Presentation

Adreno-medullary tumours (phaeochromocytomas) tend to present at a younger age when associated with the MEN syndrome, whereas sporadic tumours tend to occur in older patients. Classical symptoms include paroxysmal headaches, palpitations, tremors, and sweating attacks. A few may be diagnosed incidentally and a rare, but occasional case may be diagnosed after a maternal death in an unrecognized MEN family. Intermittent, severe hypertension is a rare but classical presentation.

Patients often have a long history and these tumours can be difficult to diagnose. Many doctors will never see a patient with this condition, but a low threshold for investigation is appropriate.

Adrenocortical tumours have a more varied presentation. About 40% are functioning; the rest are non-functional and only present with pressure symptoms such as pain in the abdomen or flank. They may also present with metastases. Others secrete steroids including oestrogens, testosterone, aldosterone, or even combinations and, depending upon the predominant steroid produced, the symptoms will vary.

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