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ADV PATH > 1M-LIVER PANC BILIARY > Flashcards

1M-LIVER PANC BILIARY Flashcards

1
Q

As a way to protect itself, the liver transforms the liver parenchyma into regenerative nodules

A

NODULAR REGENERATIVE HYPERPLASIA (NRH)

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2
Q

Distinguishing features of NRH from Liver Cirrhosis

A
  • Absent fibrous septa
  • Minimal inflammation
  • Liver parenchyma softer (d/t lack of fibrosis)
  • Nodules are less well defined
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3
Q

Cause of NRH

A

Non-cirrhotic portal hypertension

Others:
- autoimmune diseases
- common variable immunodeficiency
- hematological disorders
-drugs: azathioprine, didanosine, and
oxaliplatin
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4
Q

Rare entity in which the nodules may be larger than in NRH and are often restricted to the perihilar region, mimicking a neoplasm

A

Partial nodular transformation

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5
Q

Grossly, this presents as a subcapsular, gray-white, solid mass that is sometimes pedunculated.

On cut section, a white depressed area of fibrosis is often seen in the center, with broad strands radiating from it to the periphery in a stellate configuration

A

FOCAL NODULAR HYPERPLASIA (FNH)

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6
Q

FNH: T or F

  • MC in 3rd-5th decade
  • MC in women
  • Most cases are symptomatic
  • Usually multicentric
A

FALSE

  • MC in 3rd-5th decade
  • MC in women
  • > 80% of cases are ASYMPTOMATIC
  • > Usually SOLITARY
    * Multicentricity is higher in pediatric cases; assoc with vascular lesions
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7
Q

Pathogenesis of FNH

A

Unknown

Possible causes:

  • Relationship with oral contraceptives
  • Vascular anomalies or hyperplastic/regenerative response to localized insult in liver parenchyma
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8
Q

Characteristic finding of FNH

A

Central stellate scar

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9
Q

Microscopic features of FNH

A

-Nodular appearance with a central stellate scar
-Large, thick walled blood vessels present in the fibrous bands
-Patchy ductular reaction (composed of
lymphocytes and plasma cells) at the
junction of fibrous septa
-Map-like reactivity

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10
Q

Map-like reactivity of FNH

A
  • with glutamine synthetase

- to differentiate FNH from hepatocellular adenoma (liver cell adenoma)

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11
Q

FNH differential diagnosis

A
  • Hepatocellular adenoma
  • Well-differentiated hepatocellular carcinoma
  • Cirrhosis
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12
Q

What stain is used in diagnosing FNH to differentiate benign from malignant liver

A

Reticulin stain

*Benign liver: hepatic trabeculae less than 3-cell thick

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13
Q
  • MC in 3rd to 5th decade
  • MC in women
  • Most are symptomatic
  • Linked to oral contraceptive use
A

HEPATOCELLULAR ADENOMA

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14
Q

Usual symptom presenting in pxs with HCA

A

Fatal intraperitoneal hemorrhage

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15
Q

T or F: HCA can regress

A

TRUE

*Because of its link with contraceptives use, they can regress if contraceptives are discontinued.

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16
Q

Solitary lesion in non-cirrhotic livers, usually in the right lobe

A

HEPATOCELLULAR ADENOMA

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17
Q

HCA assoc with steatosis & insulin resistance

IHC: Absence of LFABP

Absent inflammation and nuclear atypia

A

Hepatocyte nuclear factor 1α (HNF1α) mutations

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18
Q

HCA that is most highly correlated with malignant transformation

A

Catenin beta-1 (CTNNB1) mutation

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19
Q

HCA assoc with male gender, androgen use, and glycogenesis

There is patchy nuclear staining

Mild atypia with focal rosette formation; no inflammation and steatosis

A

Catenin beta-1 (CTNNB1) mutation

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20
Q

Most common type of HCA

A

Inflammatory or telangiectatic adenoma

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21
Q

HCA assoc with male gender & increased BMI; presents with fever

Has sinusoidal dilatation and dystrophic vessels filled with RBCs and inflammatory cells

Normal LFABP

A

Inflammatory or telangiectatic adenoma

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22
Q

o Cirrhotic liver nodules greater than 1.0cm
o Normal architecture and intact reticulin
o Probably not pre-malignant

