3.2.1 Adrenal Cortex and Medulla Clinical Cases I Flashcards
What are some of the typical findings in Cushing Syndrome?
What is the triad of pheochromocytoma?
headache, sweating, and papitations
(50% have sustained HTN)
Name the layers of the adrenal gland. Be specific.
What is shown in this MRI?
Large mass in the left adrenal gland
What is produced by the tumor in pheochromocytoma?
Excess catcholamines
If a patient with pheochromocytoma doesn’t present with sustained HTN, what are they likely to present with?
Paroxysmal spells
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?
Inferior petrosal sinus sampling (IPSS)
-Testing ACTH levels before and after CRH stimulation, ratio >3 is consistent with Cushing’s Dz
What are some conditions that can lead to cushing syndrome?
ACTH producing pituitary adenoma (Cushing Dz)
Exogenous steriod use
Ectopic ACTH syndrome (lung cancer)
Androgen producing tumor
PCOS
Morbid obesity
What are the three layers of the adrenal cortex?
Zona glomerulosa
Zona fasciculata
Zona reticularis
(GFR)
Where are hemangioblastomas found in VHL patients?
Brain, spinal cord, and retina
What are some of the other conditions aside from pheochromocytoma found in neurofibromatosis, type 1 (Von Recklinghausen Syndrome)?
Peripheral neurofibromas, Cafe-au-lait pigmentation, bone dysplasia
What are two important bodily changes that occur in Cushing’s syndrome?
Purple abdominal Striae
Proximal muscle weakness/wasting
Treatment options for pheochromocytoma
Surgical removal
Pre-op prep with alpha blockers (two weeks) followed by beta-blockers
Need for volume replacement
What are some other conditions aside from pheochromocytoma associated with multiple endocrine neoplasia, type 2?
Medullary thyroid carcinoma, primary hyperparathyroidism, mucosal neuromas (MEN 2B)
What is the rule associated with pheochromocytoma?
Rule of 10
10% bilateral
10% extraadrenal (paragangliomas)
10% above the diaphragm
10% familial (now closer to 30%)
10% malignant
What are some conditions secondary to Cushing’s syndrome?
Diabetes, HTN, hyperlipidemia, coagulopathy, osteoporosis, depression, hypogonadism, central hypothyroidism, decreased growth velocity (children)
What are some labratory results that can help in the diagnosis of pheochromocytoma?
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
How does one treat a patient with Cushing Syndrome?
Surgery (depending on location)
Medications inhibiting cortisol production
Role of bilateral adrenalectomy when other options fail
What are the three tests used in diagnosing Cushing’s syndrome? The results and findings?
Need a positive on 2 out of 3
Dexamthasone CRH is used to confirm diagnosis
What does that person have?
Multiple endocrine neoplasia, type 2
Name this medical student.
If you said Megan McCracken, you are correct.
What are some conditions that have similar clinical presentations to pheochromocytoma?
Anxiety attacks, thyrotoxicosis, amphetamine/cocaine use, clonidine withdrawl, hypoglycemia, angina/MI, brain tumor, subarachnoid hemorrhage, nueroblastoma (child), preeclampsia
What are familial syndromes associated with pheochromocytoma?
VHL syndrome
Neurofibromatosis, Type 1
Multiple endocrine neoplasia, type 2
Familial paraganglimoas (SDH mutation)
TMEM 127 germ line mutation
What enzymes are important in the metabolism of catecholamines?
COMT
MA + AO
What enzyme is important in the conversion of norepinephrine to epinephrine?
PNMT
What is the rate limiting step in the production of catecholamines?
Tyrosine to DOPA
What are some other cancers that VHL patients are at increased of?
Renal cysts and renal cell carcinoma
Endolyphatic sac tumors
Papillary cystadenomas of the epididymis (males) or broad ligament (females)
What is important in differentiating the cause of Cushing’s syndrome?
ACTH independence
