Pheochromocytoma: Not your everyday diagnosis

Looking scared, Joan Ware, age 46, comes to the emergency department (ED) for the third time in the last 2 months. She says she just doesn’t understand what’s wrong; she’s always been healthy. Her symptoms, she says, begin suddenly with a headache, sweating, and palpitations. Then come the attacks in which she experiences severe fright, a feeling of doom, chest tightness, and light-headedness. The episodes are sporadic and distressing.
At her last ED visit, a physician referred her to a psychiatrist. She tried to explain that she wasn’t having anxiety attacks—and that she wasn’t crazy. Her blood pressure was 160/110, and her heart rate was 160 beats/minute. Her electrocardiogram showed sinus tachycardia. Her laboratory test results were normal.
What Ms. Ware doesn’t yet know is that she has a rare, lethal tumor called pheochromocytoma.

What is pheochromocytoma?
Pheochromocytoma is a tumor that comes from the chromaffin cells of the adrenal medulla and secretes catecholamines. Tumors can appear anywhere along the sympathetic chain, but 85% are in the adrenal gland. About 1 in 1,000 people with high blood pressure has a pheo­chromocytoma. The cause is unknown. Tumors may appear at any age, but most commonly, they appear in the fourth and fifth decades of life.
The secreted catecholamines may bind to:
•      alpha-adrenergic receptors, causing vasoconstriction
•      beta1-adrenergic receptors, causing inotropic effects
•      beta2-adrenergic receptors, causing vasodilation.
Thus, norepinephrine-dominant pheochromocytomas increase systolic and diastolic blood pressure from alpha receptor stimulation, and epinephrine-secreting tumors can cause hypotension from beta2 stimulation.
Tumors can cause wild swings in blood pressure. In paroxysmal and sustained hypertension, blood pressure may be dangerously high. Such hypertension may result in target organ disease, such as left ventricular hyper­trophy, retinopathy, encephalopathy, and proteinuria. Signs and symptoms typically occur weekly, but may occur several times a day or every few months.
Other signs and symptoms include headache, sweating, palpitations, chest pain, hot flashes, and psychological symptoms, such as anxiety, despair, and even aggression. Rarely, a pheo­chromocytoma is asymptomatic. Complications of pheochromocytomas include pulmonary edema, cardiac arrhythmias, myocarditis, dilated congestive cardiomyopathy, cerebral vascular accidents, and renal failure. Without effective treatment, the complications will almost inevitably be fatal.

Uncovering diagnostic clues
On Ms. Ware’s third visit to the ED, a nurse’s careful history began with this open-ended statement, “Tell me your story.” As the patient spoke, the nurse considered how the signs and symptoms were connected. Then, she remembered that years ago, she had a patient with similar signs and symptoms, who was diagnosed with a pheochromocytoma.
Ms. Ware’s story provided many clues, including the fact that her signs and symptoms were precipitated by exertion and anxiety. As the nurse knew, pheochromocytoma attacks can also be triggered by foods containing tyramine, certain drugs, abdominal pressure, trauma, pain, anesthesia, intubation, chemotherapy, bladder distention, and micturition. Her knowledge, willingness to listen to the patient, detective work, and astute clinical decision making led her to the possibility that Ms. Ware had a pheo­chro­mo­cytoma.
The nurse discussed her suspicions with the physician, who then ordered a 24-hour urine collection. After Ms. Ware’s blood pressure was stabilized with an alpha blocker, she left the ED with a big plastic urine jug and the hope that she would find out what was happening to her.

Zeroing in on pheochromocytoma
Diagnosing a pheochromocytoma requires measuring plasma-free meta­-
­n­ephrine and catecholamine levels and analyzing a 24-hour urine specimen for fractionated metanephrine, catecholamines, total metanephrine, and vanillymandelic acid levels. The first two urine tests have good sensitivity; the latter two have good specificity.
Analysis of Ms. Ware’s 24-hour urine specimen revealed excessive catecholamine levels. The next step was to find the tumor, so the physician ordered magnetic resonance imaging (MRI) of her abdomen, and the radiologist ordered whole-body scintigraphy using metaiodobenzylguanidine (MIBG). The MRI revealed a mass on her left adrenal gland, and the MIBG confirmed that it was a pheochromocytoma and revealed no extra-adrenal tumors.
Ms. Ware’s physician told her that the tumor could be removed surgically and explained that surgery is the preferred treatment. Without it, the tumor could cause dangerous complications down the road.

