1M-LIVER PANC BILIARY Flashcards
(146 cards)
1
Q
As a way to protect itself, the liver transforms the liver parenchyma into regenerative nodules
A
NODULAR REGENERATIVE HYPERPLASIA (NRH)
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
3
Q
Cause of NRH
A
Non-cirrhotic portal hypertension
Others: - autoimmune diseases - common variable immunodeficiency - hematological disorders -drugs: azathioprine, didanosine, and oxaliplatin
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
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)
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
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
8
Q
Characteristic finding of FNH
A
Central stellate scar
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
10
Q
Map-like reactivity of FNH
A
- with glutamine synthetase
- to differentiate FNH from hepatocellular adenoma (liver cell adenoma)
11
Q
FNH differential diagnosis
A
- Hepatocellular adenoma
- Well-differentiated hepatocellular carcinoma
- Cirrhosis
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
13
Q
- MC in 3rd to 5th decade
- MC in women
- Most are symptomatic
- Linked to oral contraceptive use
A
HEPATOCELLULAR ADENOMA
14
Q
Usual symptom presenting in pxs with HCA
A
Fatal intraperitoneal hemorrhage
15
Q
T or F: HCA can regress
A
TRUE
*Because of its link with contraceptives use, they can regress if contraceptives are discontinued.
16
Q
Solitary lesion in non-cirrhotic livers, usually in the right lobe
A
HEPATOCELLULAR ADENOMA
17
Q
HCA assoc with steatosis & insulin resistance
IHC: Absence of LFABP
Absent inflammation and nuclear atypia
A
Hepatocyte nuclear factor 1α (HNF1α) mutations
18
Q
HCA that is most highly correlated with malignant transformation
A
Catenin beta-1 (CTNNB1) mutation
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
20
Q
Most common type of HCA
A
Inflammatory or telangiectatic adenoma
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
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)
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
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
25
Most common primary malignant neoplasm of the liver
HEPATOCELLULAR CARCINOMA
26
Predisposing factors for HCC
```
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)
```
27
T or F: Hep B positive patients will develop HCC
FASLE
*Not all, only 30%
28
Microscopic patterns of HCC
Pseudoglandular - has bile pigments; some areas retain polygonal shape of hepatocytes
Trabecular - forms cords or bands of neoplastic cells
Solid - solid sheets of tumor
29
Most common pattern of growth of HCC
Trabecular
30
Characteristics to diagnose well-differentiated HCC
- Unpaired arteries
- Absence of portal triads
- Endothelial wrapping with bile pigments seen in cytologic specimens
31
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
FIBROLAMELLAR VARIANT OF HCC
32
HCC grading
"Edmondson and Steinier"
o G1: well-differentiated
o G2: moderately-differentiated
o G3: poorly- differentiated
- Grading is reserved for resected specimen
33
Portal venous system involvement of HCC
80%
34
Location of metastases in HCC
Lungs
Abdominal lymph node
Bone
35
Treatment for HCC
- Resection: first line therapy
- Liver transplantation
- Ablative therapy: single small tumors
36
Criteria for liver transplantation in treating patients with HCC
Milan criteria
- Single tumor <5cm
- 2-3 nodules all <3cm
- Without gross vascular invasion or hepatic spread
37
Most common pediatric liver tumor
HEPATOBLASTOMA
38
MC age of presentation of Hepatoblastoma
First 3 years of life
39
Hepatic tumor characteristics:
- majority are sporadic
- elevated serum AFP
- 67% are pure/epithelial type
- extramedullary hematopoiesis
HEPATOBLASTOMA
40
What conditions is hepatoblastoma associated with?
Beckwith-wiedemann syndrome, trisomy 18, familial colonic polyposis
41
Which variant of Hepatoblastoma?
Hepatocytes arranged in irregular laminae two cells thick; cells are smaller, more hyperchromatic, with a clear cytoplasm
FETAL VARIANT
42
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
EMBRYONAL VARIANT
43
Hepatoblastoma that may consist of spindled cells, osteoid, skeletal muscle, or cartilage; teratoid features
MIXED EMBRYONAL-MESENCHYMAL
44
Hepatoblastoma types that are distinguished by low levels of AFP. Have poor prognosis
CHOLANGIOBLASTIC AND MACROTRABECULAR
45
Hepatoblastoma which contains a tubular component with cholangioblastic features
CHOLANGIOBLASTIC TYPE
46
Hepatoblastoma type that can mimic HCC
MACROTRABECULAR TYPE
47
Clinical features:
- Most occur after 60 years
- Has male predilection
- Often asymptomatic
INTRAHEPATIC CHOLANGIOCARCINOMA
48
Genetic mutation involved in IHCC
KRAS mutation
49
Most IHC cases are asymptomatic but...
