55

who gets GI lymphoma

Back 55

1. pts w/H pylori
2. meteterraneans
3. HIV
4. sprue
5. immunosuppressive therapy

Front 56

tell me about Enteropathy-type intestinal T-cell (EITCL)

Back 56

complicated celiac disease (non tropical sprue)

widespread at dx

CD130, NOT CD4 or 8

Front 57

tell me about medeterranian lymphoma

Back 57

low SES, men

a heave chain disease: immunoproliferative small intestinal disease

Diffuse infiltrate of plasma cells/plasmacytoid
lymphocytes
• Infiltrates in mucosa lead to atrophy and
malabsorption

Front 58

Western-Type Intestinal Lymphoma

who
arise?
complication/sx

Back 58

>40 <10
arises from MALT

obstruction, intussusception, perforation. occult bleed

Front 59

in addition to the normal "GI" sx- diarrhea, pain, weight loss. what additional feature is seen in lymphoma

Back 59

clubbing of fingers

Enteropathy-type intestinal T-cell lymphoma
• Mediterranean Lymphoma
• Western-type intestinal lymphoma

Front 60

how do GI lymphomas typically fo

Back 60

good, 85% ten year survival

present with insidious or blood loss and obstruction

Front 61

whats GIST

Back 61

GastroIntestinal Stromal Tumor

1. SM differentiation
2. neural differntiation
3. SM AND NEURO
4. no differentiation

**more common in stomach
**cKIT mutation

Front 62

what mutation is assoicated with GIST

Back 62

cKIT

Front 63

now that we can recognize cKIT what changed

Back 63

its the mutation in GIST

Now we can have drugs specifically to tumor

the RTK inhibitor imatinib used :)

Front 64

give me an example of a GIST

Back 64

lipoma- well demarcated, submucosa or muscularis propria

leiomyosarcoma- large bulky

Front 65

what are the kinds of anal canal tumors

Back 65

Basaloid pattern
• Squamous cell carcinoma
• Adenocarcinoma

Front 66

sq cell carcinoma of anal canal is associated with what

Back 66

lower anal cancal

Front 67

what is the most common tumor inthe appendix, prognosis

Back 67

carcinoid, good :) mets is rare

Front 68

a dilated appendix full of mucin is called what

Back 68

Mucocele
• Globular enlargement due to inspissated mucus,
usually result of obstruction (fecalith)
• Distention atrophies cells, mucin production stops
• Rare rupture
• Usually asymptomatic

Front 69

what are the 3 mucinous lesions of the apendix

Back 69

- Benign mucocele
– Mucinous cystadenoma**most common (benign)
– Mucinous cystadenocarcinoma, malignant

**all get dilated

Front 70

what is pseudomyxoma peritoneii, what cells are in it

Back 70

its tenacious semisolid mucin that fills the abdomen in Malignant mucinous cystadenocarcinoma. continued cell proliforation nad mucin secreiotn after spread to peritoneum


differentiated adenocarcinoma cells can be
found in mucin

Front 71

whats Mucinous Cystadenoma

Back 71

most common mucinous lesion of appendix. benign

distends, perforation 20%, NOT malignant

Front 72

Malignant mucinous cystadenocarcinoma

Back 72

not as common, mucin filled appendix

*dilation, penetrate and spread to peritoneum

pseudomyxoma peritonei- when you get proliforation and mucin secretion adn it fills the abdomen with tenacious semisolic mucin

Front 73

what are the 2 types of peritoneal tumors

Back 73

Primary: mesothelioma

Secondary: common. met seeding from other cancer. ovary and pancreas is common

**almost ALL are malignant

Front 74

what are the 2 primary tpes of peritoneal tumors

Back 74

mesotheliomas,

Desmoplastic small round cell tumor

Front 75

what 2 tumors in the peritoneum commonly cause produce serosal implantation (secondary)

