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88 Cards in this Set
- Front
- Back
where are most GI tumors |
large intestine, LOTS of primary tumors |
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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 |
1. <5%, uncommon
Benign 1. ademona 2. GI stromal tumor (GIST) 3. Peutz Jaghers polyp Malignant: Adenocarcinoma Risk: chrons, FAP, HNPCC, Celiac |
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what is the dif btwn adenomas and adenocarcinoma
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both are sm intestine tumor
adeonom is benign adenocarcinoma is malignant: risk- chrons, FAP, HNPCC, celiac |
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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 |
adenocarcinoma, only malignant tumor of sm intestine
**can cause intussucption, at dx most are stage 4 |
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what are 3 things to classify polyps
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1. attachment to wall- pedunculated or sessile
2. histology- hyperplastic or adenomatous 3. Neoplastic Potential- benign or malignant |
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adenoma
GIST Peutz Jeghers |
all are benign tumor of small intestine
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what are the 4 types of non neoplastic polyps
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1. inflammatory: solitary rectal ulcer syndrome
2. harmartomatous- sporadic or syndrome. Juvenile, peutx jegher, cowden, babbayab ruvalcabe, Cronkhite-Canada syndrome 3. lymphoid 5. hyperplastic |
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what are the 5 GI polyosis with hamartomatous polyp
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1. Juveline
2. Peutz Jeghers 3. Cowden 4. Cronkite |
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tell me about Juveline polyps
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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 |
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SMAD4, BMPR1A
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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) |
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Peutz Jeghers
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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 |
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what hamartomous polyp is ossociated with blue/bwron/block hyperpigmentation around mucosa
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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 |
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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
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Peutz-Jeghers Syndrome- hamartomous polyp
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tell me about the hemartomatous polyps syndrome Cowden and Babbayab-Ruvalcabe syndromes
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LOF mutation in PTEN
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what is associated with:
1. PTEN 2. SMDA4, BMPR1A 3. KHB1/STK11 |
1. PTEN- Cowden and Babbayab-Ruvalcabe (<15)
2. Juvelile polyps (<5) 3. Peutz Jeghers (10-15) |
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tell me about hte hamartomatous polyp syndrime cronkhite canada syndrome
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>50
NOT hereditary wt loss, abd pain, weakness. 1/2 are fatal |
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what is teh hamartomatous polys that is NOT congenital
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crinkhite, polyps
>50, not hereditary, half are fatal weight loss, abd pain, weakness |
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what does a hyperplastic polyp look like
are they malignant what do you need to be able to tell them apart from |
1. goblet cells pile up, left colon, <5mm, multiple
2. NO malignant potential 3. distingiush from sessile serrated ademoma which DOES have malignant potential |
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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
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hyperplastic
distinguish from sessile serrated ademoma which DOES have malignant potential: located on R side, precursor of colorectal colon |
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what is the most common/clinically important NEOPLASTIC poly in teh colon
what do they look like, who gets them |
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 |
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tell me about colonic adenomas
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precancerous, take them out
small pedunculated to large sessile lesions, like M adn Women adn common in ppl over 50 |
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do most colonic adenomas progress to carconomas
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nope, most are silent. BUT they can turn into colorectal cancer
**they range from small pedunculatd ones to large sessile ones. |
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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 |
1. Tubular adenoma- low risk
2. sessile villous- high risk **maximum diameter is the cheif determinant in risk of adenoma --> carcinoma |
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sessile villous
adenomas |
old ppl
Velvety, cauliflower -like masses, project above surrounding mucus surrounded with sysplastic colmunar epithelium |
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contrast a sessile serrated adenoma with typical ademona
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Sessile serrated: overlap histo with hyperplastic, R colon, serrated throughout full length of glands, potential for malignancy
Typical: |
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FAP
1. Pathway 2. # polyps for dx 3. cancer prevention 4. how common is cancer development |
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 |
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HNPCC (Hereditary nonpolypsosis colorectal cancer)
1. genetic defect 2. genetic transmission 3. other name 4. increased risk of... 3. |
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 |
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what is APC/WNT associated with
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FAP, autosomal dom transmission. tubular villious typical adenocarcinoma
**less commonly FAP is associated with DNA mismatch repair. sessil eserrated adenoma |
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which polyp disease is assoicated with 100% cancer progression, and 100's of polyps
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FAP- APC/WNT autosomal dom transmission
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whats gardner syndrom
what is turcot syndrome |
variant of FAP, you get lots of osteomas in skull, mandible, long bones. specific APC mutation
variant of APC with CNS tumors |
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what is the variation of FAP that has lots of osteomas
what about hte FAP variant that has lots of CNS gliomas |
gardners
turcot |
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whats attenuated FAP
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MUHY mutation, delayed polyp development
less than 100 adenomas *delayed appearance of cancer |
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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
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lynch, Hereditary nonpolymosis CRC (HNCRC)
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what is the most common cancer of GI tract? whats it associated with
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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 |
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epidemiology of adenocarcinoma of colon
(MOST common GI tumor and 2 most common cancer killer) |
? old ppl
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what are the 3 molecular pathways for the development of colon cancer
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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 |
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tell me about APC/B catenin path
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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 |
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tell me about the 2 hit for HNPCC
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HNPCC patients inherit one mutant DNA repair
gene (first hit) and are susceptible to second somatic mutation (second hit) |
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how do cecal and R sided lesions present
what about L sided |
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, |
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what is Fe deficit in an old man mean
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Gi cancer until proven otherwise
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where does colon cancer mets to
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regional LN
liver lung bone |
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what is the most important prognostic marker for CRC,
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mucin,
aids in dissemination through wall, worsens prognosis |
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staging for CRC
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TNM classiciation
Tumor insitu, mucosa only T1into submucosa T2 into muscularis T3 entire wall |
