adrenals and pancreas pathology; MENs, and other odd tumors

Question 1

insulinoma

Answer 1

tumor of pancreatic b-cells; hyperinsulinemia

presentaiton:

• episodic hypoglycemia: stupor/confusion/pass out
  
  • precipitated by exercise or fasting
  • relieved by food
• elevated c-peptide

relatively benign(90%)

Question 2

gastrinoma

Answer 2

gastrin secreting tumor of pancreas or duodenum

Zollinger-ellison syndrome

presentation:

• abdominal pain – severe peptic ulcers from hypersecretion of acid
• diarrhea
• can present as a part of MEN1

Question 3

adrenal cortical adenoma vs carcinoma

Answer 3

adenomas are smaller, less likely to make androgens

carcinomas are much larger, can present with virilization(Androgens)

Question 4

which adrenal zones growth the most in adrenal hyperplasia?

Answer 4

zona fasculata and reticularis

Question 5

myelolipoma

Answer 5

benign tumor of fat and hematopoeitic cells in the adrenal glands

looks like bone-marrow in the adrenals

Question 6

pheochromocytoma

presentation?

Answer 6

tumor of adrenal medulla

presentaiton:

• paroxysmal HTN
• tachycardia, palpitations, HA, sweat, tremor, nervous

Question 7

what is used to diagnose pheochromocytoma

Answer 7

measuring urine catecholamines: VMA, metanephrines

Question 8

what syndromes are pheochromocytomas seen in?

Answer 8

MEN2A, B

Neurofibromatosis 1(NF1)

Von Hippel Lindau(VHL)

Question 9

rule of 10s for pheochromocytoma

Answer 9

• 10% bilateral
• 10% extraadrenal(paragangliomas)
• 10% malignant
• 10% asymptomatic
• 10% familial

apparently some of these are not really 10%….BUT WHO CAYAS

Question 10

neuroblastoma

Answer 10

adrenal or paraganglia tumor of young ppl

solitary lesion presenting as:

abdominal mass

watery diarrhea

child(80% <4 yo)

Question 11

histology of neuroblastoma

Answer 11

small blue cell tumor

homer wright rosettes in 25%

Question 12

ganglioneuroma

Answer 12

benign paraganglia tumor in mostly adults

differentiated ganglion cells and spindled schwann-like cells

Question 13

ganglioneuroblastoma

Answer 13

paraganglia(mainly) tumor of children(mainly)

features of ganglioma and neuroblastoma present

Question 14

most common origin of mets to adrenal glands?

Answer 14

lung

breast

Question 15

MEN1

Answer 15

3 P’s:

• parathyroid - usually present first
• pancreas - usually aggressive
• pituitary - prolactinoma mostly

Question 16

MEN2A

Answer 16

medullary thyroid carcinoma

pheochromocytoma - bilateral, increased extra-adrenal paraganglioams

parathyroid hyperplasia

RET gene mutation

Question 17

MEN 2B

Answer 17

medullary thyroid carcinoma

pheochromocytoma

mucosal neuromas

marfanoid habitus

still due to RET mutation like MEN2A