Back 75

ovary
pancreas

Front 76

M.R. is a 60 y/o female patient who was in good health until she tripped and fell in her home and sustained a lumbar compression fracture of L1 and
L2. Unexplained anemia was discovered when she
was admitted to the hospital. The rectal exam was
omitted by the orthopedic surgeon because of the
back injury.
• Test results:
– Hbg 10
– Hct 31
– MCV 61 (80-90)
– Serum iron 49 (45-160)

Two fecal hemoccult test were negative
• She was discharged after three days, and her internist
initiated a work-up for anemia. Iron replacent
therapy was commenced. Upper GI x-ray studies
were normal. Barium enema demonstrated a cecal
mass measuring about 4x4 cm. A small polyp was
seen in the transverse colon. Colonoscopy was done
for tissue diagnosis and to R/O synchronous lesions
in the colon. Biopsy of the cecal mass showed a
malignant lesion. The polyp was removed by
endoscopic polypectomy and was a pedunculated
adenomatous polyp. A partial colectomy was performedThere was a
polypoid mass, 7x4.5 cm. The lesion was
histologically malignant. 28 lymph nodes were
negative for tumor cells. The tumor was classified
as Duke’s B2 colon carcinoma. The post op course
was uneventful. Barium enema exam one yer after
surgery was negative and colonoscopy two yers after
resection revealed a normal ileocolic anastamosis and a 1 cm adenomatous polyp in the sigmoid colon. Her CBC remained normal after surgery. Carcinoembryonic antigen (CEA) was normal preop. However, 4 years after surgery her CEA value
increased to 60 (<3). Other labs included a alkaline
phosphatase of 202 (30-115), AST 240 (10-41). A
liver-spleen scan revealed multiple masses in the
liver.

Back 76

sporadic CRC

Front 77

Neoplastic Epithelial Lesions

Back 77

Benign polyps
Adenomas
Malignant lesions
Adenocarcinoma
Squamous cell carcinoma of the anus

Front 78

Non-neoplastic Polyps

Back 78

Hyperplastic polyps Hamartomatous polyps
Juvenile polyps
Peutz Jheger
Inflammatory
Lymphoid

Front 79

Other Tumors

Back 79

Gastrointestinal stromal tumors
Carcinoid tumor
Lymphoma

Front 80

what are the 2 types of hereditary CRC carcinoma

Back 80

1. FAP: adenocarcinoma sequence. TONS of polyps, germline APC inactivation.

2. HNPCC: microsatellite instability, mucinious, poorly differentiated, lymphocytic infiltrates. germ line mutation of MLH1 or MSH2.

Front 81

tell me about R and L sided sporadic cases of CRC

Back 81

LEFT: tubular, tubulovillous, villious adenomas. mod differentiated. comatic inactivation of mutation of several genes

RIGHT: NO precursor lesion. sessile serrated adenomas, large hyperplastic polyps, mucunous carcinomas. somatic inactivation of MLH1 otMSH2 repain genes (GERM line of these genes is HNPCC)

Front 82

A 43 y/o man came into the emergency room
because of intense abdominal pain associated with
abdominal swelling. A plain film of the abdomen
showed numerous dilated loops of small intestine. A
laparotomy was performed. The lower ileum was
found to have a palpable mass lesion in the wall that
obstructed the lumen. A segmental resection of
ileum was performed. The section shows a segment
of buckled small bowel. At the apex of the buckle,
the mucosa is ulcerated. Beneath this are small
invasive nests of a neoplasm composed of
monotonous, bland cells

Dx

Common Location

Associated sx

Back 82

Diagnosis: Carcinoid
• Most common sites: Small intestine,
appendix
• Associated syndrome: Carcinoid syndrome
due to mets in the liver, produce serotonin,
gets flushing, diarrhea, asthma

Front 83

A 45 y/o man was found to have a positive guaiac
test on routine physical exam. During colonoscopy,
a 1.3 cm diameter polypoid lesion on a short stalk
was found in the descending colon and was resected.
The gross photograph shows another case of a
patient with several of these lesions. Another patient
with a similar disease but different gross appearance
is shown next. The microscopic section shows a
polyp at low power that consists of closely packed
tubular glands lined by cells with depleted
cytoplasmic mucin and hyperchromatic, stratified
nuclei and occasional mitosis.

