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Adrenals

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%)

Hypercortisolism (Cushing's Syndrome) - Adrenal


Pathology
Exogenous Steroids----------------> Bilateral Atrophy
Pituitary Adenoma-----------------> Bilateral Hyperplasia
+ Some Nodularity
Ectopic (Paraneoplastic)-----------> Bilateral Hyperplasia
+ Some Nodularity
Primary Adrenal Neoplasia-------> Unilateral Nodule/Mass
with Atrophy

Cushing's Syndrome
(Hypercorticosteroids)
Glucocorticosteroids----> * BP : Wt. Gain
*Truncal Obesity, "Moon
Facies"
*Buffalo Hump
*Diabetes
Hyperglycemia,
Glycosuria, Polydipsia
*Cutaneous Striae
*Osteoporosis

Cushing's Syndrome Clinical Syndrome

Hypertension
Weight Gain
Truncal Obesity
"Moon" Facies
Buffalo Hump
Muscle Atrophy

Decreased Muscle Mass


Diabetes (the 3P's)
Fragile Skin
Osteoporosis
Infections
Mental Disturbances

Cushing's Syndrome Lab Diagnosis


1. 24-hr Urine Free Cortisol (& Plasma
Cortisol)
2. Loss of Diurnal Cortisol Secretion
3. Dexamethasone Suppression Test

Dexamethasone Suppression Test


Disorder
UFC ACTH LD-DST HD-DST
Cushing's
No
Yes

Disease
Adrenocortical
No
No

Tumor
Ectopic
No
No

Iatrogenic

No

No

Cushing's Syndrome Abnormal Lab Findings


Na+

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 )

Hereditary Defect in Cortisol Biosynthesis


21-hydroxylase deficiency- 95% (Chromosome 6)
17-hydroxylase deficiency
11-beta hydroxylase deficiency

Adrenogenital Syndromes
Primary Adrenal Disorders
or
Primary Gonadal

Virilization

Affects of Excess Androgens in CAH

Females < 12 Weeks Gestation

Male Type External Genitalia


Various Degrees of Pseudo-hermaphroditism

Prepubertal Adult Females

Various Degrees of Masculinization

Prepubertal Males

Precocious development of secondary sex characteristics

Adult Males

Accentuates existing characteristics

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

Adrenal Insufficiency Clinical (Insidious)

Weakness & Fatigue


GI Sxs
Anorexia, Nausea
Vomiting, Weight Loss

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

Adrenal Medulla - Neoplastic


Chromaffin Cells
Pheochromocytoma

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

Small (mg) Circumscribed


K-Dichromate

Large (kg) Masses

Yellow Tan
Dark Brown
Small Polygonal Cells Arranged in Vascular Nests
EM-Membrane Bound Granules (Catecholamines)
Malignancy Based on Metastasis

Pheochromocytoma - Clinical

Hypertension ( BP) - 90%


75% - Sustained
25% - Paroxysmal

ACTH or Somatostatin - In Some Cases With


Associated Clinical Features

Pheochromocytoma Laboratory Diagnosis

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

Islet Cell Tumors


Rare - 1 in 100,000 Patients
May or May not Produce Hormones
2/3 Benign
1/3 Malignant

Islet Cell Tumors


Hyperinsulinemias
Gastrinomas (Zollinger - Ellison's Syndrome)
Multiple Endocrine Neoplasia (MEN)

Insulinoma (Beta Cell Tumors)


Clinical
Paroxysmal
Hypoglycemia
( Glucose: Insulin)
CNS - Confusion,
Unconsciousness
Relief with Glucose
Administration

Pathologic Findings
70% Single Adenomas
10% Multiple Adenomas
10% Metastasizing
10% Hyperplasia

Gastrinoma
(Zollinger-Ellison Syndrome)

Recalcitrant Peptic Ulcer Disease


Gastric Hypersecretion of H2
Diarrhea
Rx H2 Blockers and Resection
40% Adenomas
60% Carcinomas

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