A

Regenerative nodules (low-grade dysplastic nodules)

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23
Q

Nodules in cirrhotic px with both architectural and cytologic atypia

(Hepatocytes are larger with slight increase in nucleus-cytoplasm ratio)

A

High grade dysplastic nodules

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24
Q

Difference between the two types of liver cell dysplasia

A

SMALL CELL CHANGE

  • More ominous -> premalignant
  • Increased N:C ratio -> dec. cytoplasm, mod. enlarged nucleus

LARGE CELL CHANGE

  • Preserved N:C ratio -> both nuclear and cytoplasmic enlargement
  • Nuclear pleomorphism, hyperchromasia, multinucleation
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25
Q

Most common primary malignant neoplasm of the liver

A

HEPATOCELLULAR CARCINOMA

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26
Q

Predisposing factors for HCC

A 
o Hepatotropic viruses (HBV and HCV)
   - Pxs w/ HBV who are male, African-american, aflatoxin exposure, alcohol use
   - Pxs w/ HBV coinfected w/ HCV
   - Higher levels of HBV DNA
   - Positivity to HBE antigen
   - Infected w/ HBV longer
o Alcohol use
o Non-alcoholic fatty liver disease
o Thorium dioxide (Thorotrast)
o Aspergillus flavus (alfatoxin B1)
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27
Q

T or F: Hep B positive patients will develop HCC

A

FASLE

*Not all, only 30%

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28
Q

Microscopic patterns of HCC

A

Pseudoglandular - has bile pigments; some areas retain polygonal shape of hepatocytes

Trabecular - forms cords or bands of neoplastic cells

Solid - solid sheets of tumor

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29
Q

Most common pattern of growth of HCC

A

Trabecular

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30
Q

Characteristics to diagnose well-differentiated HCC

A
  • Unpaired arteries
  • Absence of portal triads
  • Endothelial wrapping with bile pigments seen in cytologic specimens
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31
Q

HCC seen in young patients without cirrhosis

Has favorable prognosis

  • nests and cords of neoplastic cells surrounded by lamellar bands of fibrosis
  • deeply eosinophilic abundant cytoplasm & prominent nucleoli
A

FIBROLAMELLAR VARIANT OF HCC

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32
Q

HCC grading

A

“Edmondson and Steinier”
o G1: well-differentiated
o G2: moderately-differentiated
o G3: poorly- differentiated

  • Grading is reserved for resected specimen
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33
Q

Portal venous system involvement of HCC

A

80%

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34
Q

Location of metastases in HCC

A

Lungs
Abdominal lymph node
Bone

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35
Q

Treatment for HCC

A
  • Resection: first line therapy
  • Liver transplantation
  • Ablative therapy: single small tumors
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36
Q

Criteria for liver transplantation in treating patients with HCC

A

Milan criteria

  • Single tumor <5cm
  • 2-3 nodules all <3cm
  • Without gross vascular invasion or hepatic spread
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37
Q

Most common pediatric liver tumor

A

HEPATOBLASTOMA

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38
Q

MC age of presentation of Hepatoblastoma

A

First 3 years of life

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39
Q

Hepatic tumor characteristics:

  • majority are sporadic
  • elevated serum AFP
  • 67% are pure/epithelial type
  • extramedullary hematopoiesis
A

HEPATOBLASTOMA

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40
Q

What conditions is hepatoblastoma associated with?

A

Beckwith-wiedemann syndrome, trisomy 18, familial colonic polyposis

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41
Q

Which variant of Hepatoblastoma?

Hepatocytes arranged in irregular laminae two cells thick; cells are smaller, more hyperchromatic, with a clear cytoplasm

A

FETAL VARIANT

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42
Q

Which variant of Hepatoblastoma?

