Coupon Accepted Successfully!


Adrenal Cortex

A. Hyperadrenalism: 3 distinctive patterns Q

  1. Cushing syndrome
  2. Hyperaldosteronism
  3. Adrenogenital syndrome

B. Cushing Syndrome: 4 causes Q

  1. Primary hypersecretion due to increased ACTH women; 30-40 yrs.
  2. Adrenal over secretion due to adenomas carcinomas. No effect of ACTH; known as ACTH independent Cushing syndrome
  3. Secretion of Ectopic ACTH. Small Cell Ca lung, Carcinoid tumors
  4. Administration of exogenous corticosteroids 


Pituitary - Crooke hyaline change (intermediate keratin -basophilic) Adrenal

  1. Cortical atrophy
  2. Diffuse hyperplasia
  3. Nodular hyperplasia
  4. Adenoma rarely carcinoma 

Diagnosis: Q

  1. 24 hour Urine Free Cortisol level
  2. Loss of normal diurnal pattern of cortisol secretion 

For D/D - Cause of Cushing SyndromeQ

  1. Often low dos~ dexamethasone no effect ACTH
    High dose dexamethasone → ACTH o normal
    Indicates Pituitary Cushing Syndrome
  2. Ectopic ACTH production is unresponsive to dexamethasone
  3. Adrenal Cushing Syndrome ACTH; no effect of dexamethasone on ACTH
    Primary Hyperaldosteronism Aldosterone Na+ K+

Primary (Conn Syndrome) - Aldosterone secreting neoplasia

Secondary Aldosterone due to activation or rennin angiotensin

21 Hydroxylase Deficiency defective conversion of progesterone to 11 deoxy cortisone by 21 hydroxylase.

3 syndromes­ Q

  1. Salt Wasting Syndrome
  2. Simple Virilising adrenogenitalism
  3. Non Classic adrenogenitalism

Adrenals are hyperplastic bilaterally with nodular cortex that is brown (as no lipid)

Adrenal Insufficiency:  
Primary Loss of Cortex: Idiopathic immune Infection (TB)
Acute Hemorrhage Necrosis (Waterhouse Friderichsen Syndrome)
Congenital Hypoplasia
Secondary Hypothalamus
Pituitary                                 Neoplasm/TB/Long Term Steroid treatment
Adrenal Carcinoma Highly malignant, Large hemorrhagic, Cystic
Cells are atypical, anaplastic with tumor giant cells
Cause hypertension

  1. Pheochromocytomas are neoplasms composed of chromaffin cells, which, like their non-neoplastic counterparts, synthesize and release catecholamines and, in some cases, other peptide hormones.
  2. These tumors are of special importance because, although uncommon, they (like aldosterone-secreting adenomas) give rise to a surgically correctable form of hypertension
Pheochromocytomas = "rule of 10s":Q
  • 10% = familial [associated with = MEN-2A, MEN-2B, type-1 NF, VHL and Sturge-Weber syndrome].
  • 10% = extra-adrenal, occurring most commonly at the organ of Zuckerkandl.
  • 10% = bilateral.
  • 10% = malignant; Frank malignancy is somewhat more common in tumors arising in extra-adrenal sites.

  1. Microscopically, pheochromocytomas are composed of polygonal to spindle-shaped chromaffin cells and their supporting cells, compartmentalized into small nests, or "Zellballen," by a rich vascular network.Q
  2. The cytoplasm of the neoplastic cells often has a finely granular appearance, highlighted by a variety of silver stains. The nuclei of the neoplastic cells are often quite pleomorphic.
  3. Both capsular and vascular invasion may be encountered in benign lesions, and the presence of mitotic figures per se does not imply malignancy. Therefore, the definitive diagnosis of malignancy in pheochromocytomas is based exclusively on the presence of metastases. These may involve regional lymph nodes as well as more distant sites, including liver, lung, and bone.Q
Biochemical & Pharmacologic Tests:
  1. 24h urine metanephrines, vanillylmandelic acid (VMA), and free catecholamines: sensitivity is broad
  2. Plasma catecholamines: Must be drawn under strict guidelines including patient fasting, supine for 30 minutes, and needle inserted at least 20 minutes prior to blood draw. Norepinephrine >2000pg/ml or epinephrine >200pg/ml is 95% specific and 85% sensitive.
  3. Clonidine suppression test: indicated if urine and plasma studies are non-diagnostic and history is still suggestive. Clonidine will suppress the sympathetic nervous system but not the pheochromocytoma production of catecholamines.
  4. Biopsy: contraindicated. Q

Test Your Skills Now!
Take a Quiz now
Reviewer Name