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OSPE: Refer histoloy of adrenal gland to identify the zones.

TUMOURS OF THE ADRENAL CORTEX

image Adenoma

image Adenocarcinoma

  1. Adenoma
  2. Adenocarcinoma (malignant)
  • Rare
  • Hormonal symptoms (depending on the hormone hypersecretion)
  • Virilization
  • Hyperaldosteronism, Cushing’s
  • Association with Li-Fraumeni syndrome (rare autosomal dominant hereditary disorder)
  • Carney complex (autosomal dominant)

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CT scan. White: bone

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MRI. Bone is not white.

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Adrenal Cortical Adenoma

  • ‘Incidentalomas’ – incidental finding in 4% of patient’s imaging.
  • Most common: primary hyperaldosteronism

Clinical features:

  • Depends on hormonal secretory status
  • Retroperitoneum structure: more space, less mass effect (more hormonal effect)
  • Most common: Aldosterone producing CONN’s syndrome
  • Follower by – > Cushing’s & virilization.

Macroscopic:

image

  • well-circumscribed
  • intra-adrenal (inside adrenal gland)
  • ‘testical’ appearance
  • rarely haemorrhagic/necrotic
  • aldosterone-secreting: bright yellow
  • Cushing’s: yellow –> tan
  • Lipofuscin deposition: black adenoma

Microscopic:

image image

  • well-circumscribed
  • recapitulates the cells of the adrenal cortex
  • clear cells with abundant intracytoplasmic lipid (zona fasciculata)
  • compact cells (zona glomerulosa)
  • intermediate cells
  • adjacent adrenal cortex: suppressed/atrophy
  • when treated: presence of spironolactone bodies

image spironolactone bodies.

CONN’s Syndrome (hyperaldosteronism)

image  yellow in colour

  • Hypertension (mild to moderate)
  • Hypokalemia (episodic) –> muscle weakness, cramping, arrhythmia
  • Headaches
  • Polydipsia, polyuria (not diagnostic)

Blood test done during episode.

  • Rarely caused by adrenal cortical carcinoma
  • Adenoma on CT not always Conn’s
  • Conn’s may be due to small nodules on CT

Therefore a blood test will be more diagnostic than CT.

Cushing’s

Cushing’s syndrome: disorder caused by high levels of cortisol
Cushing’s disease: disorder caused by high levels of ACTH secretion by pituitary gland.

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Adrenal Cortical Carcinoma

  • 3% of all endocrine neoplasm
  • more in females

Investigation:

  • CT scan
  • MRI
  • PET scan

Tumour usually quite large – more than 5cm.

image

  • Recapitulate normal endocrine cortex
  • Patternless
  • Degenerative changes (hemorrhage, necrosis)
  • Mitoses
  • Nuclear atypia
  • Invasion of capsure & large veins

Criteria for malignancy:

  • Tumour size – more than 5cm
  • Mitotic rate – more than 2/10 high power field
  • Presence of capsular/vascular invasion
  • MIB1/Ki-67 labelling index – 5-20%
  • NO STAGING SYSTEM (because rare)

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