More immature and predominantly solid pattern, but also ribbons, rosettes and papillary formation; vesicular nuclei that are larger and nuclear molding

A

EMBRYONAL VARIANT

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43
Q

Hepatoblastoma that may consist of spindled cells, osteoid, skeletal muscle, or cartilage; teratoid features

A

MIXED EMBRYONAL-MESENCHYMAL

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44
Q

Hepatoblastoma types that are distinguished by low levels of AFP. Have poor prognosis

A

CHOLANGIOBLASTIC AND MACROTRABECULAR

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45
Q

Hepatoblastoma which contains a tubular component with cholangioblastic features

A

CHOLANGIOBLASTIC TYPE

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46
Q

Hepatoblastoma type that can mimic HCC

A

MACROTRABECULAR TYPE

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47
Q

Clinical features:

  • Most occur after 60 years
  • Has male predilection
  • Often asymptomatic
A

INTRAHEPATIC CHOLANGIOCARCINOMA

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48
Q

Genetic mutation involved in IHCC

A

KRAS mutation

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49
Q

Most IHC cases are asymptomatic but…

Intrahepatic vs Extrahepatic CC Sxs when symptomatic

A

INTRAHEPATIC CHOLANGIOCARCINOMA
-Abdominal pain & weight loss

EXTRAHEPATIC CHOLANGIOCARCINOMA
-Jaundice

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50
Q

IHCC risk factors

A

o Fibropolycystic liver disease
o Chronic inflammatory conditions (primary
sclerosing cholangitis and Opisthorhis and
Clonorchis infection)
o Hepatolithiasis
o Chronic liver disease

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51
Q

Microscopic characteristics:

  • Tubular glands that appear normal with prominent reactive, sclerotic, fibrous, desmoplastic stroma
  • On a closer look, there is heterogeneity of neoplastic cells. There is variation in nuclear size and irregularity of nuclear membranes
  • There is perineural invasion
A

INTRAHEPATIC CHOLANGIOCARCINOMA

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52
Q

Most common benign tumor of the liver

A

HEMANGIOMA

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53
Q

A vascular tumor of the liver that may rupture and lead to spontaneous bleeding

A

HEMANGIOMA

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54
Q

Microscopic characteristics:

Dilated vascular spaces lined by flat endothelial cells filled with RBCs within dense fibrous stroma

A

HEMANGIOMA

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55
Q

Early vs Late stage Hemangioma

A

EARLY STAGE:
- Typical spongy deep red cut surface in
hemangioma of the liver.

LATER STAGE:
- Tumor accompanied by large fibrin deposits secondary to thrombosis.

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56
Q

Almost exclusively occurs in children; sometimes in neonates

A

BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA

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57
Q
  • Highly cellular variant of hemangioma that typically appears in a lobular pattern
  • The dilated BV are smaller, lined by one or more layers of plump endothelial cells, and are surrounded by prominent perithelial cells
  • Elevated serum AFP
  • Some may regress
A

BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA

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58
Q

Conditions assoc with BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA

A

o Beckwith-Wiedemann syndrome
o Hepatic failure
o CHF
o Hyperconsumptive coagulopathy (Kasabach-Meritt syndrome)

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59
Q

IHC of BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA

A

GLUT-1 (+)

60
Q

Hepatic vascular tumor that occurs predominantly in adult, females, and those associated with OCP use

A

EPITHELOID HEMANGIOENDOTHELIOMA

61
Q

This tumor has a multifocal growth pattern and may stimulate Budd-Chiari syndrome when there is involvement of the hepatic veins

A

EPITHELOID HEMANGIOENDOTHELIOMA

62
Q

Microscopic characteristics:

-Epithelioid tumor cells with occasional intracytoplasmic lumens within abundant stroma

A

EPITHELOID HEMANGIOENDOTHELIOMA

63
Q

T or F:

Extrahepatic metastases could occur in EHE making the prognosis poorer than angiosarcoma

A

FALSE

*Extrahepatic metastases (27%) but the prognosis is
MORE FAVORABLE than angiosarcoma

64
Q
  • Malignant endothelial cells infiltrating into vascular structures
  • Most common in adults
  • Has extremely poor prognosis
A

ANGIOSARCOMA

65
Q

Cut surface:
- Deep tan red surface with areas of necrosis and hemorrhage

Microscopic:

  • Malignant atypical endothelial cells replace the liver parenchyma; spindle shaped or even epithelioid looking cells
  • Sinusoids are dilated and are anastomosing; presence of intracytoplasmic RBCs
A

