Adrenal Cortical Tumors

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Adrenal Cortex Anatomy & Tumor Intro - Gland Overview

  • Location: Bilateral, retroperitoneal, superomedial to kidneys.
  • Arterial Supply: Superior, middle, & inferior adrenal arteries.
  • Venous Drainage:

    ⭐ Left adrenal vein → left renal vein; Right adrenal vein → Inferior Vena Cava (IVC).

  • Cortex Layers & Hormones (Outer to Inner): 📌 GFR Mnemonic
    • Zona Glomerulosa: Mineralocorticoids (Aldosterone) → Salt
    • Zona Fasciculata: Glucocorticoids (Cortisol) → Sugar
    • Zona Reticularis: Androgens (DHEA) → Sex
  • Adrenal Tumors Overview:
    • Benign (e.g., adenoma) vs. Malignant (e.g., carcinoma).
    • Functional (hormone-secreting) vs. Non-functional.

Adrenal gland arterial supply and venous drainage

Functional Tumors (Cushing's, Conn's) - Hormone Havoc

Adrenal cortex overproduction: Cortisol (Cushing's) or Aldosterone (Conn's).

SyndromeKey FeaturesScreening TestsConfirmatory Tests
Cushing'sCentral obesity, moon facies, purple striae, HTN, hyperglycemia, weaknessLDDST (1mg): Cortisol >1.8 µg/dL; ↑24hr UFC; ↑Late-night salivary cortisolHDDST (8mg); CRH stimulation test
Conn'sHTN (resistant), hypokalemia (not always), metabolic alkalosis, fatigueARR >20-30 (PAC/PRA); PAC >15 ng/dL; Suppressed PRASaline infusion; Oral Na+ load; Fludrocortisone supp.
📌 Conn's: Think CONNecting HTN and Low K+ (Potassium).

Diagnostic Algorithm (Conn's Syndrome):

⭐ Most common cause of endogenous Cushing's syndrome is Cushing's disease (pituitary adenoma), not primary adrenal tumors.

Management: Unilateral adenoma: Surgery. Bilateral hyperplasia/non-surgical: Medical (e.g., spironolactone for Conn's, ketoconazole for Cushing's).

Adrenocortical Carcinoma (ACC) - Malignant Mayhem

  • Rare, aggressive; often >4-6 cm. Bimodal age: <5 yrs, 40s-50s.
  • Presentation:
    • Functional (~60%): Cushing's, virilization.
    • Non-functional (~40%): Abd pain/mass.
  • Diagnosis:
    • CT/MRI: Large (>4-6 cm), irregular, heterogeneous, necrosis, calcification, invasion; >10 HU (non-contrast).
    • Weiss Score: Histopathology; ≥3 criteria (mitoses, necrosis, invasion) = ACC.
  • Staging: ENSAT System.
    ENSAT StageDescription
    Stage ITumor ≤5 cm, confined to adrenal
    Stage IITumor >5 cm, confined to adrenal
    Stage IIILocal invasion (nodes, vein, adjacent organs)
    Stage IVDistant metastases
  • Management:
    • Surgery: Radical adrenalectomy (local).
    • Adjuvant: Mitotane (high risk).
    • Metastatic: Mitotane, chemo.
  • Prognosis: Poor; 5-yr survival 15-40%.

⭐ Mitotane is an adrenolytic agent used as adjuvant therapy in ACC and for metastatic disease.

Adrenal Incidentalomas - Surprise Masses

  • Adrenal mass >1 cm discovered incidentally. Prevalence: ~5%.
  • Essential Workup:
    • Exclude functionality:
      • Pheochromocytoma: Plasma free metanephrines or 24-hr urinary metanephrines/catecholamines.
      • Cushing's syndrome: 1mg Dexamethasone Suppression Test (DST); overnight or low-dose.
      • Primary aldosteronism (if hypertensive/hypokalemic): Aldosterone-Renin Ratio (ARR).
    • Assess malignant potential (Non-Contrast CT):
      • Benign features: Size <4 cm, smooth margins, homogenous, Hounsfield Units (HU) ≤10.
      • Suspicious/Malignant: Size >4-6 cm, irregular, heterogeneous, HU >10, growth on follow-up.
  • Management Algorithm:

⭐ Majority (70-80%) of adrenal incidentalomas are benign, non-hypersecreting adenomas.

High‑Yield Points - ⚡ Biggest Takeaways

  • Conn's syndrome (primary hyperaldosteronism): adenoma causing hypertension, hypokalemia. ↑Aldo:Renin ratio.
  • Cushing's syndrome (cortisol excess): adenoma/carcinoma causing central obesity, striae. Dexamethasone suppression test.
  • Adrenocortical carcinoma (ACC): Aggressive, often functional, typically >6 cm. Weiss criteria for malignancy.
  • Incidentalomas: Require hormonal workup. Resect if functional, malignant features, or >4-6 cm.
  • Laparoscopic adrenalectomy: Standard for most benign functional tumors and smaller ACCs (<6cm).

Practice Questions: Adrenal Cortical Tumors

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Flashcards: Adrenal Cortical Tumors

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Patients undergoing unilateral adrenalectomy typically require _____ post-op

exogenous steroids

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