What is it?
Pheochromocytoma is a very rare type of tumor that arises from a specific type of cell called chromaffin cells. Chromaffin cells produce hormones called catecholamines which include epinephrine, norepinephrine, and dopamine. These hormones affect metabolism, blood pressure, and heart rate. They also regulate our body’s response to stress. Chromaffin cells are found in the adrenal glands and in groups of nerves in other areas of the body, called ganglia. The adrenal glands are two small organs that are located on top of both kidneys.
The term pheochromocytoma specifically refers to tumors that come from chromaffin cells in the adrenal glands. Usually, pheochromocytoma originates in only one of the adrenal glands, but it can develop in both adrenals at the same time. If the tumor arises from chromaffin cells outside of the adrenal glands, it is called a paraganglioma. Paragangliomas can grow in different locations in the body such as the other areas inside the abdomen, the chest, and even the neck.
Pheochromocytomas are usually noncancerous (benign) tumors but they often cause the adrenal glands to produce and secrete too many catecholamines. Excessive catecholamine levels can lead to high blood pressure and cause symptoms such as headaches, excessive sweating, and the feeling of your heart beating too strong or too fast, called palpitations.
Pheochromocytomas are usually diagnosed in adults, 30 to 50 years old but it can develop at any age. Sometimes it is a part of a larger disorder such as multiple endocrine neoplasia syndrome type 2 (MEN2), Von Hippel-Lindau syndrome, and Neurofibromatosis type 1. Only about 10% of pheochromocytoma is cancerous or malignant.
What are the signs and symptoms?
A common finding associated with pheochromocytoma is hypertension. The blood pressure elevation can be sustained/constant or it can occur intermittently as paroxysmal attacks. A patient with pheochromocytoma can also have normal blood pressure.
Pheochromocytoma can present with a variety of symptoms. The classic symptoms include the “triad” of headache, sweating, and palpitations. They usually occur as intermittent or paroxysmal attacks. Most patients will not have all three symptoms and some patients can be asymptomatic also. Other non-specific symptoms include abdominal pain, chest pain, flushing/pallor, nausea, constipation, generalized weakness, and weight loss. Patients can also present with anxiety and panic attacks. In some patients, there is elevation of blood sugar or the development of diabetes.
What are the risk factors and how is it diagnosed?
Patients with symptoms suggestive of pheochromocytoma, those with incidental finding of adrenal tumor seen in an imaging procedure like CT scan, and those with a strong family history of pheochromocytoma and paraganglioma should undergo evaluation.
Pheochromocytoma should also be considered among individuals who develop hypertension at a young age (< 35 years old) or those with hypertension that is very difficult to control and require multiple (> 4) antihypertensive medications.
Laboratory testing to detect the presence of excess catecholamines is done to confirm the diagnosis. Imaging procedures such as CT scan and MRI are used to locate and characterize the tumor.
How is it treated?
Surgical removal of the tumor is the treatment of choice. Other treatment options are available depending on individual needs and situations. In addition to tumor resection, blood pressure control and treatment of complications are also integral part of management. A multi-specialty team approach is often necessary to achieve best outcomes.
What are the complications?
Early recognition and treatment are very important. Although pheochromocytoma is very rare, failure to diagnose can lead to catastrophic outcomes. It can lead to cardiovascular complications such as fatal arrhythmias, heart failure, and myocardial infarction (heart attack). Uncontrolled hypertension can also result in stroke and cerebrovascular bleeding.
When to see an endocrinologist?
If you are experiencing signs and symptoms indicative of pheochromocytoma particularly its triad of headache, sweating and palpitations, and f your blood pressure is very hard to control needing at least 4 medications, contact your doctor for proper evaluation and treatment.