Adrenal Cortical Tumors

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.

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

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

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 Stage	Description
  Stage I	Tumor ≤5 cm, confined to adrenal
  Stage II	Tumor >5 cm, confined to adrenal
  Stage III	Local invasion (nodes, vein, adjacent organs)
  Stage IV	Distant 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).