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Cortex
Medulla
Glucocorticoids (Cortisol)
Mineralocorticoids (Aldosterone)
Adrenacortical Androgens (Testosterone)
Catecholamines
Adrenal Cortex
Hypofunction (Insufficiency)
Acute
W-F Syndrome
Steroid withdrawal
Chronic (Addison's Disease)
Autoimmune
TB/Fungal
Metastatic disease
Amyloid/
Hematochromatosis
Hyperfunction
Cushing's Syndrome
Hyperaldosteronism
Virilization Syndromes
Adrenocortical Hyperfunction
Syndromes
Cortisol-------------> Cushing's Syndrome
Aldosterone--------> Hyperaldosteronism
Testosterone-------> Virilization Syndromes
Adrenocortical Hyperfunction
(Cushing's Syndrome)
1.Exogenous Glucocorticoid (Steroids) Administration
2.Endogenous
a. Primary - Hypothalamic - Pituitary Disease
ACTH - Cushing's Disease (~50%)
b. Primary Adrenocortical
Hyperplasia/Neoplasia (~25%)
c. Ectopic ACTH by Non-Endocrine Neoplasia
(~25%)
Cushing's Syndrome
(Hypercorticosteroids)
Glucocorticosteroids----> * BP : Wt. Gain
*Truncal Obesity, "Moon
Facies"
*Buffalo Hump
*Diabetes
Hyperglycemia,
Glycosuria, Polydipsia
*Cutaneous Striae
*Osteoporosis
Hypertension
Weight Gain
Truncal Obesity
"Moon" Facies
Buffalo Hump
Muscle Atrophy
Disease
Adrenocortical
No
No
Tumor
Ectopic
No
No
Iatrogenic
No
No
Hyperglycemia (80%)
K+
Eosinophils
Lymphocytes
Hyperaldosteronism
Primary - Adrenal Adenoma/Hyperplasia
Aldosterone Renin
BP Na
K
Secondary - CHF, Renal Disease, Pregnancy
Renin Aldosterone
BP Na
K
Hyperaldosteronism
Primary Aldosterone - Adenoma (80%) - Conn's Syndrome
Adrenal Hyperplasia (15%)
Hyperaldosteronism
Aldosterone
Na+
K+
BP
Congenital Adrenal
R
Hyperplasia (A )
Adrenogenital Syndromes
Primary Adrenal Disorders
or
Primary Gonadal
Virilization
Prepubertal Males
Adult Males
Adrenal Insufficiency
Primary ( ACTH)
Chronic (Addison's)
Acute Adrenal
Insufficiency
Secondary ( ACTH)
Hypopituitarism
Pituitary Adenomas (nonfunctioning)
Hypothalamic Lesions
Adrenal Insufficiency
Chronic
<10% of Adrenal Function
Autoimmune (60%-70%)
50% - Adrenals Only
50% - Other Organs
Infections (TB)
Metastatic Disease
Lung/Breast Cancer
Hyperpigmentation
Seen in Primary (JFK)
Not in Secondary
Na
K+
BP
Adrenal Insufficiency
Acute (Addison's Disease)
Waterhouse - Frederickson
Withdrawal of Steroids
Stress w/Chronic
Insufficiency
Adrenal Medulla
Physiologic
Stimuli
Epinephrine
Norepinephrine
Fight or
Flight
Catecholamine Metabolism
Norepinephrine
Normetanephrine
VMA
Epinephrine
Metanephrine
Neuronal Cells
Neuroblastoma
Ganglion Cell Tumors
Pheochromocytoma
Catecholamines
Norepinephrine
Epinephrine
Peptides
Neoplasm of chromaffin cells
Norepinephrine Only
Extraadrenal Sites - Paragangliomas
Adrenal pheochromocytomas
10% Malignant*
10% Bilateral
10% Familial (men, etc.) 10% Normotensive
*Malignancy More Common in Extra-Adrenal Sites
Pheochromocytoma - Morphology
Yellow Tan
Dark Brown
Small Polygonal Cells Arranged in Vascular Nests
EM-Membrane Bound Granules (Catecholamines)
Malignancy Based on Metastasis
Pheochromocytoma - Clinical
Urinary Free
Catecholamines
Vanillylmandelic
Acid (VMA)
Metanephrines
Neuroblastoma
Malignancy of Sympathetic
Neuronal Cells
90% < 5y/o
90% in Abdomen
60% in Adrenal
90% Catecholamines
BP is Rare
Multiple Endocrine
Neoplasia MEN Syndrome
Group of Familial Diseases (AD) Associated with
Neoplasms and/or Hyperplasia of Various Organs
MEN I - (Wermer's) - 11 q 13
Parathyroid Adenoma
Pancreatic Adenoma/Ca
Z-E Syndrome
Insulinomas
Pituitary Adenomas
Multiple Endocrine
Neoplasia MEN Syndrome
Group of Familial Diseases (AD) Associated with
Neoplasms and/or Hyperplasia of Various Organs
MEN IIA - (Sipple's) 10 q 11.2 (RET)
Pheochromocytoma
Medullary Ca of Thyroid
Multiple Endocrine
Neoplasia MEN Syndrome
Group of Familial Diseases (AD) Associated with
Neoplasms and/or Hyperplasia of Various Organs
MEN IIB or MEN III - 10 q 11.2 (RET)
Pheochromocytoma
Medullary Ca of Thyroid
PLUS
Neuromas/Ganglioneuroma
Pathologic Findings
70% Single Adenomas
10% Multiple Adenomas
10% Metastasizing
10% Hyperplasia
Gastrinoma
(Zollinger-Ellison Syndrome)