Adrenal medulla

Question 1

Pheochromocytoma

Answer 1

MC adult adrenal medulla tumor
Derived from chromaffin cells – neural crest
Produces catecholamines –DA, NE, Epi

Episodes of HTN, HA, palpitaitons, tachycardia, diaphoresis

Dx: Plasma metanephrine, normetanephrine, urine vanillylmandelic acid (VMA)
Catecholamines

90%: benign, u/l, adrenal medulla, do not calcify, adults

10% malignant, b/l, extraadrenal, calcify, kids

Assoc w/ MEN2A, MEN 2B, Neurofibromatosis type 1

Can secrete erythropoietin -> polycythemia

Tx: pretreat with alpha blocker – phenoxybenzamine (nonselective) then a beta blocker
Surgery

Question 2

Erythropoietin secreting tumors

Answer 2

Pheochromocytoma
RCC
Hemangioblastoma (vascular tumor of CNS)
Hepatocellular carcinoma

Uterine fibroids – leiomyoma (fibroids + heavy vleeding but not anemic)

Question 3

Neuroblastoma

Answer 3

Tumor sympathetic ganglion cells
40% in adrenals
MC adrenal medulla tumor in kids

Defective in catecholamine production – little DA, VMA, HMA
-> mild HTN

monitor VMA/HMA in blood

N-myc oncogene

Bombesin tumor marker

Neurofilament stain

Histo:
Homer-wright rosettes
Tumor cells around central tangle of fibrils

Question 4

MEN1

Answer 4

3 Ps
Parathyroid adenoma – 100% penetrance, one gland
Pituitary adenoma
Pancreatic tumor
-gastrinoma: Zollinger-Ellison – peptic ulcers
-insulinoma -> hypoglycemia
-glucagonomas -> hyperclycemia and DM
-VIPoma -> diarrhea

commoly present as kidney stones and stomach ulcers

Question 5

MEN2A

Answer 5

RET oncogene

PPM
Pheochromocytoma – 45%
Parathyroid hyperplasia – all glands
Medullary thyroid cancer – neck mass
-90%

Question 6

MEN2B

Answer 6

RET oncogene

PMM
Pheochromocytoma
Medullary thyroid
Mucosal neuromas – oral and intestinal ganglioneuromatosis