Pancreas 3

Question 1

Syndromes that predispose to pancreatic cancer (in decreasing order of lifetime risk) (six)

Answer 1

HPF FLO

Hereditary pancreatitis - 40%
Peutz-Jeghers - 25%
Familial atypical multiple mole melanoma syndrome - 17%
Familial pancreatic cancer - 10%
Lynch syndrome - 4%
Hereditary ovarian/breast cancer syndrome (BRCA) - 2%

Question 2

Enlarged palpable gallbladder and jaundice - name of sign? Suggests?

Answer 2

Courvoisier’s sign ; Pancreatic cancer

Question 3

Biomarkers associated with pancreatic cancer-associated diabetes?

Answer 3

Vanin-1 and Matrix metalloproteinase 9

Question 4

Proliferation markers for neuroendocrine tumors?

Answer 4

Mitotic index; Ki-67

Question 5

When should endoscopic stent placement be considered in patients with malignant gastric outlet obstruction? Otherwise?

Answer 5

Estimated three month survival

Gastrojejunostomy

Question 6

Specialized imaging studies for pNETs?

Answer 6

Octreoscan (indium-111 labeled octreotide

Gallium-68 DOTATATE PET

Question 7

Most common functional pNET?

Tests/cutoff?

Answer 7

Insulinoma

72 hour fast with hypoglycemia (glucose < 55) with elevated insulin (>3) and elevated c-peptide (>.6)

Question 8

Treatment of insulinoma? Second line?

Answer 8

Surgery

EUS-guided ethanol ablation

Question 9

Most common malignant functional pNET?

Answer 9

Pancreatic gastrinoma

Question 10

Patient presents with pancreatic mass and pruritic maculopapular rash that develops into bullae with crusting.

tumor? Name of Dermatologic finding?

Patients also present with?

Diagnosis?

Answer 10

Glucagonoma; Necrolytic migratory erythema

DVT

Glucagon over 500

Question 11

pNETs That are usually metastatic at the time diagnosis?

All should be treated with?

Answer 11

Glucagonoma
Somatostatinoma
VIPoma

Surgical resection and octreotide

Question 12

Tumor the presents with watery diarrhea, hypokalemia, and achlorhydria?

Answer 12

VIPoma

WDHA syndrome

Question 13

Autosomal dominant disorder on chromosome 11 that results in multiple pNETs?

Alternative mutation?

Most common types?

Answer 13

MEN type one

CDKN1B (MEN type 4)

Gastrinomas and insulinomas

Question 14

Autosomal dominant disorder on chromosome 4 that results in pNETs?

Types?

Answer 14

VHL

Usually non-functional pNETs

Question 15

Tumors Associated with neurofibromatosis type one?

Answer 15

Small intestinal GISTs,
Somatostatinomas,
Neurofibromas

Question 16

Disease associated with G.I. tract hamartomas, insulinoma, somatostatinoma?

Gene? Chromosome?

Answer 16

Tuberous sclerosis

tuberous sclerosis complex 1 and 2 on chromosome 9 and 16