Pheochromocytoma

Pheochromocytomas are rare and usually non-cancerous adrenaline-producing tumors that develop in the core (medulla) of the adrenal gland. Adrenal glands are located just above each kidney and are part of the endocrine system. One function of the adrenal glands is to produce hormones called catecholamines, such as epinephrine and norepinephrine. These hormones prepare the body for the “Fight or Flight” response, such as the reaction to a stressful situation. Pheochromocytomas in the adrenal glands overproduce these catecholamines, which can result in episodic high blood pressure and attacks of headaches, sweating, palpitations, fainting spells, and other symptoms. If untreated, this tumor can be life threatening due to the overproduction of these hormones. A high level of catecholamines can cause life-threatening health problems, such as a stroke or heart attack. 

Benign pheochromocytomas do not spread. In cases where the pheochromocytoma is cancerous, there may be a risk of metastasis throughout the body. There are about 1,000 new cases of pheochromocytomas in the United States each year. Pheochromocytomas most commonly develop in middle-aged people, but they can develop at any age. These tumors are generally referred to as the “10 percent tumor” because the following was thought to be true: 
  • 10 percent are bilateral (both adrenal glands) 
  • 10 percent are found in children 
  • 10 percent are genetic 
  • 10 percent are cancerous 
  • 10 percent are found outside of the adrenal glands (paragangliomas) 
Today, as we learn more about pheochromocytomas, we know that the 10 percent rule is not necessarily accurate. For example, 25 percent are thought to be genetic. 

At Yale Endocrine Surgery, we offer surgical treatment and management options for pheochromocytomas. In addition to being well versed with the traditional and classic techniques and surgeries, we can offer procedures offered by our innovative surgeons who are at the leading edge of endocrine and minimally invasive surgery. Our multidisciplinary team, which includes world-renowned surgeons and endocrinologists, works collectively to create personalized treatment plans that provide the best options for each patient, reflecting his or her specific condition and individual needs.

Causes of Pheochromocytomas

There is no known reason why tumors develop in the adrenal glands. Most pheochromocytomas arise spontaneously. Pheochromocytomas develop from special cells, called chromaffin cells, in the core of the adrenal gland. 

Generally, these tumors tend to grow in only one adrenal gland, though in some cases both adrenal glands may develop tumors. 

Chromaffin cells can be found on nerve tissue throughout the body, so it is possible for pheochromocytomas sometimes to develop outside the adrenal glands. The most common locations outside the adrenal glands are the heart, neck, bladder, back wall of the abdomen, and along the spine.

Risk Factors

A risk factor is anything that increases a person's chance of getting a disease. Different diseases have different risk factors. Some risk factors can be controlled with lifestyle changes. Other risk factors cannot be changed.

Pheochromocytomas may run in families or tend to be associated with certain genetic conditions. These conditions include: 
  • Von Hippel-Lindau disease 
  • Multiple endocrine neoplasia, type II (or MEN II) 
  • Neurofibromatosis 1 (or NF1). 
Having a genetic risk factor does not mean that you will develop a pheochromocytoma. Understanding your risk factors will help you determine, what, if any, precautions and possible screening options you should consider.

Symptoms

In 40 percent of all cases, patients with pheochromocytomas have classic symptoms, while in 10 percent of cases a patient may present in “pheo crisis.” Another 50 percent of cases have no symptoms. Of the asymptomatic patients, the tumors are found accidentally in approximately 40 percent of the cases; in 10 percent of the cases, they are found during a screening for other genetic disorders. 

Classic symptoms of a pheochromocytoma are produced by the oversecretion of catecholamines. These symptoms include: 
  • Episodic (once in a while) or continuous high blood pressure. High blood pressure is the most common symptom, but having high blood pressure does not mean that you have a pheochromocytoma. 
  • The classic “triad” of headaches, palpitations, and cold sweating 
  • Difficulty breathing 
  • Weakness 
  • Panic attacks
Less common classic symptoms include: 
  • Pale skin 
  • Low blood pressure 
  • Blurred vision 
  • Weight loss 
  • Increased thirst and urination 
  • Constipation 
  • Abdominal pain 
  • Elevated blood sugar and elevated while blood cell counts 
  • Psychiatric disturbances 
  • Heart muscle dysfunction 
  • Elevated red blood cell count 
When a patient presents in “pheo crisis,” it means that they are experiencing life-threatening symptoms due to the release of excessive and uncontrolled amounts of adrenaline. This state can lead to stroke, heart attack, organ failure, coma, and death. 

Additionally, there is a condition called hypertensive crisis associated with pheochromocytomas. Hypertensive crisis is an attack of high blood pressure that usually lasts less than an hour.

Hypertensive crisis (and symptoms) may be triggered by emotional distress, anesthesia, undergoing radiographic imaging with intravenous contrast media, or physical activities or changes that press on the tumor, including:
  • Changing body position 
  • Exercise 
  • Pregnancy 
  • Lifting 
  • Bowel movements 
  • Urination

If you have one or more of the above symptoms, it does not mean that you have a pheochromocytoma. If you think that you may have the condition, please call your doctor.

Treatment

In the majority of cases, surgery to remove the affected adrenal gland, known as an adrenalectomy, is the preferred treatment option for pheochromocytomas. Removing the affected adrenal gland generally reverses any symptoms caused by the tumor. Prior to undergoing surgery, patients will need to take temporary medications to block the effects of the catecholamines, such that the surgery can be performed safely. If both adrenal glands need to be removed, then patients will need to take medications lifelong to replace the hormones that would normally be excreted by the adrenal glands. 

Surgical Treatment
Adrenalectomy
In a traditional open adrenalectomy, an incision is made over the abdomen or side in order to provide direct access to the adrenal gland. A laparoscopic adrenalectomy requires smaller incisions. During a laparoscopic procedure, three to four half-inch incisions are made, and surgeons use a camera for visual guidance and special instruments to remove the adrenal gland or glands. With a laparoscopic adrenalectomy, most patients go home within 24 to 72 hours and experience less postoperative discomfort and a shorter recovery time than patients who have a traditional adrenalectomy. 

The majority of our adrenalectomies are performed using laparoscopic methods. 

At Yale Endocrine Surgery, our multidisciplinary team is highly experienced in the surgical treatment of pheochromocytomas. Our surgeons are specially trained in both traditional and laparoscopic techniques. Before any procedure, our surgeons will answer all your questions and address all your concerns so that you feel comfortable with what to expect. 

Non-Surgical Treatment 
Some patients may not be able to have their pheochromocytomas treated surgically because of the way the tumor is growing or if it is cancerous and has spread to other parts of the body. In these cases, the patient should work with their endocrinologist and surgeon to determine the best course of treatment.