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Systems Exam 3 > Endocrine Pathology III > Flashcards

Endocrine Pathology III Flashcards

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1
Q

What are the Adrenocortical Hyperfunction Diseases?

A
  • Cushing Syndrome: Excess cortisol
  • Hyperaldosteronism: Excess aldosterone
  • Adrenogenital/Virilizing Syndrome: Excess androgens
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2
Q

What are the Adrenocortical Hypofunction Diseases?

A

Primary (adrenal origin):
- Acute: Adrenal crisis
- Chronic: Addison’s disease

Secondary (pituitary/hypothalamic origin): Due to ↓ ACTH

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3
Q

What is Hypercortisolism (Cushing Syndrome) and what are the two causes?

A

Excess cortisol due to either:
- Exogenous steroids (most common cause)
- Endogenous overproduction either ACTH-Dependent or ACTH-Independent

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4
Q

What is the main causative agent for Exogenous Crushing Syndrome?

A

Glucocorticoid therapy

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5
Q

What are the main causes dependent and independent ACTH Endogenous overproduction in Crushing Syndrome?

A

ACTH-Dependent:
- Pituitary adenoma (Cushing Disease): 70% of cases, mostly in young women
- Ectopic ACTH production: 10%, from lung small cell carcinoma or carcinoid tumors

ACTH-Independent:
- Adrenal adenoma (10%)
- Adrenal carcinoma (5%)
- Cortical hyperplasia

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6
Q

What is the adrenal gland change seen in Crushing Syndrome from exogenous steroid use?

A

cortical atrophy
- ↓ ACTH → shrinkage of cortex

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7
Q

What is the adrenal gland change seen in Crushing Syndrome from endogenous ACTH dependent types?

A

Diffuse Hyperplasia
- Both adrenal glands are enlarged (up to 30g) especially in zona fasciculata and zona reticularis

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8
Q

What are the types of Adrenal Cortical Neoplasms and their general features?

A

adenomas and carcinomas
- Most common in ages 30–40
- Carcinomas more common in children
- Mostly sporadic
- may be linked to: Li-Fraumeni Syndrome (germline p53) or Beckwith-Wiedemann Syndrome (gigantism, organ overgrowth)

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9
Q

What are the specific features of Adrenal Cortical Adenomas?

A
  • Clinically silent; often found incidentally
  • Small (<30g, <2.5 cm), well-encapsulated, nodular
  • Yellow cut surface (due to lipids)
  • Made of normal adrenal cortex cells
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10
Q

What are the specific features of Adrenal Cortical Carcinomas?

A
  • Often functional (hormone-producing)
  • Large (>20 cm), invasive
  • Disrupt normal adrenal structure
  • Cut surface shows necrosis, hemorrhage, cysts
  • Invades adrenal vein, vena cava, lymphatics
  • Distant metastases are common
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11
Q

What are the clinical features and symptoms of Cushing Syndrome?

A
  • Develops gradually
  • Early signs: Weight gain, hypertension, Fat redistribution: central obesity, moon facies, buffalo hump
  • Muscle weakness, especially proximally
  • Glucose intolerance, osteoporosis
  • Skin changes: thinning, bruising, poor wound healing, abdominal striae
  • Increased infections
  • Hirsutism, menstrual issues in women
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12
Q

How is Crushing Syndrome diagnosed?

A
  • ↑ 24-hour urine free cortisol
  • Loss of diurnal cortisol rhythm
  • Dexamethasone suppression test: measures ACTH and steroid levels after dexamethasone administration
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13
Q

What is Hyperaldosteronism and what are the types?

A

Chronic excess secretion of aldosterone
- Primary Hyperaldosteronism (feedback ↓ plasma renin)
- Secondary Hyperaldosteronism (activation of RAAS → ↑ aldosterone + ↑ plasma renin)

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14
Q

What are the main causes of Primary Hyperaldosteronism?

A
  • Idiopathic bilateral adrenal hyperplasia (60%)
  • Aldosterone-producing adenoma (35%): Conn syndrome
  • Glucocorticoid-remediable hyperaldosteronism (rare)
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15
Q

What causes Secondary Hyperaldosteronism and what are the outcomes?