Intrahepatic vs Extrahepatic CC Sxs when symptomatic
INTRAHEPATIC CHOLANGIOCARCINOMA
-Abdominal pain & weight loss
EXTRAHEPATIC CHOLANGIOCARCINOMA
-Jaundice
50
IHCC risk factors
o Fibropolycystic liver disease
o Chronic inflammatory conditions (primary
sclerosing cholangitis and Opisthorhis and
Clonorchis infection)
o Hepatolithiasis
o Chronic liver disease
51
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
INTRAHEPATIC CHOLANGIOCARCINOMA
52
Most common benign tumor of the liver
HEMANGIOMA
53
A vascular tumor of the liver that may rupture and lead to spontaneous bleeding
HEMANGIOMA
54
Microscopic characteristics:
Dilated vascular spaces lined by flat endothelial cells filled with RBCs within dense fibrous stroma
HEMANGIOMA
55
Early vs Late stage Hemangioma
EARLY STAGE:
- Typical spongy deep red cut surface in
hemangioma of the liver.
LATER STAGE:
- Tumor accompanied by large fibrin deposits secondary to thrombosis.
56
Almost exclusively occurs in children; sometimes in neonates
BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA
57
- 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
BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA
58
Conditions assoc with BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA
o Beckwith-Wiedemann syndrome
o Hepatic failure
o CHF
o Hyperconsumptive coagulopathy (Kasabach-Meritt syndrome)
59
IHC of BENIGN (INFANTILE) HEMANGIOENDOTHELIOMA
GLUT-1 (+)
60
Hepatic vascular tumor that occurs predominantly in adult, females, and those associated with OCP use
EPITHELOID HEMANGIOENDOTHELIOMA
61
This tumor has a multifocal growth pattern and may stimulate Budd-Chiari syndrome when there is involvement of the hepatic veins
EPITHELOID HEMANGIOENDOTHELIOMA
62
Microscopic characteristics:
| -Epithelioid tumor cells with occasional intracytoplasmic lumens within abundant stroma
EPITHELOID HEMANGIOENDOTHELIOMA
63
T or F:
Extrahepatic metastases could occur in EHE making the prognosis poorer than angiosarcoma
FALSE
*Extrahepatic metastases (27%) but the prognosis is
MORE FAVORABLE than angiosarcoma
64
- Malignant endothelial cells infiltrating into vascular structures
- Most common in adults
- Has extremely poor prognosis
ANGIOSARCOMA
65
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
ANGIOSARCOMA
66
Risk factors of ANGIOSARCOMA
o Cirrhosis
o Vinyl Chloride
o Thorium dioxide
o Arsenic
67
T or F: Liver is a repository for metastatic tumor
TRUE
68
Metastatic tumor through direct extension
gallbladder, extrahepatic bile ducts, pancreas and stomach
69
Liver metastasis locations
Large bowel, lung, breast, pancreas, kidney, stomach and others
70
Most common tumor of the gallbladder
ADENOCARCINOMA
71
Risk factors associated with GB AC
- Female
- >50 y/o
- Latin American, Asian
- Porcelain GB, PSC, obesity
72
Most common abnormal lab finding in GB AC
Increased alkaline phosphatase
73
Most common location of GB AC
70% arise from fundus/ distal portion of GB
74
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)
GB ADENOCARCINOMA
75
Most common pattern for adenocarcinoma of the gallbladder
-> Infiltrative tubular glands with widened lumen, surrounded by desmoplastic stroma
Pancreatobiliary type gallbladder adenoCA
76
Metastatic locations of Gallbladder AdenoCA
* Direct invasion to liver
| * Others: stomach, duodenum
77
Lymph node involvement in Gallbladder AdenoCA
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
Tumor staging & treatment of choice in Gallbladder AdenoCA
* 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
Most common tumor of the extrahepatic bile ducts
Adenocarcinoma/ Extrahepatic cholangiocarcinoma
80
- Occurs with equal frequency in males and females
- Ave age is 60 y/o
- Most patients are symptomatic
Adenocarcinoma/ Extrahepatic cholangiocarcinoma
81
