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GI Malignant Flashcards

1
Q

Signs of PPI drug use?

A

Parietal cell hyperplasia

Parietal cell hypertrophy

Parietal cell vaculolation

Semi-serrated apperance

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2
Q

Familial adenomatous polyposis (genes)?

What is Menerier’s disease?

A

APC (90%), b-catenin (10%) in familair/ sporatic is reversed

Multiple hyperplastic polyps, Watermelon stomach, dilated corkscrew glands, can extend into muscularis mucosa, possible increased intraepithelial lymphocytes

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3
Q

Findings in Peutz-Jegher’s Hamartomatous polyp?

A

Epithelium: Unremarkable

Pit and Glands: Grouped or packed with intervening septation of smooth muscle

Lamina propria: Unremarkable

Smooth Muscle: Short wispy or chunky bundles not connecte dto muscularis mucosae; vs Hyperplastic that connect

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4
Q

Findings in Juvenile Polyp (Hamartomatous)?

A

Epithelium: Eroded or Normal

Pits and glands: Disorganized with varying shapes/sizes. Edematous club shapped or irredular villiform structures

Lamina propria: Edematours, Granulation tissue common

Smooth muscle: Unremarkable

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5
Q

Hyperplastic (Hamartomatous polyp) findings?

A

Epithelium: Damaged wtih reactive or regenreative chagnes, chemical gastropathy changes

Pit and Gland: Surface pits connected to deeper portions of glands (linear trajectory), small, regular and orderly glands with possible surface erosion

Lamina Propria: Unremarkable or inflammed

Smooth muscle: Long sweeping bundles that connected to muscularis mucosae

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6
Q
A
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7
Q

Gene in Peutz-Jegher syndrome?

What cancers in skin, GI, breast, and GYN?

A

LKB1/STK11

Skin: Melanotic pigmentation of digits, genitalia, lips, oral mucosa, etc.

GI: Hamartoma, increased colorectal risk, intussusception and bleeding, Adeno of GI (including pancreas)

Breast: CA

GYN: Sex-cord tumor with annular tubules (almost all), ovarian mucinous tumor, adenoma malignum of cervic well differentiated gastic type mucosa)

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8
Q

Juvenile polyposis syndrome, Autodominant genes?

True or false large proportion have no family history?

Diagnostic criteria?

True or false: Increased risk of colorectal gastric carcinoma?

A

Dysregulation of TGF-B, SMAD4 (20% familial cases), BMPR1A (25%)

True: incomplete penetrance=large population w/o family history

3-10 juvenile polyps or JP in entire GI tract, Any number with history of juvenile polyposis

True; 55% increase in colorectal and gastric

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9
Q

Cowden syndrome?

What is Cronkhite-Canada Syndomre?

A

Hamartomatous overgrowths:
Skin: Trichilemmoma, Acral Keratosis, Oral papillomas

GI: Hamartoma polyps (similar to juvenile), increased CA risk

Breast: Fibroadenoma, FCC, CA

Endometrium: increased risk of CA

Thyroid: Goiter, Adenoma, CA

Gene: AD/Germline PTEN 70-80% of patients, SDH less common

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10
Q

Cronkhite-Canada syndrome?

Is it possibly an autoimmune disorder?

A

Not inheritied-unknown etiology (autoimmune?)

Entire GI tract (except esophagus) with polyps (CA 25% of cases), ectodermal changes (alopecia, nail atrophy, hyperpigmentation),

Polyps are of juvenile morphology

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11
Q

In what familial disease do gastric adenomas occurs?

Are they more common in the US or Asia?

A

FAP, or spontaneously

Asia 9-27% in Japan/China, 0.6-4% in Western Countries

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12
Q

Stomach dysplasia IHCs?

Intestinal ademona/dysplasia: CD10, MUC2?

Foveolar MUC5AC, MUC6?

Pyloric MUC5AC, MUC6?

A

Intestinal ademoa: CD10+, MUC2+, looks like colonic TA

Foveolar: MUC5AC+, MUC6+/-, MUC2- Tall cells with clear cytoplasma and low columnar cuboidal nuclei

Pyloric: MUC5AC+/-, MUC6+, pale eosinophilic ground glass cytomplasm with round/oval nuclei

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13
Q

Glomus tumor tumor morphology/stains?

Arises from?

Common locations?

Confused with?

