Adrenocortical carcinoma
Core idea
Aggressive primary adrenal cortical malignancy, often large, heterogeneous, and frequently functioning (cortisol/androgen excess).
Demographics & Risks
- Bimodal: children <5 yrs and adults ~40–50 yrs.
- Most cases are sporadic; <10% syndromic.
- Syndromes with increased risk:
- Li–Fraumeni (TP53) – highest-yield association
- Beckwith–Wiedemann syndrome
- MEN1
- APC-related / FAP (incl. Gardner variant)
- Carney complex
Clinical features
- ~60% functioning tumours →
- Cushing’s (most common)
- Virilisation (androgen excess)
- Mixed cortisol/androgen excess
- Rapid-onset endocrine syndrome + large adrenal mass → think ACC until proven otherwise.
Imaging Features
CT/MRI
- Large heterogeneous adrenal mass (often >6 cm, big fucking angry mass) at the time of presentation.
- Central necrosis / haemorrhage, irregular margins; may contain coarse calcification.
- Lipid-poor: high non-contrast attenuation; low washout, unlike benign adenoma.
- Local invasion of adjacent organs possible.
- Vascular invasion: renal vein and IVC tumour thrombus are classic.
- Lymphatic metastasis often present at diagnosis.
Functional Imaging
- FDG PET: typically very avid.
- Metastatic pattern: lung, liver, nodes.
Differentials
For giant adrenal mass
- Adrenal metastasis
- Usually bilateral
- History of known primary.
- Phaeochromocytoma
- More avid enhancement
- “Lightbulb” T2, may be cystic/haemorrhagic
- Often functioning with catecholamine symptoms.
- Adrenal lymphoma
- Homogeneous soft-tissue mass, little necrosis.
- Often bilateral.
