3.2.1 Adrenal Cortex and Medulla Clinical Cases I

Question 1

What are some of the typical findings in Cushing Syndrome?

Answer 1

Question 2

What is the triad of pheochromocytoma?

Answer 2

headache, sweating, and papitations

(50% have sustained HTN)

Question 3

Name the layers of the adrenal gland. Be specific.

Answer 3

Question 4

What is shown in this MRI?

Answer 4

Large mass in the left adrenal gland

Question 5

What is produced by the tumor in pheochromocytoma?

Answer 5

Excess catcholamines

Question 6

If a patient with pheochromocytoma doesn’t present with sustained HTN, what are they likely to present with?

Answer 6

Paroxysmal spells

Question 7

If MRI imaging doesn’t initially qualify a patient with Cushing’s syndrome for surgery, what is the next step or test in the treatment of this patient?

Answer 7

Inferior petrosal sinus sampling (IPSS)

-Testing ACTH levels before and after CRH stimulation, ratio >3 is consistent with Cushing’s Dz

Question 8

What are some conditions that can lead to cushing syndrome?

Answer 8

ACTH producing pituitary adenoma (Cushing Dz)

Exogenous steriod use

Ectopic ACTH syndrome (lung cancer)

Androgen producing tumor

PCOS

Morbid obesity

Question 9

What are the three layers of the adrenal cortex?

Answer 9

Zona glomerulosa

Zona fasciculata

Zona reticularis

(GFR)

Question 10

Where are hemangioblastomas found in VHL patients?

Answer 10

Brain, spinal cord, and retina

Question 11

What are some of the other conditions aside from pheochromocytoma found in neurofibromatosis, type 1 (Von Recklinghausen Syndrome)?

Answer 11

Peripheral neurofibromas, Cafe-au-lait pigmentation, bone dysplasia

Question 12

What are two important bodily changes that occur in Cushing’s syndrome?

Answer 12

Purple abdominal Striae

Proximal muscle weakness/wasting

Question 13

Treatment options for pheochromocytoma

Answer 13

Surgical removal

Pre-op prep with alpha blockers (two weeks) followed by beta-blockers

Need for volume replacement

Question 14

What are some other conditions aside from pheochromocytoma associated with multiple endocrine neoplasia, type 2?

Answer 14

Medullary thyroid carcinoma, primary hyperparathyroidism, mucosal neuromas (MEN 2B)

Question 15

What is the rule associated with pheochromocytoma?

Answer 15

Rule of 10

10% bilateral

10% extraadrenal (paragangliomas)

10% above the diaphragm

10% familial (now closer to 30%)

10% malignant

Question 16

What are some conditions secondary to Cushing’s syndrome?

Answer 16

Diabetes, HTN, hyperlipidemia, coagulopathy, osteoporosis, depression, hypogonadism, central hypothyroidism, decreased growth velocity (children)

Question 17

What are some labratory results that can help in the diagnosis of pheochromocytoma?

Answer 17

Elevated plasma fractionated metanephrines

Elevated 24 hr urinary fractionated catecholamines, metanephrines, and VMA (vanillylmandelic acid)

Presence of adrenal mass on CT

Special techniques: MIBG scan, pentetreotide, PET scan

Question 18

How does one treat a patient with Cushing Syndrome?

Answer 18

Surgery (depending on location)

Medications inhibiting cortisol production

Role of bilateral adrenalectomy when other options fail

Question 19

What are the three tests used in diagnosing Cushing’s syndrome? The results and findings?

Answer 19

Need a positive on 2 out of 3

Dexamthasone CRH is used to confirm diagnosis

Question 20

What does that person have?

Answer 20

Multiple endocrine neoplasia, type 2

Question 21

Name this medical student.

Answer 21

If you said Megan McCracken, you are correct.

Question 22

What are some conditions that have similar clinical presentations to pheochromocytoma?

Answer 22

Anxiety attacks, thyrotoxicosis, amphetamine/cocaine use, clonidine withdrawl, hypoglycemia, angina/MI, brain tumor, subarachnoid hemorrhage, nueroblastoma (child), preeclampsia

Question 23

What are familial syndromes associated with pheochromocytoma?

Answer 23

VHL syndrome

Neurofibromatosis, Type 1

Multiple endocrine neoplasia, type 2

Familial paraganglimoas (SDH mutation)

TMEM 127 germ line mutation

Question 24

What enzymes are important in the metabolism of catecholamines?

Answer 24

COMT

MA + AO

Question 25

What enzyme is important in the conversion of norepinephrine to epinephrine?

Answer 25

PNMT

Question 26

What is the rate limiting step in the production of catecholamines?

Answer 26

Tyrosine to DOPA

Question 27

What are some other cancers that VHL patients are at increased of?

Answer 27

Renal cysts and renal cell carcinoma

Endolyphatic sac tumors

Papillary cystadenomas of the epididymis (males) or broad ligament (females)

Question 28

What is important in differentiating the cause of Cushing’s syndrome?

Answer 28

ACTH independence