Presenting symptom in patients with Extrahepatic cholangiocarcinoma/ BD AdenoCA
Jaundice
82
Risk factors assoc w/ BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma
○ Primary sclerosing cholangitis
○ Clonorchis sinensis infestation
○ Congenital abnormalities of intra- and extrahepatic bile duct (i.e. choledochal cysts, Caroli disease, congenital hepatic fibrosis)
83
BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma locations
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
Most common BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma tumor & it's location
Klatskin tumor/ Hilar Cholangiocarcinoma
| - located at hilum
85
Gross & microscopic characteristic of BD Adenocarcinoma/ Extrahepatic cholangiocarcinoma
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
Metastatic locations of BD AdencoCA/ EHCC
- 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
Treatment of BD AdencoCA/ EHCC
○ Surgical: primary mode of tx
• Proximal lesion: Resection (Hepatic lobectomy) and Roux-en-Y hepaticojejunostomy
• Distal lesion: Whipple procedure
○ Liver transplant
88
T or F: Distal lesions of BD AdencoCA/ EHCC have worse prognosis
FALSE
• PROXIMAL carcinomas have WORSE prognosis
o Not amenable to surgical treatments
89
Five groups of BD AdencoCA/ EHCC for staging purposes
```
o intrahepatic
o perihilar
o cystic duct,
o distal bile duct/ Supplying the pancreas
o intraampullary common bile duct
```
90
Most common type of pancreatic cyst
PSEUDOCYST
91
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)
PSEUDOCYST
92
Complications of pancreatic pseudocyst
○ Perforation
○ Infection
○ Hemorrhage
93
Most common source of hemorrhage in pancreatic pseudocyst
Splenic artery
94
Preferred treatment in cases of pancreatic pseudocyst
Endoscopic drainage
| - except: if located in tail of pancreas (w/ splenic involvement)
95
Preferred treatment in cases of pancreatic pseudocyst located in the tail
Surgical management
96
Pancreatic cystic lesion characteristics:
- Single later of flat, nonmucinous epithelial lining
- Has no communication with the ductal system
CONGENITAL CYST
97
Pancreatic Congenital Cyst is assoc with what diseases
- VHL disease
- Fibropolycystic kidney/liver disease
- Oral-facial-digital syndrome
98
Composes 90% of tumors of the exocrine pancreas
PANCREATIC DUCTAL ADENOCA
99
Risk factors in Pancreatic Ductal AdenoCA
- Slight predilection in males
- Elderly
- Smoking
100
Clinical features of Pancreatic Ductal AdenoCA
○ 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
Reason behind presence of DVT in Pancreatic Ductal AdenoCA
Due to the:
■ Release of TNF, IL-1, and IL-6 by
macrophages in tumor stroma
■ Tumor cells release procoagulant
102
Conditions associated with Inherited Pancreatic AdenoCA together with their mutations
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
Most common location of Pancreatic AdenoCA
Head of pancreas
104
Diagnostic features in Pancreatic AdenoCA
* Glands present outside the normal lobular architecture
* Presence of glands immediately adjacent to muscular arteries
* Necrotic debris within gland lumens
* Perineural invasion
105
IHC and molecular features of Pancreatic AdenoCA
IHC: Express cell-surface associated mucins - MUC1
(*also MUC3, 4, 5AC; vs. colonic adca: MUC2)
MOLECULAR: KRAS mutation
106
Mutations present in Genetic Progression of Pancreatic Carcinogenesis
EARLY MUTATION
- Telomere shortening (normal -> PanIN1)
- KRAS2 mutation (PanIN1)
INTERMEDIATE
- P16 loss (PanIN2)
LATE
- Mutations of DPC4, TP53, BRCA2 (PanIN3)
107
Metastatic locations of Pancreatic AdenoCA
- Direct invasion: duodenum (ampulla), CBD
| - Distant metastasis: liver, peritoneum, lung, adrenal, bone, distant lymph nodes, skin and CNS
108
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.