ANGIOSARCOMA

66
Q

Risk factors of ANGIOSARCOMA

A

o Cirrhosis
o Vinyl Chloride
o Thorium dioxide
o Arsenic

67
Q

T or F: Liver is a repository for metastatic tumor

A

TRUE

68
Q

Metastatic tumor through direct extension

A

gallbladder, extrahepatic bile ducts, pancreas and stomach

69
Q

Liver metastasis locations

A

Large bowel, lung, breast, pancreas, kidney, stomach and others

70
Q

Most common tumor of the gallbladder

A

ADENOCARCINOMA

71
Q

Risk factors associated with GB AC

A
  • Female
  • > 50 y/o
  • Latin American, Asian
  • Porcelain GB, PSC, obesity
72
Q

Most common abnormal lab finding in GB AC

A

Increased alkaline phosphatase

73
Q

Most common location of GB AC

A

70% arise from fundus/ distal portion of GB

74
Q

Gross:

  • Diffusely growing or nodular, polypoid, or papillary mass
  • Sometimes, it may not be grossly apparent. But a thickened mucosa is seen.

Micro:

  • Cells are dysplastic, hyperchromatic, there is loss of polarity
  • Dysplastic cells are having a pseudostratified arrangement within the glands
  • Glands lie parallel to the muscularis propia
  • There is heterogeneity of neoplastic cells (normal looking cells with atypical cells)
A

GB ADENOCARCINOMA

75
Q

Most common pattern for adenocarcinoma of the gallbladder

-> Infiltrative tubular glands with widened lumen, surrounded by desmoplastic stroma

A

Pancreatobiliary type gallbladder adenoCA

76
Q

Metastatic locations of Gallbladder AdenoCA

A
  • Direct invasion to liver

* Others: stomach, duodenum

77
Q

Lymph node involvement in Gallbladder AdenoCA

A

o Cystic and pericholedochal lymph nodes in the
lesser omentum
o Lymph nodes behind first portion of duodenum

• Lymph node involvement is highly dependent on depth of invasion of primary tumor

78
Q

Tumor staging & treatment of choice in Gallbladder AdenoCA

A
  • T1a (limited to lamina propria): Simple cholecystectomy with negative margin
  • T1b (invasion of muscular wall): tx same as T2 but still controversial
  • T2 (involving perimuscular connective tissue): Radical resection with lymph node dissection
  • T3 (penetrates visceral peritoneum or invades adjacent structure): Radical surgery depending on patient’s fitness
  • T4 (presence of metastasis): Palliative
79
Q

Most common tumor of the extrahepatic bile ducts

A

Adenocarcinoma/ Extrahepatic cholangiocarcinoma

80
Q
  • Occurs with equal frequency in males and females
  • Ave age is 60 y/o
  • Most patients are symptomatic
A

Adenocarcinoma/ Extrahepatic cholangiocarcinoma

81
Q

Presenting symptom in patients with Extrahepatic cholangiocarcinoma/ BD AdenoCA

A

Jaundice

82
Q

Risk factors assoc w/ BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma

A

○ Primary sclerosing cholangitis
○ Clonorchis sinensis infestation
○ Congenital abnormalities of intra- and extrahepatic bile duct (i.e. choledochal cysts, Caroli disease, congenital hepatic fibrosis)

83
Q

BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma locations

A

Can develop at any level of the biliary tree

○ Upper third, including the hilum (50%–75%); Most common
○ Middle third (upper half of the common bile duct, 10%–25%)
○ Lower third (distal half of the CBD, 10%–20%)

84
Q

Most common BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma tumor & it’s location

A

Klatskin tumor/ Hilar Cholangiocarcinoma

- located at hilum

85
Q

Gross & microscopic characteristic of BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma

A

GROSS

  • Nodular tumor w/ deep penetration into the wall
  • Presence of intraductal / intrahepatic spread (**in hilar CC only???)