A

SMA+, Caponin+, Coollagen 4+, S-100-, C-kit-, chromogramin-: Cellular nodules, hemorrhage common, Staghorn vessels, Sharp cell membranes

Neuromyoarterial glomus body

Common in fingers

Confused with GIST (DOG1+, C-kit+)

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14
Q

GIST, 3 histologic types/stains?

What is seen in the paranuclear space?

Can it have myxoid changes and osseous metaplasia?

What is the Kit status of dedifferentieded GIST?

A

Circumscribed in wall of stomach, Spindle, Epithelioid, Mixed

C-kit, DOG1 Positive

**Paranuclear vaculolization (vs leiomyoma)

Yes, can mimic schwanoma

C-kit Negative c/ atypia**

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15
Q

GIST (KIT and PDGFRA are on what chromosome?

GIST on Exon 11 what is better “inframe deletion or missense”?

Small intesting GIST good or bad prognosis/exon?

Do some GISTS have BRAF mutations?

A

Long arm 4; PDGFRA (7%); activated tyrosine kinase

Missense! ; deletion bad prognosis

BAD! Exon 9

Yes, Classic V600E

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16
Q

In stomach were are inflammatory myofibroblastic tumors seem?

Uniform or not?

IHC stains?

Schwanoma stains?

A

Mesenary?

Uniform spindle cells

Actin +, calponin +, focal desmin +, caldesmon +, CD34-

S100+, CD117-, CD34-

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17
Q

Inflammatory fibroid polyp (gene involved)?

Staining pattern?

A

Can look like anaplastic cells with some spindled; PDGFRA

CD34+, PDGFR-a +, Calponin +, Cyclin D1 +, Fascin +, CD117-, Caldesmon -, S100 -, Desmin -, keratin -

18
Q

T/F Diffuse type adeno carcinoma comes from athrophic metaplasia?

What is the mutation in diffuse type?

Intestinal type comes from?

A

F: Comes from chronic inflammation

Her-2neu mutation with poor prognosis

Multiple steps form atrophy to metaplasia to cancer, H. pylori and diet

Better prognosis

19
Q

Gastric xanthoma mimics? IHC?

Hepatoid/a-fetoprotein Carcinomas, IHC vs metastatic HCC?

Medullary Carcimoma of stomach, microscopic finding and viral association?

A

Foamy cytoplasm can mimic gastric adeno; CD68+, CK-

CK19+,CK20+, Hep-par1-

EBV, see lymphoepithelioma-like areas

20
Q

Serous cystadenoma, assocated with?

CEA level?

Gross finding?

H and E?

A

Well circumscribed thin wall cyst, VHL; NORMAL CEA

Microcysstic without communication to normal ducts

Central stellate fibrous scar (other board finding is FNH in liver)

Cuboidal lined cysts with flat epithelium (CK7+, GLUT1+)

21
Q
A
22
Q

Mucinous cystadenoma?

CEA levels?

Cancer risk?

H and E/IHC?

High grade transformation has what gene issue?

A

~40 yrs old; does not communicate with duct

Multiloculated cystic mass; CEA levels >250 ng/mL

15% have cancer

Ovarian stroma, ER/PR+

KRAS2+

23
Q

IPMN?

Amylase levels?

4 types?

Genes?

A

Grossly visable, papillary, non-invasive; must involve main or branch duct; head of pancreas

High (also high in pseudocyst)

Gastric (MUCSAC+), intestinal (MUC2/CDX2+), pancreatobiliar (MUC1+), oncocytic (MUC6); high and low dysplasia

KRAS, TP53, CDKN2A

24
Q

PanIN definition?

A

Microscopic (vs IPMN); flat or papillary non invasive neoplasm; <5 mm

Can be low or high grade (non discript- to TA like; low grade); high grade smaller and ugly

25
Q

Intraductal tubulopapillay neoplasm (ITPN)?

H and E?
IHC?

Molecular?

A

Solid and nodule tumor obstructing dilated main pancreatic ducts without mucin

Tubulopapillary (cribiform) growth, High grade atypia

CK7+, CK19+, typsin -, MUC2-, MUC5AC-

Genes: Loss of CDKN2A; PI3K pathway

Mutation of chromatin remodeling genes (MLL1/2/3, BAP1)

26
Q

What is colloid carcinoma (variant of pancreatic ductal adenocarcinoma)?