A

↓ renal perfusion leads to:
- Congestive heart failure
- Cirrhosis
- Nephrotic syndrome
- Pregnancy

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16
Q

What are the main clinical features of both primary and secondary Hyperaldosteronism?

A
  • hypertension
  • hypokalemia
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17
Q

How are primary and secondary Hyperaldosteronism diagnosed and treated?

A

Measure aldosterone and plasma renin concentrations:
- Primary = ↑ aldosterone, ↓ renin
- Secondary = ↑ aldosterone, ↑ renin

Treatment
- Primary: Surgery (adenoma), drugs (aldosterone antagonists)
- Secondary: Treat underlying cause (e.g., CHF, cirrhosis)

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18
Q

What are Adrenogenital Syndromes and what are the main causes?

A

Disorders of sexual differentiation due to abnormal adrenal androgen production controlled by ACTH → stimulates adrenal androgens
- Congenital Adrenal Hyperplasia (CAH)
- Adrenocortical Neoplasms

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19
Q

What is Congenital Adrenal Hyperplasia (CAH) and what are key features?

A

Autosomal recessive, enzyme deficiency in cortical steroid synthesis
- 90% due to 21-hydroxylase deficiency
- More common in Hispanics and Ashkenazi Jews
- Suspect in newborns with ambiguous genitalia

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20
Q

What are the clinical consequences of Adrenogenital Syndromes?

A
  • Excess androgens → virilization or ambiguous genitalia
  • Block in cortisol synthesis → ↑ ACTH → adrenal hyperplasia
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21
Q

What are the types of Adrenocortical Insufficiencies?

Study These Flashcards
A
  1. Primary Hypoadrenalism (Adrenal cause)
    - acute: adrenal crisis
    - chronic: addisons disease
  2. Secondary Hypoadrenalism
22
Q

What is Acute Primary Hypoadrenalism triggered by and what are the causes?

Study These Flashcards
A

Triggered by stress or steroid withdrawal caused by:
- long-term steroid use with sudden stop
- Massive adrenal hemorrhage (e.g., Waterhouse-Friderichsen syndrome)
- Infection (e.g., Neisseria meningitidis)
- Trauma or prolonged birth in neonates

23
Q

What is Chronic Primary Hypoadrenalism and what is it caused by?

Study These Flashcards
A

Uncommon disorder; symptoms appear when 90% of adrenal cortex is destroyed caused by:
- Autoimmune adrenalitis (60–70%)
- Infections (fungal or TB)
- Metastatic cancer (lung, breast)
- Genetic disorders

24
Q

What are the clinical features of Addison’s Disease?

