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Pathology
Catecholamine secreting tumours of the adrenal medulla
Aetiology
Common in 3-4th decade
Can be sporadic (unilateral < 10cm)
Familial autosomal dominant: MEN-2, Chr-10, RET
Proto-oncogene mutation: Von Hippel-Lindau and Neurofibromatosis
Signs
Labile hypertension, postural hypotension, features of cardiac failure, tremor
Symptoms
Sweating, flushing, pallor, pyrexia, headache, palpitations
Investigations
Bloods: Elevated 24hr catecholamines, calcium (MEN), serum calcitonin
(medullary thyroid carcinoma)
Examination: Fundoscopy for retinal angiomas (VHL)
Imaging: MRI/CT adrenals, radionuclide imaging
Treatment
Medical: Alpha blockade followed by beta-blockade for reflex tachycardia. Surgery: Adrenalectomy +/- adjuvant treatment
Prognosis
Hypertensive is cured in ~ 75% with surgery.
5 yr survival ~44% for metastatic disease
Hypertensive crisis – if beta blockers started without adequate alpha blockade, end organ damage from hypertension, operative mortality<2%
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Key References
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