The first successful removal of a phaeochromocytoma was almost 75 years ago in 1926 by Dr Charles Mayo. Surgical excision is still the treatment of choice, and for non-malignant tumours the 5-year survival rate is 95%. The operation is complicated by the dangers of both hypertension and hypotension, and must be performed by an experienced surgeon with expert anaesthesia (see section on "special anaesthetic management for patients with phaeochromocytoma").
There is no effective medical treatment, but adrenalectomy should not be undertaken without adequate preparation of the patient. The introduction of alpha-adrenergic blockade has reduced the perioperative mortality from 13-45% down to 0-3%.
Blood pressure should be controlled for at least 2 weeks prior to surgery, usually with phenoxybenzamine, starting at a dose of 20-60mg per day in divided doses. This is increased gradually until hypertension is controlled, postural hypotension becomes a problem or the maximum dose of 160mg per day is reached. If tachycardia becomes a problem, then beta-blockade can be introduced, but only after adequate alpha-blockade has been achieved. Despite traditional wisdom Mr Lynn has found that beta-blockade is often not necessary.The use of beta blockade may mask incomplete alpha blockage and result in critical intra-operative increases in systolic blood pressure. During the operation the patient must have central venous pressure monitoring and intra-arterial blood pressure monitoring.
The adrenalectomy can generally be achieved by laparoscopic (keyhole) surgery, except in the case of very large tumours or those suspected to be malignant, when open operation is safer. In fact laparoscopic operation is probably safer than open surgery for phaeochromocytoma as there tends to be much less manipulation of the gland prior to clipping of the adrenal vein.
To avoid the release of any catecholamines into the systemic circulation, the priority of the surgeon is to isolate the adrenal vein with as little manipulation of the gland as possible, to prevent release of a massive dose of catecholamines into the circulation and precipitation of a hypertensive crisis. Excellent communication between the anaesthetist and surgeon is needed to prevent sudden changes in blood pressure, either up (with gland manipulation) or down (after clamping of the adrenal vein).
As a result of surgery 75% can expect a normalisation of blood pressure with the remission of paroxysms. If the tumour is malignant and metastases are present then 123I-MIBG is used to visualise any additional tumours. Elevated catecholamine levels in the urine may also confirm their presence post-operatively.
In the case of malignant tumours the 5-year survival rate is 44%. It may prove too difficult to locate and remove all metastases, so medical therapy and radiotherapy are used. Alpha- and beta-blockers may be used, but the tyrosine hydroxylase inhibitor alpha-methylparatyrosine is more powerful in controlling symptoms. 131I-MIBG is used to treat the tumours, as it is selectively taken up and has proven effective in shrinking the metastatic tumours and helping catecholamine levels return to normal. The recommended dose of 5550MBq can be given safely up to a limit of 33750Mbq in total.
Although surgery has a high success rate, all patients should attend annual follow up appointments to have urinary catecholamine levels checked so that any further tumours may be detected.
The combination of phaeochromocytoma and pregnancy can be lethal for both mother and baby. It is usually diagnosed when the mother is noted to be hypertensive during antenatal care, confirmed by urinary catecholamines and MRI scanning (which is safe in pregnant women). CT and MIBG scanning is not possible during pregnancy.
Alpha blockade can be given and successfully control the symptoms and risks without harming the foetus. Our view is that mothers (who have been fully alpha-blocked) should be taken to term, and delivered by caesarean section, without any attempt made to remove the tumour simultaneously. Further radiological investigations, such as CT and MIBG scanning, can be undertaken without risk to the baby, while the vascularity of the abdomen is allowed to settle over the next six to eight weeks. This gives the mother the opportunity to undergo laparoscopic resection of the phaechromocytoma, rather than having to have an open procedure at the same time as the delivery.