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where is the cancer
Fatigue, weaknes, Fe deficit anemia- lots of bleeding, bulky lesion occult bleed, cramping, changes in poo |
right
left, obstructive |
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where do carcinoids come from
where do they arise often what do they look like |
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 |
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aggressive tumors correlate with what
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1. site
2. depth/penetration 3. size of tumor **most ileal, gastric and colon spread. the rectal and appendix ones dont spread |
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what does mucin in a colon cancer mean
what is THE bad prognostic marker of carcinoid tumor |
BAD news
location location location (appendix/rectum least likely to spread) |
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carcinoids where dont usually meds,
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in the appendix, rectal
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what is carcinoid syndrome likely due to
what are 3 common sx of carcinoid syndrome |
excess erotonin, urine 5HiAA is dx'
**needs tumor to secrete directly into non portal veins, strong association with liver mets |
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what is the overall survival rate for carcinoids
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all are potentially malignant, LOCATION, depth of penetration, adn the size all determine aggressiveness. appendix and rectal is a good location
seems good |
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what tumor in the GI is common in appendix, tan/yellow, and has scant ppink granular cytoplasm
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carcinoids- the neuroendocrine tumor
really granular |
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what is the clinical for carcinoids
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1. sx based on secretory products
gastric/pancreatic release secretory products directly into circulation zollinger ellison- gastrin carcinoid |
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where are GI lymphomas common
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**2 most common SI cancer in industrualized places)
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what are the 3 types of GI lymphoma
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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 |
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who gets GI lymphoma
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1. pts w/H pylori
2. meteterraneans 3. HIV 4. sprue 5. immunosuppressive therapy |
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tell me about Enteropathy-type intestinal T-cell (EITCL)
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complicated celiac disease (non tropical sprue)
widespread at dx CD130, NOT CD4 or 8 |
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tell me about medeterranian lymphoma
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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 |
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Western-Type Intestinal Lymphoma
who arise? complication/sx |
>40 <10
arises from MALT obstruction, intussusception, perforation. occult bleed |
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in addition to the normal "GI" sx- diarrhea, pain, weight loss. what additional feature is seen in lymphoma
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clubbing of fingers
Enteropathy-type intestinal T-cell lymphoma • Mediterranean Lymphoma • Western-type intestinal lymphoma |
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how do GI lymphomas typically fo
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good, 85% ten year survival
present with insidious or blood loss and obstruction |
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whats GIST
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GastroIntestinal Stromal Tumor
1. SM differentiation 2. neural differntiation 3. SM AND NEURO 4. no differentiation **more common in stomach **cKIT mutation |
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what mutation is assoicated with GIST
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cKIT
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now that we can recognize cKIT what changed
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its the mutation in GIST
Now we can have drugs specifically to tumor the RTK inhibitor imatinib used :) |
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give me an example of a GIST
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lipoma- well demarcated, submucosa or muscularis propria
leiomyosarcoma- large bulky |
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what are the kinds of anal canal tumors
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Basaloid pattern
• Squamous cell carcinoma • Adenocarcinoma |
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sq cell carcinoma of anal canal is associated with what
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lower anal cancal
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what is the most common tumor inthe appendix, prognosis
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carcinoid, good :) mets is rare
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a dilated appendix full of mucin is called what
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Mucocele
• Globular enlargement due to inspissated mucus, usually result of obstruction (fecalith) • Distention atrophies cells, mucin production stops • Rare rupture • Usually asymptomatic |
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what are the 3 mucinous lesions of the apendix
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- Benign mucocele
– Mucinous cystadenoma**most common (benign) – Mucinous cystadenocarcinoma, malignant **all get dilated |
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what is pseudomyxoma peritoneii, what cells are in it
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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 |
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whats Mucinous Cystadenoma
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most common mucinous lesion of appendix. benign
distends, perforation 20%, NOT malignant |
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Malignant mucinous cystadenocarcinoma
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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 |
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what are the 2 types of peritoneal tumors
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Primary: mesothelioma
Secondary: common. met seeding from other cancer. ovary and pancreas is common **almost ALL are malignant |
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what are the 2 primary tpes of peritoneal tumors
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mesotheliomas,
Desmoplastic small round cell tumor |
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what 2 tumors in the peritoneum commonly cause produce serosal implantation (secondary)
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ovary
pancreas |
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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. |
sporadic CRC
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Neoplastic Epithelial Lesions
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Benign polyps
Adenomas Malignant lesions Adenocarcinoma Squamous cell carcinoma of the anus |
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Non-neoplastic Polyps
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Hyperplastic polyps Hamartomatous polyps
Juvenile polyps Peutz Jheger Inflammatory Lymphoid |
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Other Tumors
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Gastrointestinal stromal tumors
Carcinoid tumor Lymphoma |
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what are the 2 types of hereditary CRC carcinoma
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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. |
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tell me about R and L sided sporadic cases of CRC
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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) |
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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 |
Diagnosis: Carcinoid
• Most common sites: Small intestine, appendix • Associated syndrome: Carcinoid syndrome due to mets in the liver, produce serotonin, gets flushing, diarrhea, asthma |
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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 |
Diagnosis
– Tubular adenoma, precursor of colon cancer |
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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 |
Diagnosis
– Lipoma |
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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 |
FAP
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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 |
maybe HNPCC?
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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. |
??
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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 |
??
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