MICRO

  • Desmoplastic stroma
  • Heterogeneity of cells (with increased NC ratio, nucleolar prominence, loss of nuclear polarity)
  • Perinueral invaion
86
Q

Metastatic locations of BD AdencoCA/ EHCC

A
  • Direct extension: liver (in Upper third/hilar tumors)
  • Distal lesions: pancreas
  • Regional/ peripancreatic nodes:
    ○ Lower portion of the hepatoduodenal ligament
    ○ Superoposterior pancreaticoduodenal group
    ○ Superior mesenteric artery group
87
Q

Treatment of BD AdencoCA/ EHCC

A

○ Surgical: primary mode of tx
• Proximal lesion: Resection (Hepatic lobectomy) and Roux-en-Y hepaticojejunostomy
• Distal lesion: Whipple procedure
○ Liver transplant

88
Q

T or F: Distal lesions of BD AdencoCA/ EHCC have worse prognosis

A

FALSE

• PROXIMAL carcinomas have WORSE prognosis
o Not amenable to surgical treatments

89
Q

Five groups of BD AdencoCA/ EHCC for staging purposes

A 
o intrahepatic
o perihilar
o cystic duct,
o distal bile duct/ Supplying the pancreas
o intraampullary common bile duct
90
Q

Most common type of pancreatic cyst

A

PSEUDOCYST

91
Q

Pancreatic cystic lesion characteristics:

  • No true lining; only has fibrous stroma with moderate inflammation consisting of lymphocytes and plasma cells
  • Related to: acute or chronic pancreatitis, trauma, or neoplastic obstruction
  • Can become very large (3-20 cm)
A

PSEUDOCYST

92
Q

Complications of pancreatic pseudocyst

A

○ Perforation
○ Infection
○ Hemorrhage

93
Q

Most common source of hemorrhage in pancreatic pseudocyst

A

Splenic artery

94
Q

Preferred treatment in cases of pancreatic pseudocyst

A

Endoscopic drainage

- except: if located in tail of pancreas (w/ splenic involvement)

95
Q

Preferred treatment in cases of pancreatic pseudocyst located in the tail

A

Surgical management

96
Q

Pancreatic cystic lesion characteristics:

  • Single later of flat, nonmucinous epithelial lining
  • Has no communication with the ductal system
A

CONGENITAL CYST

97
Q

Pancreatic Congenital Cyst is assoc with what diseases

A
  • VHL disease
  • Fibropolycystic kidney/liver disease
  • Oral-facial-digital syndrome
98
Q

Composes 90% of tumors of the exocrine pancreas

A

PANCREATIC DUCTAL ADENOCA

99
Q

Risk factors in Pancreatic Ductal AdenoCA

A
  • Slight predilection in males
  • Elderly
  • Smoking
100
Q

Clinical features of Pancreatic Ductal AdenoCA

A

○ Vague abdominal pain
○ Progressive jaundice (head of pancreas)
○ Relatively large (5 cm) and extended beyond pancreas
○ Unique feature: association with DVT (Thrombophlebitis migrans/ Trosseau
syndrome)

101
Q

Reason behind presence of DVT in Pancreatic Ductal AdenoCA

A

Due to the:
■ Release of TNF, IL-1, and IL-6 by
macrophages in tumor stroma
■ Tumor cells release procoagulant

102
Q

Conditions associated with Inherited Pancreatic AdenoCA together with their mutations

A
  1. Hereditary breast and ovarian cancer syndrome (BRCA2)
  2. Familial atypical multiple mole melanoma syndrome (p16)
  3. Peutz-Jeghers syndrome (STK11/LKB1 gene)
  4. Hereditary nonpolyposis colorectal cancer (DNA mismatch repair)
  5. Hereditary pancreatitis (PRSS1 gene)
103
Q

Most common location of Pancreatic AdenoCA

A

Head of pancreas

104
Q

Diagnostic features in Pancreatic AdenoCA

A
  • Glands present outside the normal lobular architecture
  • Presence of glands immediately adjacent to muscular arteries
  • Necrotic debris within gland lumens
  • Perineural invasion
105
Q

IHC and molecular features of Pancreatic AdenoCA

A

IHC: Express cell-surface associated mucins - MUC1
(*also MUC3, 4, 5AC; vs. colonic adca: MUC2)

MOLECULAR: KRAS mutation

106
Q

Mutations present in Genetic Progression of Pancreatic Carcinogenesis

A

EARLY MUTATION

  • Telomere shortening (normal -> PanIN1)
  • KRAS2 mutation (PanIN1)

INTERMEDIATE
- P16 loss (PanIN2)

