PanNETs

Question 1

Pancreatic Neuroendocrine Tumors are tumors of what cells?

Answer 1

islets

Question 2

Unequivocal criteria for malignancy of PanNETs

Answer 2

Metastases, vascular invasion, and local infiltration

Question 3

1. Are most (90%) functional insulin producing PanNETs benign or malignant?
2. Are most (60-90%) other functioning and nonfunctioning NETs benign or malignant?

Answer 3

1. benign if insulin producing

2. malignant if non-insulin producing

Question 4

MEN1 (causing PanNETs) due to loss-of-function mutations what two tumor suppressor genes?

Answer 4

PTEN and TSC2

Question 5

MEN1 (causing PanNETs) due to inactivating mutations what two genes?

Answer 5

– Alpha-thalassemia/mental retardation syndrome, X-linked (ATRX)
– Death-domain associated protein (DAXX)

Question 6

A person presents with hypoglycemic episodes (blood glucose below 50 mg/dL)
– Confusion, stupor, and loss of consciousness
– Precipitated by fasting or exercise
– Relieved by feeding or parenteral administration of glucose.
Most likely?

Answer 6

Hyperinsulinism due to insulin secreted by Insulinoma

Question 7

What is an insulinoma

Answer 7

B-cell tumor MOST COMMON PANCREATIC neoplasm. Can secrete enough insulin to induce clinically significant hypoglycemia

Question 8

~20% may be asx, but have high circulating levels of insulin and a high insulin-to-glucose ratio

Answer 8

insulinoma

Question 9

tx of insulinoma

Answer 9

surgical removal of tumor

Question 10

Clinically present with peptic ulceration (in the duodenum and stomach)
• Ulcers are often unresponsive to therapy
• Can also get ulcers in unusual locations such as the jejunum
• >50% have diarrhea

Answer 10

hypergastrinemia (extreme gastric acid secretion) due to Zollinger Ellison Syndrome

Question 11

Most likely locations of ZES gastrinomas a possible location of metastasis.

Answer 11

As likely to arise in the duodenum and peripancreatic soft tissues as in the pancreas
-Met to liver –> liver failure in 10 years

Question 12

What syndrome is associated with ZES?

Answer 12

MEN-1

Question 13

Tx ZES with what?

Answer 13

PPI to control gastric acid secretion. Total resection when possible to eliminate syndrome.

Question 14

Tumor that causes: Watery diarrhea, hypokalemia, achlorhydria (WDHA syndrome)

Answer 14

VIPoma (release of vasoactive intestinal peptide (VIP))

Question 15

Tumor that causes:
– Diabetes mellitus, cholelithiasis, steatorrhea, and
hypochlorhydria
– Difficult to localize preoperatively

Answer 15

δ-cell tumors (somatostatinomas)

Question 16

Tumor that causes:
– Mild diabetes mellitus, a characteristic skin rash (necrolytic
migratory erythema), and anemia
– Most frequently in perimenopausal and postmenopausal women

Answer 16

α-cell tumors (glucagonomas)

Question 17

Major lab difference between Hyperosmolar Hyperosmotic Syndrome and Diabetic Ketoacidosis

Answer 17

Hyperglycemia:
HHS - 600-1200 (EXTREMELY high)
DK - 250-600 (Very high)

Question 18

ulcers in unusual locations such as the jejunum

Answer 18

hypergastrinemia (extreme gastric acid secretion) due to Zollinger Ellison Syndrome