Phaeochromocytoma

Pathology:                    Catecholamine secretingtumours of the adrenal medulla

Aetiology:                    Commonin 3-4^th decade

Can be sporadic (unilateral < 10cm)

Familial autosomal dominant: MEN-2, Chr-10, RET

Proto-oncogene mutation: Von Hippel-Lindau andNeurofibromatosis

Symptoms:                   Sweating, flushing, pallor,pyrexia, headache, palpitations

Signs:                              Labile hypertension,postural hypotension, features of cardiac failure, tremor

Investigations:          Bloods:Elevated 24hr catecholamines, calcium (MEN), serum calcitonin

 (medullary thyroid carcinoma)

                                        Examination: Fundoscopy forretinal angiomas (VHL)

Imaging: MRI/CTadrenals, radionuclide imaging

Treatment:                 Medical: Alphablockade followed by beta-blockade for reflex tachycardia. Surgery: Adrenalectomy +/- adjuvant treatment

Complications:          Hypertensive crisis – if beta blockersstarted without adequate alpha blockade, end organ damage from hypertension,operative mortality<2%

Prognosis:                    Hypertensive is cured in ~ 75%with surgery.

5 yr survival ~44% for metastatic disease

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