Dx

Back 83

Diagnosis
– Tubular adenoma, precursor of colon cancer

Front 84

This was an incidental finding in a 15 y/o girl
that was found at the time of appendectomy. A
discrete yellow mass was observed in the small
bowel wall and was resected.

dx

Back 84

Diagnosis
– Lipoma

Front 85

A 21 y/o male is found to have a positive stool
guaiac from rectal exam during a routine physical.
• He tells you that several relatives died from “cancer
of the bowel” at an early age.

Dx

Back 85

FAP

Front 86

DN is a 60 y/o female who saw her primary care
physician for rectal bleeding of 3 months duration.
She experienced rectal pain followed by bloodon the
toilet paper. No blood stained her stool. Her bowel
habits were reported unchanged: she had 2 loose
stools/day. There was no straining at stool. Her
appetite was good. There was no weight loss. Past
surgeries were hysterectomy and lumpectomy of the
breast. Hgb 12.6
• Hct 37.2
• BUN 30(7-22)
• Creat 1.7(<1.2)
• GGTP 77 (0-45)
• On physical exam, a palpable mass was detected by
digital rectal exam. Hemoccult of the stool was
strongly positive. Colonoscopy was performed. A
circumferential, neraly obstructing, fixed, firm,
ulcerated tumor occupied the rectum. The adult
colonoscope would not pass through the tumor
surrounded lumen so a pediatric scope was used

Back 86

maybe HNPCC?

Front 87

VE is a 73 y/o female who presented to her primary
care physician c/o diarrhea for 2 months duration.
Bowel habits had changed at least 6 months ealier
with frequent episodes of diarrhea. She was passing
4-5 stools/day that were loose with no blood or pus.
She reported feeling a constant urge to defecate.
• She did not smoke. 1 glass wine/week. No family hx
of cancer. Appendectomy 45 yrs. ago
• Barium enema showed an obstructing apple core
lesion of the splenic flexure.
• Hgb 11.1
• Hct 33

She was taken to surgery where a hemicolectomy
was performed to include the splenic flexure. The
gallbladder was found to be distended with stones
and also removed.
• The path report read: Infiltrating, ulcerated, annular,
moderately differentiated adenocarcinoma of the
colon with transmural invasion. Regional lymph
nodes were negative for metastatic tumor.

Back 87

??

Front 88

ML is a 76 y/o female who was seen by her primary
care physician due to recent onset of melena and
passage of bright red blood per rectum staining the
stool. Hemorrhoids were noted on examination and
she had previously attributed rectal bleeding to their
presence. She took aspirin regularly for arthritis
pain.
• Past history included an appendectomy
cancer.
• Lab tests: Hbg 13.4 gm, Hct 40 % with remainder of
CBC within normal limits. Chem profile results
were within normal limits except for cholesterol 241
and triglycerides 335. She underwent total colonoscopy. Four
adenomatous polyps were found distributed
throughout the colon from the sigmoid to the hepatic
flexure. The largest measured 2 cm and was located
in the transverse colon. The polyps were removed
totally by snare. She was scheduled for a repeat
colonoscopy in 6 months. ML was seen 2 weeks later in the emergency room
c/o bright red blood staining the toilet bowel water
after a bowel movement. She was hemodynamically
stable. There was no significant drop in Hbg and
Hct. She was scheduled for repeat colonoscopy. A
small, flat, elevated tumor was visualized in the
distal ascending colon. Biopsy of the lesion was
infiltrating adenocarcinoma. ML underwent hemicolectomy with excision of
ileocecal valve and distal ileum. The resection
specimen contained a fixed, indurated, elevated
tumor 1.6x1.6x1 cm located 4cm distal to the
ileocecal valve. Pathologic diagnosis was:
• Infiltrating poorly differentiated adenocarcinoma of
the ascending colon with invasion into but not
through the muscularis. Regional lymph nodes were
negative for metastatic carcinoma

Back 88

??