“ Rare catecholamine-secreting tumour, a type of paraganglioma (same as glomus tumours). More common in children, associated with syndromes ...
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Rare catecholamine-secreting tumour, a type of paraganglioma (same as glomus tumours). More common in children, associated with syndromes (Von Hippel-Lindau disease, multiple endocrine neoplasia type-2 and neurofibromatosis type 1).
Bilateral in up to a third, extra-adrenal in 10% (bladder and organ of Zuckerkandl—near aortic bifurcation). Beware biopsy, which may induce a hypertensive crisis!
CT
• Soft tissue adrenal mass, homogeneous enhancement, prone to haemorrhage (then appears heterogeneous)
• May have peripheral calcification
MRI
• Hyperintense on T2and (may give rise to a ‘light bulb’ appearance) , may have areas of T1 hyperintensity from previous haemorrhage
• Enhancement as for CT (however, flow voids may be apparent—salt and pepper appearance)
• T1WI + Gad: intense enhancement ▸ there is no significant SI loss with in-phase and out-of-phase imaging
NUCLEAR MEDICINE
• MIBG is most sensitive for the detection of extra-adrenal disease and metastases.
Urinary free catecholamine measurement aids diagnosis
Axial (a) and coronal (b) post-contrast computed tomography scan of the abdomen shows an enhancing soft tissue mass arising from the right adrenal. There is intense, increased activity on the iodine-123-meta-iodobenzylguanidine (MIBG) scan (c) in keeping with a pheochromocytoma.
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