Rare, presents when large and commonly (50%) associated with an endocrine syndrome (Li-Fraumeni syndrome or Beckwith-Wiedemann syndrome) . ...
Rare, presents when large and commonly (50%) associated with an endocrine syndrome (Li-Fraumeni syndrome or Beckwith-Wiedemann syndrome). More common in children.
Also linked to hemihypertrophy and astrocytomas.
Hormonally active—children present with virilisation/Cushing syndrome.
CT
• Central necrosis/haemorrhage typical.
• Delayed washout (like metastases).
• 30% calcify.
• Check the IVC and renal vein for thrombosis.
• Metastasises to the liver and lymph nodes.
POSITRON EMISSION TOMOGRAPHY (PET)/CT
• Useful for diagnosing ACC, recurrence and metastases
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