Nuroendocrine Tumors

Question 1

Multiple Endocrine Neoplasms (MEN)

Answer 1

The term MEN applies specifically when two or more endocrine glands are affected.

Question 2

MEN Syndromes

Answer 2

MEN 1 = parathyroid tumors, pancreatic tumors, pituitary tumors

MEN 2a = medullary thyroid cancers, pheochromocytoma, parathyroid

MEN 2b = Medullary thyroid cancers, pheochromocytoma, neuromas

Question 3

MEN 1

Answer 3

Almost all who inherit MEN1 develop over-activity of the parathyroid. (which is usually the first clinical manifestation.- hyperparathyroidism, bones stones moans and groans)
Pancreas is next.
Pituitary adenoma occurs last

Question 4

MEN 2

Answer 4

Divided into:

MEN 2a = medullary thyroid cancers, pheochromocytoma, parathyroid…do genetic testing

MEN 2b = Medullary thyroid cancers, pheochromocytoma, neuromas…have developmental abnormalities looking like Marfan’s, take thyroid out!

Question 5

Whipple’s Triad, Insulinoma Dx.

Answer 5

Hypoglycemia during fasting
Serum glucose less than 50 mg/dL during sx’s
Relief of hypoglycemic symptoms by exogenous glucose administration

Question 6

GastrinomaZollinger-Ellison Syndrome

Answer 6

Tumor of pancreas or duodenum
Produces excess levels of gastrin (Gastrin stimulates gastric acid and pepsin production)
25% are part of MEN-1 syndrome

Question 7

GastrinomaZollinger-Ellison Syndrome Treatment

Answer 7

Control gastric hypersecretion:
High Doses of Proton pump inhibitors

Surgical removal of gastinoma

Question 8

VIPoma = Verner-Morrison Syndrome

Answer 8

Pancreatic neoplasm secreting vasoactive intestinal polypeptide (VIP).
Tail of the Pancreas
Get watery diarrhea

Question 9

Glucagonoma

Answer 9

Pancreatic Islet Cell tumor that secretes glucagon
Increases levels of glucose in blood
Produces a distinctive rash
80% are cancerous
Grows slowly
80% women

Question 10

Carcinoid Tumors

Answer 10

well-differentiated neuroendocrine tumors.

Originate mostly in the digestive tract, and lungs, or rare primary sites such as kidneys or ovaries.

Question 11

Carcinoid syndrome

Answer 11

the term applied to a constellation of symptoms mediated by various humoral factors that are elaborated by some carcinoid tumors
The typical carcinoid syndrome occurs predominantly in patients with metastatic carcinoid tumors originating in the small intestine.

Question 12

Carcinoid Syndrome Symptoms & Signs

Answer 12

Periodic abdominal pain
Flushing (Episodic, Exercise, stress, certain foods, Increasing duration)
Diarrhea/malabsorption