Association?

A

Large extracellular stromal mucin with pools of floating tumor (80%)s

A/W IPMN

27
Q

Hepatoid carcinoma (Pancreatic ductal variant)?

IHC?

A

Large polygonal cells with eosinophlic cyto

AFP/HepPar1+, canaliclular CD10 and pCEA

28
Q

Medullary carcinoma (Pancreatic ductal variant)?

H and E?

Associated/exclude?

IHC?

A

Poorly differentiated with pushing boarders, syncytial growth, and lots of lymphocytes

Lynch associated; also sporatic

Keratin +, MMR loss in many; EXCLUDED ACINIC CELL

29
Q

Pancreatic ductal variant with large cells?

H and E?

IHC?

A

Undifferentiated carcinoma with Osteoclast like giant cells, Rare older patients, poor prognosis

Highly pleomorphic cells and large non-neoplastic giant cells

CK7+, Vimentin +, CD68+ giant cells

30
Q

Pancreatic ductal adenocarcinoma genes?

AJCC staging based on?

A

KRAS 60-90%

TP53

CDKN2A (p16)

SMAD4/DPC4 ~60%

SIZE: pT3>4cm!; extending outside pancreas is not T3 but T4

31
Q

Common causes of chronic pancreatitis?

Gross?

H and E?

A

Alcohol (70%); smoking 10x risk

Changes are irreversible

Gross: Focal or diffuse with indurated and fibrosis and obstruction of ducts with Calcs

H and E: Lobular configuration is maintained even if acinar are lost!

32
Q

Autoimmune pancreatitis?

Diagnosis?

Tx with steroids

A

Mass forming lesion; Prominent periductal lymphoplasmacytic infiltrate, periductal sclerosis, storiform fibrosis, and obliterative phlebitis

Full organ involvement

IgG4>135 mg/DL

>10 IgG4 plasma cells/HPF

IgG4:IgG.–>at least 40% on slides

33
Q

Hereditary pancreatitis family pattern?

Genes?

Risk?

A 
AD
PRSS1 (cationic trypsin gene) SPINK1 (serine peptidase inhibitor, Kazal type 1

50x risk of pancreatic adenocarcinoma; 40% lifetime

34
Q

Acinar cell carcinoma?

H and E?

Secretes?

Gross?

A

Elderly pancreatic carcinoma; early mets; poor survival (liver and LN’s)

Dense tumor with (acinar, glandular, trabecular); granular cytoplasm, prominent nucleoli, PAS-D+, Trypsin +, Chymotrypsin +, Focal Snapto and Chromo, CK8+, CK18+, Some Amlylase/CK7/CK20/aFP+

Secretes: Lipase: Subq fat necrosis, poly arthralgia

Solid, well circumscribed and fleshy

35
Q

Who gets solid pseudopapillary neoplasm?

Genes?

Histology

A

Young women; large mass but rare mets; solid and cystic areas

Missense exon 3 CTNNB1; prevents B-Catenin degredation

Solid, monomorphic sheet polygonal cells with fine chromatin and possible roseets, PASD+ globules and blood lakes, CD10+ (perinucler), Nuclear B-catenin

36
Q
A
37
Q

More common secreting pancreatic NE tumor?

Gastrinoma associated with?

A

Insulinoma > Glucogonoma

ZE syndrome and MEN1

38
Q

Pancreatoblastoma?

H and E?

Lab?

Genes?

A

Maligant from multiple lines of differentiation; rare but seen in kids; liver mets w/ poor prognosis

Uniform small cells with ducts and acinie and endocrine differentiation; Squamous corpuscles ARE DIAGNOSTIC (B-cat+, cyclin D1+)

AFP increased

APC/B-catinin: LOH Chr 11p15.5, Abnormal SMAD4/DPC4 and Isolated Bechwith-Wiedmann FAP

39
Q
A
40
Q

Ducts dilated with eosinophlic material, think?

Gene?

Random ?: Pancreatic pseudocyst cytology?

Solid pseudopapillary neoplasm cyto?

Acinar cytology?

A

Cystic fibrosis

CFTR Chr7q3.1 delta F508

Has macrophages/histeocytes in grungy background; high amylase (Also IPMN) and lipase

SPN: Globules and papillae

Acinic: ugly nuclei with nucleoli

41
Q
A

GI (4 decks)

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