Tumours of the Adrenal Gland –Adrenal Cortex

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

TUMOURS OF THE ADRENAL CORTEX

Adenoma

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)

CT scan. White: bone

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:

• 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:

• 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

spironolactone bodies.

CONN’s Syndrome (hyperaldosteronism)

  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.

• 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)