Pancreatic serous cystadenoma
| -aka: Microcystic adenoma, glycogen-rich cystadenoma
109
Clinical features of Pancreatic serous cystadenoma (Microcystic adenoma/ Glycogen-rich cystadenoma)
○ 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
Symptom presenting in patients with Pancreatic serous cystadenoma (Microcystic adenoma/ Glycogen-rich cystadenoma)
Abdominal mass with local discomfort
111
Treatment for Pancreatic serous cystadenoma (Microcystic adenoma/ Glycogen-rich cystadenoma)
Excision is curative
112
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
Pancreatic Mucinous Cystic Neoplasm
113
Moust common location of Pancreatic Mucinous Cystic Neoplasm
Body or tail
114
IHC: Pancreatic Mucinous Cystic Neoplasm
Progesterone (+)
| Inhibin (+)
115
T or F: Pancreatic Mucinous Cystic Neoplasm may be assoc with invasive carcinoma
TRUE
*clue is desmoplastic stromal response
116
- Formation of papillary epithelial projections
- Intraluminal mucin production
- Intraductal involvement
Pancreatic Intraductal Mucinous Neoplasm
117
Most common location of Pancreatic Intraductal Mucinous Neoplasm
Head
118
T or F: Pancreatic Intraductal Mucinous Neoplasm may be assoc with invasive carcinoma
TRUE
119
Incidence of invasion in Pancreatic Intraductal Mucinous Neoplasm
- Main duct: 30-35%
| - Branch duct: 15%
120
Two microscopic types of invasion in Pancreatic Intraductal Mucinous Neoplasm
■ Intestinal-type IPMN: colloid type
| ■ Pancreaticobillary type-IPMN: tubular type
121
- Occurs in adults
- Gross: Variegated; solid with degenerated cystic area with necrosis
- Micro: Uniform, monotonous, hyperchromatic with small, sometimes prominent nuclei with scant cytoplasm
Acinar Cell Carcinoma
122
Stains for Acinar Cell Carcinoma
- PAS (+), Diastase resistant
| - Immunoreactive for Trypsin, Chymotrypsin, Lipase, Amylase
123
T or F: Acinar Cell Carcinoma prognosis is poor especially since metastases are present at the time of diagnosis in 50% of cases
TRUE
124
Most common site of metastasis in Acinar Cell Carcinoma
- Regional LN
| - Liver
125
Most common pancreatic neoplasia in childhood
Pancreatoblastoma
126
- SLight male predominance
- Partial encapsulation
- Large nests of neoplastic cells
- Presence of squamoid islands or corpuscles with optically clear nuclei
Pancreatoblastoma
127
- Young women
- Presents with abdominal pain
- Gross: Tan red tumor; solid, papillary
- Micro: Papillary structures, fibrovascular core and cholesterol clefts; discohesive cells
Solid Pseudopapillary Tumor (SPPT/SPEN)
128
Treatment and prognosis of SPPT/SPEN
- Surgery is curative
- Rarely recurs/metastasizes
- Location of metastases: liver, peritoneum
- Considered as neoplasm of low malignant potential
129
- MC in adults
- Solitary or multifocal
- Typically encapsulated
- Recurs in 5%
- Micro: Small size neoplastic cells, inconspicuous nuclei, scant cytoplasm
- Stippled chromatin (salt & pepper)
Pancreatic Neuroendocrine Tumor (PanNETs)
130
Patterns of Pancreatic Neuroendocrine Tumor (PanNETs)
- Solid
- Trabecular
- Pseudoglandular
131
Incidence of PanNETs (benign vs malignant)
* 90% of insulin- producing tumors are BENIGN
| * 60-90% of other functioning and nonfunctioning tumors are MALIGNANT
132
Citeria of malignancy in PanNETs
○ Metastases
○ Vascular invasion
○ Local Infiltration
133
Associated syndromes in Pancreatic Neuroendocrine Tumor (PanNETs)
* MEN1
* VHL Disease
* Neurofibromatosis 1
* Tuberous Sclerosis
134
Most common PanNETs
Insulinoma (B-cell tumor)
135
Triad present in Insulinoma (B-cell tumor)
WHIPPLE TRIAD
○ Mental confusion, fatigue, weakness and convulsions
○ Clinically significant hypoglycemia (<50 mg/dl)
○ Relief of symptoms by administration of glucose
136
Characteristic feature of Insulinoma (B-cell tumor)
Deposition of amyloid
137
Which pNET:
• Monotonous neoplastic cell, minimal pleomorphism or mitotic activity
• Abundant Amyloid deposition (insulinoma)
• Clinically: episodic hypoglycemia
INSULINOMA (B-CELL TUMOR)
or
PANCREATIC ENDOCRINE NEOPLASM –
(ISLET CELL TUMOR)
138
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
GASTRINOMA/ ZOLLINGER-ELLISON SYNDROME
139
Most common location of Gastrinoma/ ZES
Duodenum and peripancreatic soft
| tissues
140
pNET Grading
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
NEC: small cell vs large cell
- 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
Which pNET; what hormone?
| • Sx: weakness, sweating, tremors, palpitations, confusion, visual changes
* Sx is Hypolycemia
* Hormone: Insulin
* Dx: Insulinoma
143
Which pNET; what hormone?
| • Abdominal pain, refractory peptic ulcer disease, secretory diarrhea
* Hormone: Gastrin
| * Dx: Gastrinoma/ ZES
144
Which pNET; what hormone?
| • Dermatitis, diabetes mellitus, diarrhea, DVT
* Sx 4Ds syndrome
* Hormone: Glucagon
* Dx: Glucagonoma
145
Which pNET; what hormone?
| • Profuse watery diarrhea, dehydration, hypokalemia, achlorhydria
* Sx: WDHA syndrome
* Hormone: VIP
* Dx: VIPoma (Verner-Morrison)
146
Which pNET; what hormone?
| • Diabetes mellitus, cholelithiasis, steatorrhea, anemia, weight loss
* Hormone: Somatostatin
| * Dx: Somatostatinoma
