Phaeochromocytoma is one of the important tumors of the adrenal, because if left untreated can result in uncontrolled hypertensive crisis and can be lethal. They secrete a hormone known as adrenaline/ noradrenaline and metanephrine. Which maintains the blood pressure of the body by acting on the blood vessels and resulting their contraction and maintains the body fluid equilibrium. Very high level of this hormones can lead to unchecked hypertensive crisis which can be very detrimental to the health or the life. They are usually not cancerous but may be associated with many other tumor of thyroid, pancreas.
Adrenal glands are a pair of endocrine glands situated just above the kidney. They secrete many hormones, which maintain the salt, sweet and sex milieu of the body. The cortex of adrenal (outer half) secrets aldosterone and cortisol, which maintains the body equilibrium of salt (electrolyte), glucose and fluid of the body. The medulla (inner half) secrets metanephrines, which maintains the blood pressure of the body.
Phaeochromocytoma is can be associated with 1% of young hepertensive patient which can be corrected surgically. The incidence is same in male and female and can be seen at any age but most commonly seen in 20-40 yrs of age. One-third of the patient can present with tumor of other organs such as thyroid, parathyroid or pancreas. This syndromic presentation is more common in children and includes multiple endocrine neoplasia type 2, Von Hipple-Lindau syndrome, neurofibromatosis and hereditary paraganglioma syndrome.
The classic triad of phaeochromocytoma is headache, profuse sweating and palpitations. 15-20% of patient can have normal blood pressure or episodic rise in blood pressure. Other symptoms includes anxiety, chest pain, abdominal pain, fatigue, weight loss, vision problems, feeling of impending doom, panic attacks and seizures.
Hypertensive episodes can be triggered by trivial physical, emotional or pharmacological stimuli which includes bending over, pressure on the abdomen, fear, tobacco, histamine and glucagons, or foods rich in tyramine (e.g., beer, wine and cheese).
Patient with following criteria should be screened for hypertension:
Once phaeochromocytoma is suspected 24 hour urinary VMA/ Metanephrine and a serum metanephrine is done. To lateralize or locate the tumor a CT scan or MRI. In patient with extraadrenal phaeochromocytoma or recurrent or multiple phaeochromocytoma a meta-iodobenzylguanidine (MIBG) scanning or DOTA pet Scan is done.
Laparoscopic / Robotic surgical removal of the tumor is the standard treatment for pheochromocytoma if safe and feasible. Before the surgery patient is prepared to prevent any hypertensive crisis intraoperative by giving some medications. The preparation may take up to 2 weeks and may require hospitalization for the same. Once the surgeon and the endocrinologist are satisfied that the patient is adequately prepared for the surgery patient is schedule for the tumor removal.
Persistent Hypertension can be present in about 25% of patients after the removal of pheochromocytoma this is because the pathophysiological changes that occur because of the long ter hypertension. Patient needs to be under long term follow up as recurrent or metastatic diseases are known. The five-year survival rate after removal of benign pheochromocytoma is around 84 to 96 percent.