Path 11 Schneider Flashcards
Cushing disease
Only pituitary adenoma/microadenomas -> ACTH
Most common ectopic ACTH production
Small cell lung CA
Dexamethasone suppression test
Won’t suppress ectopic ACTH or Hypercorticism Autonomous
Cushing disease morphologic change
Bilateral hyperplasia of adrenal Zona Fasciculata
-Atrophy in C syndrome
Cushing syndrome clinical appearance
- Moon facies
- Central obesity
- Abdominal striae (skin atrophy)
- Osteoporosis
- Muscle wasting
- Emotional disturbance
Conn syndrome
Primary aldosteronism
Primary aldosteronism causes
Adrenocortical adenoma (aldosteronoma)
- Zona Glomerulosa hyperplasia (less)
- Adrenocortical carcinoma (least)
Primary aldosteronism clinical (4)
- HTN
- Hypokalemia
- Sodium and water retention
- Decreased serum Renin
Secondary aldosteronism causes
- Renal ischemia/tumor
- Profound edema (cirrhosis, CHF, neprhotic syndrome)
Secondary aldosteronism mechanism
Renin-angiotensin stimulation
-Serum renin increased (opposite of primary aldosteronism)
Primary v secondary aldosteronism
- Unilateral v bilateral ZG hyperplasia
- Decreased v increased serum Renin
Adrenal virilism
Congenital cortisol enzyme defect -> ACTH compensation -> adrenal hyperplasia -> adrogenic steroid production
Adrenal virilism enzyme
21-hydroxylase deficiency (salt wasting form -> hypotension)
11-hydroxylase deficiency (less common, salt retention -> HTN)
21-hydroxylase function
17-hydroxyprogesterone -> 11-deoxycortisol
21-hydroxylase deficiency
17-hydroxyprogesterone -> androstenedione
Adrenal virilism morphology
Zona Reticularis adenoma, carcinoma, hyperplasia
Pituitary deficiency
Secondary adrenal failure - No ACTH
-No hyperpigmentation - No B-MSH
Addison disease
Primary adrenocortical deficiency
-Autoimmune or tuberculosis
Addison clinical
- Hypotention
- Hyperpigmentation
- Decreased electrolytes except potassium
Tuberculosis histo
Caseating granuloma
Langhans giant cell
Waterhouse-Friderichsen syndrome (3)
Catastrophic adrenal insufficiency & vascular collapse
- Meningococcal meningitis
- Hemorrhagic necrosis of adrenal cortex due to DIC
Adrenal cortical adenoma
Loss of function?
Chromaffin cell tumors
Adrenal - Pheochromocytoma
Extra-adrenal - Paraganglioma
Pheochromocytoma
Surgically correctable HTN
- due to catecholamine hyperproduction
- usually benign
Pheochromocytoma test
Urine vanillylmandelic acid
- metanephrine
- normetanephrine
Pheochromocytoma EM
Membrane-bound secretory granules (black)
Neuroblastoma
Malignant childhood catecholamine tumor
-“Small blue cell” tumor
Neuroblastoma origin
Adrenal medulla
Neuroblastoma presentation
Large abdominal mass
-HTN
Neuroblastoma cause
N-myc oncogene amplification
- Copy number related to aggressiveness
- Occasionally converts into benign ganglioneuroma