Study These Flashcards
A
  • Insidious onset
  • Weakness, fatigue
  • GI disturbances (nausea, vomiting)
  • ↓ Gluconeogenesis → low blood sugar
  • Electrolyte imbalance: Hyperkalemia, hyponatremia, hypotension
  • Skin hyperpigmentation (only in primary): ↑ ACTH → ↑ POMC → ↑ MSH
25
What is Secondary Hypoadrenalism and what is a common cause?
Pituitary/Hypothalamic origin causes ↓ ACTH → ↓ adrenal stimulation - Common with prolonged glucocorticoid therapy
26
What is Pheochromocytoma?
catecholamine-producing tumor derived from chromaffin cells (neural crest origin) that primarily arises in the adrenal medulla
27
What are the key characteristics of Pheochromocytomas?
~90% are benign; Rule of 10s: 10% are - extra-adrenal (paraganglia) - bilateral - malignant (metastasis = malignant) - not associated with hypertension
28
What are the genetic associations of Pheochromocytomas?
- Most cases (75%) are Sporadic - Familial (~25%): MEN 2A & 2B (RET mutation), younger ages, bilateral, higher malignancy risk
29
What is the morphology of Pheochromocytomas?
- Small nodules → large hemorrhagic masses (1g to 4000g; avg ~100g) - Yellow-tan cut surface; large tumors may have hemorrhage and necrosis - Polygonal to spindle-shaped cells - Classic zellballen pattern: nests of tumor cells surrounded by sustentacular cells and vasculature
30
What is the clinical course and symptoms of Pheochromocytomas?
Rare cause of surgically correctable hypertension --> seen in 90% of patients - Symptoms are often paroxysmal (episodic) due to bursts of catecholamines: - Headaches, palpitations, sweating - Paroxysmal hypertension - May also cause catecholamine-induced cardiomyopathy
31
What is used to diagnose Pheochromocytomas?
Biochemical Testing - ↑ Urinary or plasma free catecholamines and their metabolites: Metanephrines and Vanillylmandelic acid (VMA)
32
What is the treatment for Pheochromocytomas?
- Isolated Benign Tumors: surgical removal (definitive treatment) - Multifocal or unresectable: medical management for hypertension control (e.g., α-blockers)
33
What is a Neuroblastoma?
a malignant tumor of the sympathetic nervous system, occurring - mainly in children under 5 - mainly in the medulla - poorly differentiated neuroblasts and variable degrees of differentiation - Can spontaneously regress --> ganglioneuromas
34
What are the genetics associated with Neuroblastomas?
- N-myc oncogene overexpression = worse prognosis - FOXR2 activation contributes to tumor formation and proliferation
35
What are Pancreatic Endocrine Neoplasms?
Islet cell tumors = tumors of endocrine cells in the pancreas - only 2% of all pancreatic tumors - mainly in adults - Can occur anywhere along the pancreas - May also arise in tissues near the pancreas (peripancreatic).
36
What are the characteristics and major clinical syndromes of Pancreatic Endocrine Neoplasms?
Can be: - Single or multiple - Benign or malignant - Functional (secretes hormones) or nonfunctional 1. Hyperinsulinism 2. Hypergastrinemia & Zollinger-Ellison Syndrome 3. MEN (Multiple Endocrine Neoplasia)
37
What is the criteria for malignancy?
1. Metastases (spread to other organs) 2. Vascular invasion (tumor invading blood vessels) 3. Local infiltration (tumor invading nearby tissues)
38
What is an Insulinoma and what do they mainly look like?
most common pancreatic endocrine neoplasm most often found in the pancreas, generally benign and solitary - nodules are small, encapsulated, pale to red-brown located anywhere in the pancreas
39
What do Insulinomas present with clinically?
- excessive insulin secretion (80%) - only 20% have clinically significant hypoglycemia
40
What are the labratory findings and treatment for Insulinomas?
- high circulating insulin - high insulin to glucose ratio - treatment is surgical removal of the tumor
41
What is the Whipple Triad?
used to diagnose insulinoma: 1. Low Blood Glucose 2. Neuro Symptoms from Hypoglycemia 3. Rapid Relief with Glucose (eating)
42
What is Zollinger-Ellison Syndrome?
islet cell lesions (gastrinomas) characterized by hypersecretion of gastric acid and severe peptic ulceration in 90-95% of patients
43
What ways can ZES present and where does it mainly occur?
- sporadic and single tumors or multiple that occurs with MEN-1 syndrome (25%) - pancreas, duodenum, or peripancreatic tissue
44
What is Multiple Endocrine Neoplasia (MEN)?
group of genetically inherited diseases resulting in proliferative lesions (hyperplasia, adenomas, and carcinomas) of multiple endocrine organs.
45
What is MEN-1 / Wermer Syndrome?
rare heritable autosomal dominant disorder caused by germline mutations in the MEN1 tumor suppressor gene (menin) on chromosome 11q13
46
What are the 3 Ps of MEN-1 syndrome?
abnormalities involving Parathyroid, Pancreas, Pituitary - Primary Hyperparathyroidism - Pancreatic endocrine tumors - Pituitary tumors (anterior)
47
What is MEN-2?
familial medullary thyroid cancer involving the RET germline mutations - MEN2A, 2B/3
48
What is MEN-2A (Sipple Syndrome)?
Germline mutations in the RET proto-oncogene on chromosome 10q11.2 characterized by - pheochromocytoma - medullary carcinoma - parathyroid hyperplasia
49
What is MEN-2B/3?
Germline mutations in the RET proto-oncogene on chromosome 10q11.2 , distinct from the mutations seen in MEN-2A characterized by - medullary thyroid carcinomas - Pheochromocytomas - Neuromas or Ganglioneuromas - Marfanoid habitus
50
What is Familial Medullary Thyroid Cancer?
Variant of MEN-2A with a strong predisposition to medullary thyroid cancer, but not the other clinical manifestations of MEN-2A or MEN-2B (3) - older ages at diagnosis -Indolent course

Systems Exam 3 (18 decks)

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