Adrenocortical Neoplasms

• If functional may be responsible for any of the various types of hyperadrenalism
• A functional adenoma is more likely to be associated with a hyperaldosteronism and Cushing syndrome whereas a carcinoma is more likely to cause virilisation
• Functional and non functional neoplasms cannot be distinguished morphologically

 

Morphology

• Most adrenocortical adenomas are clinically silent and are encountered as incidental findings
• Adenomas are generally small (<2.5cm) and well circumscribed, if they are functional they adjacent cortex is atrophied. If non-functional the adjacent cortex is normal
• On cut surface they are yellowish due to the presence of lipid and microscopically look like cells of normal cortex
• Adrenocortical carcinomas are rare and more likely to be functional compared with adenomas
• two rare inherited caused of adrenal cortical carcinoma are;
  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
• They are large and invasive, often greater than 20cm in diameter
• Typically poorly demarcated lesions containing areas of necrosis, haemorrhage and cystic change
• Invasion of the adrenal vein and inferior vena cava is common
• Metastases particularly of bronchogenic nature may go to the adrenal and be difficult to differentiate between them and a primary
• Advanced cancers have a strong tendency to invade the adrenal vein, vena cava and lymphatics
• Metastases to regional and periaortic nodes are common as is distant haematogenous spread to the lund in particular
• Bony mets are unusual

Median patient survival is 2 year