LATE
- Mutations of DPC4, TP53, BRCA2 (PanIN3)

107
Q

Metastatic locations of Pancreatic AdenoCA

A
  • Direct invasion: duodenum (ampulla), CBD

- Distant metastasis: liver, peritoneum, lung, adrenal, bone, distant lymph nodes, skin and CNS

108
Q

Pancreatic cystic neoplasm characteristics:

  • Have micronodules that have variable sizes and spongy consistency
  • Can be unilocular with solid areas
  • Epithelium lining composed of uniform cells with minimal intervening stroma; cystic glands are various sized.
A

Pancreatic serous cystadenoma

-aka: Microcystic adenoma, glycogen-rich cystadenoma

109
Q

Clinical features of Pancreatic serous cystadenoma (Microcystic adenoma/ Glycogen-rich cystadenoma)

A

○ Women and elderly patients
○ Incidental finding or symptomatic
○ Some occur with the VHL disease
○ Diabetes may occur if sufficient islet cells are destroyed
○ May cause GI or biliary obstruction if located in the head of pancreas

110
Q

Symptom presenting in patients with Pancreatic serous cystadenoma (Microcystic adenoma/ Glycogen-rich cystadenoma)

A

Abdominal mass with local discomfort

111
Q

Treatment for Pancreatic serous cystadenoma (Microcystic adenoma/ Glycogen-rich cystadenoma)

A

Excision is curative

112
Q

Pancreatic cystic neoplasm characteristics:

  • Younger (late 40s); women
  • Can be unilocular or mulitlocular
  • Typically large (> 10 cm)
  • Produces mucin
  • Lining epithelium: mucin-type, tall columnar with basally oriented nuclei
  • Ovarian-type stroma
A

Pancreatic Mucinous Cystic Neoplasm

113
Q

Moust common location of Pancreatic Mucinous Cystic Neoplasm

A

Body or tail

114
Q

IHC: Pancreatic Mucinous Cystic Neoplasm

A

Progesterone (+)

Inhibin (+)

115
Q

T or F: Pancreatic Mucinous Cystic Neoplasm may be assoc with invasive carcinoma

A

TRUE

*clue is desmoplastic stromal response

116
Q
  • Formation of papillary epithelial projections
  • Intraluminal mucin production
  • Intraductal involvement
A

Pancreatic Intraductal Mucinous Neoplasm

117
Q

Most common location of Pancreatic Intraductal Mucinous Neoplasm

A

Head

118
Q

T or F: Pancreatic Intraductal Mucinous Neoplasm may be assoc with invasive carcinoma

A

TRUE

119
Q

Incidence of invasion in Pancreatic Intraductal Mucinous Neoplasm

A
  • Main duct: 30-35%

- Branch duct: 15%

120
Q

Two microscopic types of invasion in Pancreatic Intraductal Mucinous Neoplasm

A

■ Intestinal-type IPMN: colloid type

■ Pancreaticobillary type-IPMN: tubular type

121
Q
  • Occurs in adults
  • Gross: Variegated; solid with degenerated cystic area with necrosis
  • Micro: Uniform, monotonous, hyperchromatic with small, sometimes prominent nuclei with scant cytoplasm
A

Acinar Cell Carcinoma

122
Q

Stains for Acinar Cell Carcinoma

A
  • PAS (+), Diastase resistant

- Immunoreactive for Trypsin, Chymotrypsin, Lipase, Amylase

123
Q

T or F: Acinar Cell Carcinoma prognosis is poor especially since metastases are present at the time of diagnosis in 50% of cases

A

TRUE

124
Q

Most common site of metastasis in Acinar Cell Carcinoma

A
  • Regional LN

- Liver

125
Q

Most common pancreatic neoplasia in childhood

A

Pancreatoblastoma

126
Q
  • SLight male predominance
  • Partial encapsulation
  • Large nests of neoplastic cells
  • Presence of squamoid islands or corpuscles with optically clear nuclei
A

Pancreatoblastoma

127
Q
  • Young women
  • Presents with abdominal pain
  • Gross: Tan red tumor; solid, papillary
  • Micro: Papillary structures, fibrovascular core and cholesterol clefts; discohesive cells
A

Solid Pseudopapillary Tumor (SPPT/SPEN)

