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Phaeochromocytoma is a benign tumour that causes an overproduction of adrenal hormones. We look at how it can be treated.
In the other 10% of cases, it is found in small glands outside the adrenals. It may occur as a primary condition with no genetic cause – or it may be part of a medical problem that runs in families, such as Von Hippel Lindau syndrome. This, and a number of other conditions, can have a phaeochromocytoma as an associated feature.
During an attack of adrenaline release from the phaeochromocytoma they can become particularly severe and require urgent treatment. A surge of adrenaline can be life threatening, with blood pressure greatly elevated.
If the 24-hour urine test is positive, the consultant would arrange for the adrenal glands to be imaged, either by a CT or an MRI scan. The definitive test to diagnose this condition is a nuclear medicine scan called MIBG, involving injection of a tracer which is taken up by adrenal-producing tissues.
Approximately 10 per cent of these tumours are malignant, which adds to the importance of making a firm diagnosis.
In the vast majority of cases (90 per cent) only one side is affected. However, if both glands need to be removed, the patient will require adrenal hormone supplements for life
If the condition that caused the phaeochromocytoma was genetic, genetic counselling and longer term surveillance may be advised. Sometimes annual 24-hour urine tests are offered if there is considered to be a high risk of recurrence.