Adrenal Pathology

Question 1

Types of Hypercortisolism

Answer 1

1. Pituitary Adenoma “Cushing’s Disease”
2. Adrenal Tumor; Adrenal Nodular Hyperplasia
3. Paraneoplastic Syndrome (SCLC)
4. Iatragenic (Steroids)

Question 2

Cushing Disease

- Morph

Answer 2

• Morph: Bilateral Hyperplasia; Can be unequal + mimic unilateral neoplasm; Homogenous basophilic material due to intermediate keratin filaments i cytoplasm (Crooke Hyaline change)

Question 3

Primary Adrenal Cortical Hyperplasia

• EPI:
• A/S
• Morph

Answer 3

• EPI: 8% of Cushing Syndrome; F>M; 80% tumors nonfunctioning
• A/S: MEN1
• Morph: Discrete nodule of bland adrenal corticol cells; similar to normal; Unilateral

Question 4

Adrenal Cortical Carcinoma

- Morph

Answer 4

• Morph: Vascular invasion; Pleomorphism; nuclear hyperchromism

Question 5

Clinical Presentation Cushing Syndrome

Answer 5

• Moon Face + Buffalo Hump: Fat accumulation in face, neck, + upper body
• Thin, transparent skin w/ Striae rubrae: Catabolic effects of insulin resistance -> loss of collagen
• Plethora/Ruddy complexion
• Frequent bruising
• Females: Hursitism, acne, back pain, fatigue, Osteopenia, edema; Neuro changes
• Atrophy of fast-twitch muscle fibers

Question 6

Cortisol Lab Values Reference ranges

Answer 6

Serum: 4.5 - 23
Urinary: <100
Plasma ACTH: 10 - 60

Question 7

Which pathology shows suppression in high-dose dexamethasone test? Which fail to suppress?

Answer 7

Cushing Disease: Suppression

Ectopic ACTH: Fails

Adrenal Tumor: Fails

Question 8

Primary aldosteronism Causes

Answer 8

• Adrenal Adenoma (Conn Syndrome)
• ## Idiopathic (IHA; >/=60%)

Question 9

Conn Syndrome Morph

Answer 9

• Solitary, <2 cm, well-circumscribed bright yellow lesion
• Uniform, Lipid-laden cells similar to fasiculata cells
• Spironolactone bodies: eosinophilic laminated cytoplasmic inclusions

Question 10

Conn Syndrome Lab Testing

Answer 10

• Hypokalemia, Hypernatremia, alkalosis, hypochloremia, elevated plasma, urine aldosterone, + suppressed renin
• Failure of aldosterone to suppress following salt loading: confirms diagnosis

Question 11

Secondary Hyperaldosteronism cause

Answer 11

Enhanced production/release of Renin from kidneys

Question 12

FH Type I

• Genetics
• LabDx

Answer 12

• G: Dominant mutation in CYP11B1 (11 B-Hydro) + CYPaaB2 (Ald syn)
• LabDx: Normal ACTH

Question 13

CAH

• Genetics
• S/S
• LabDx:
• subtypes

Answer 13

• G: AR LOF CYP21A (21 hydroxylase)
• S/S: Ambiguous Genitalia, early virilization, salt wasting at birth
• LabDx: DEC cortisol; INC androgens; Bilateral Hyperplasia; thickened + nodular; depletion of lipids
• Types: Classical (Salt-wasting + life-threatening), Simple (Virilizing at birth; sex organ fusions), Non-classic (mild; precocious puberty; hirsutism)

Question 14

Addison’s Disease
- Et
- Morph
-

Answer 14

• Et: Most common TB; Autoimmune #1 in US

- Morph: Fibrous capsule w/ cortical destruction; Diffuse lymphocytic infiltration; Medulla usually intact

Question 15

Causes Acute adrenal crisis

Answer 15

• Addisonian crisis: Failure to INC steroids in response to stress;
• Rapid withdrawal of steroids
• Hemorrhage

Question 16

Water-house-Friderichsen syndrome

• Morph
• Et

Answer 16

• Vasculitis; hemorrhage within medulla extending to cortex

- Et: Neis. Menin; Pseudo sp.

Question 17

Biggest sign of benign Pheochromocytoma

Answer 17

Swings in BP btwn HTN + Hypotension

Question 18

Sites of Neural Crest Neoplasms

Answer 18

1. Adrenal glands (90%)

2. Extra-adrenal -> Abdominal Aorta is common

Question 19

Paraganglioma

Answer 19

• Extra-adrenal pheochromocytoima

“Rule of 10”

• outside the extra-adrenal
• children
• bilateral
• malignant
• recur after Sx
• familial

Question 20

Chilhood PCC A/S

Answer 20

• MEN
• VHL
• NF 1

Question 21

MEN2 A/B A/S

Answer 21

MEN2A: MTC; Primary hyperparathyroidism

MEN2B: MTC; mucosal neuroma; marfinoid habitus

Question 22

VHL

• Genetics
• A/S

Answer 22

• Mutation in TSG VHL
• A/S: Benign cerebellar hemangioblastomas, retinal angiomas, multiple pancreatic and renal cysts, extra-adrenal pheochromocytomas

Question 23

PCC Morph

Answer 23

• Necrosis + Hemorrhage
• Medulla enlargement; Cortex thinning
• Nests of tumor cells “Zellballen” surrounded by fibrovascular stroma

Question 24

NC Neoplasm Morph

Answer 24

Ganglioneuroma: encapsulated, firm Tumro w/ CS myxoid

Neuroblastoma: Soft, lobu;lated, w/ necrosis + hemorrhage -> Homer-Wright Pseudo-rosettes: small primitive cells in solid sheets

Question 25

Paraganglioma Morph

Answer 25

• 1-5 cm red-brown tumor
• Commonly on Carotid Body
• eosinophilic cytoplasm w/ inform round nuclei + endocrine granules
• enclose d by trabeculae of fibrous elongated cells