128
Q

Treatment and prognosis of SPPT/SPEN

A
  • Surgery is curative
  • Rarely recurs/metastasizes
  • Location of metastases: liver, peritoneum
  • Considered as neoplasm of low malignant potential
129
Q
  • MC in adults
  • Solitary or multifocal
  • Typically encapsulated
  • Recurs in 5%
  • Micro: Small size neoplastic cells, inconspicuous nuclei, scant cytoplasm
  • Stippled chromatin (salt & pepper)
A

Pancreatic Neuroendocrine Tumor (PanNETs)

130
Q

Patterns of Pancreatic Neuroendocrine Tumor (PanNETs)

A
  • Solid
  • Trabecular
  • Pseudoglandular
131
Q

Incidence of PanNETs (benign vs malignant)

A
  • 90% of insulin- producing tumors are BENIGN

* 60-90% of other functioning and nonfunctioning tumors are MALIGNANT

132
Q

Citeria of malignancy in PanNETs

A

○ Metastases
○ Vascular invasion
○ Local Infiltration

133
Q

Associated syndromes in Pancreatic Neuroendocrine Tumor (PanNETs)

A
  • MEN1
  • VHL Disease
  • Neurofibromatosis 1
  • Tuberous Sclerosis
134
Q

Most common PanNETs

A

Insulinoma (B-cell tumor)

135
Q

Triad present in Insulinoma (B-cell tumor)

A

WHIPPLE TRIAD
○ Mental confusion, fatigue, weakness and convulsions
○ Clinically significant hypoglycemia (<50 mg/dl)
○ Relief of symptoms by administration of glucose

136
Q

Characteristic feature of Insulinoma (B-cell tumor)

A

Deposition of amyloid

137
Q

Which pNET:
• Monotonous neoplastic cell, minimal pleomorphism or mitotic activity
• Abundant Amyloid deposition (insulinoma)
• Clinically: episodic hypoglycemia

A

INSULINOMA (B-CELL TUMOR)

or

PANCREATIC ENDOCRINE NEOPLASM –
(ISLET CELL TUMOR)

138
Q

Which pNET:
• 2nd most common pNET
• Marked hypersecretion of gastrin causing severe peptic ulceration
• More than half are invasive or already metastasized
• 25% arise as part of the MEN-1 syndrome

A

GASTRINOMA/ ZOLLINGER-ELLISON SYNDROME

139
Q

Most common location of Gastrinoma/ ZES

A

Duodenum and peripancreatic soft

tissues

140
Q

pNET Grading

A

WELL-DIFF NENs (mitosis/10 HPF ; Ki-67 index)

  • NET grade 1: <2 ; <3
  • NET 2 : 2-20 ; 3-20
  • NET 3 : >20 ; >20

POORLY DIFF
- NEC grade 3: >20 ; >20

141
Q

NEC: small cell vs large cell

A
  • Small cell NEC: Incr. nuclei cytoplasmic ratio (small cell but scant neoplasm); grow close together, darkly stained nuclei
  • Large cell NEC: Nuclei are vesicular, more abundant neoplasm
142
Q

Which pNET; what hormone?

• Sx: weakness, sweating, tremors, palpitations, confusion, visual changes

A
  • Sx is Hypolycemia
  • Hormone: Insulin
  • Dx: Insulinoma
143
Q

Which pNET; what hormone?

• Abdominal pain, refractory peptic ulcer disease, secretory diarrhea

A
  • Hormone: Gastrin

* Dx: Gastrinoma/ ZES

144
Q

Which pNET; what hormone?

• Dermatitis, diabetes mellitus, diarrhea, DVT

A
  • Sx 4Ds syndrome
  • Hormone: Glucagon
  • Dx: Glucagonoma
145
Q

Which pNET; what hormone?

• Profuse watery diarrhea, dehydration, hypokalemia, achlorhydria

A
  • Sx: WDHA syndrome
  • Hormone: VIP
  • Dx: VIPoma (Verner-Morrison)
146
Q

Which pNET; what hormone?

• Diabetes mellitus, cholelithiasis, steatorrhea, anemia, weight loss

A
  • Hormone: Somatostatin

* Dx: Somatostatinoma

ADV PATH (10 decks)

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