Treating pheochromocytoma
For 10 days before surgery, Ms. Ware took a selective postsynaptic alpha1 blocker to reduce her blood pressure and prevent intraoperative hypertensive crises. The 10-day course allows the blood volume to expand. A non­-selective alpha blocker such as phenoxybenzamine (Dibenzyline) may be prescribed to normalize blood pressure, but this type of drug can cause orthostatic hypotension and reflex tachycardia and may increase postsurgical complications. Selective postsynaptic alpha1 blockers eliminate these problems. Also to expand volume, Ms. Ware added a liberal amount of salt to her diet, as instructed by her nurse.
Surgical removal of a pheochromocytoma is a high-risk procedure. During surgery, the patient’s heart rhythm and vital signs are monitored, using an arterial line. Hypertensive episodes during surgery are treated with an I.V. infusion of phentolamine (Regitine) or nitroprusside (Nipride). Lidocaine or esmolol (Brevibloc) are used for cardiac arrhythmias.
Today, most tumors are removed laparoscopically, unless they are large, or there are lots of them. In Ms. Ware’s case, the surgeon was able to perform a laparoscopic adrenalectomy, which is less invasive and thus poses less risk than other procedures. Fortunately, her tumor was well defined, and the surgeon said it was benign.

Providing postoperative care
After surgery, your patient is at risk for hypotension from venous
dilation caused by the sudden withdrawal of catecholamines, diminished receptor sensitivity to cate­­cholamines, and the alpha blocker therapy. A postoperative hypotensive patient may need I.V. fluids, colloids, and vasopressors.
Because pheochromocytomas are highly vascular tumors, surgery causes significant blood loss and hypo­-volemia. By the time the patient is discharged, blood pressure usually has returned to normal. In Ms. Ware’s case, the surgeon successfully removed the tumor, and she didn’t have a hypertensive crisis during surgery or hypotension after it.
One to two weeks after surgery, a patient has follow-up biochemical assays to ensure that all catecholamine-producing tumor cells have been removed. Complete surgical resection of a benign pheochromocytoma usually means a normal life expectancy.


Following up with genetic counseling
For some types of pheochromocytoma, care includes genetic counseling. Between 20% and 30% of pheo­chromocytomas are familial and tend to be associated with other familial diseases, such as multiple endo­crine neoplasia type 2a, von Hippel-Lindau disease, and neurofibromatosis.
Some 10% to 50% of extra-adrenal tumors, called paragangliomas, are inherited and more common in children than in adults. These tumors lie near sympathetic and para­sympathetic ganglia in the abdomen and pelvis and occasionally in the chest or neck. About one-half of paragangliomas are malignant. Be sure to encourage any patient with a paraganglioma to follow the genetic counseling protocol.

Meeting the patient’s needs
A diagnosis of pheochromocytoma can be a relief to your patient, but facing surgery can still be frightening. Expect questions about whether the tumor will come back, how it got there in the first place, and whether or not it can be passed on to others.
Of course, the first thing a patient with a pheo­chromocytoma needs is a clinician who thinks beyond the usual causes of common signs and symptoms. Because of such thinking, Ms. Ware is doing well today and hasn’t had any frightening episodes since her surgery.
Despite all the technological advances in health care, saving a patient like Ms. Ware depends mainly on imaginative clinical decision making that leads to an accurate diagnosis and treatment for a deadly condition.

Visit AmericanNurseToday.com/journal for a list of selected references.

Katharyn F. Daub is Associate Professor and Chair at the University of Hawaii at Hilo.

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6 thoughts on “Pheochromocytoma: Not your everyday diagnosis”

  1. sandiesie says:

    I have a pheo on my right adrenal gland. I’m suppose to have surgery on the 23rd of Oct at Tripler. The surgeon nor staff has removed a pheo…trying to find someone on the island who has. My number is 808-457-7198

  2. Anonymous says:

    Interesting article. How is your brother doing?

  3. Roro says:

    I am doing some tests to rule out or confirm pheochromocytoma.. A bit scared.. Alrdy done with blood tets& stil have the urine 24 hour collection

  4. Anonymous says:

    I had a pheo removed in nov 2012, Iwas diagnois almost 8 months before surgery. I was on 22 b/p pills a day and my pressure was still spiking up to 260/140 make a long story short it was the worst time of my life, finally I found Dr Michael Grasso in Lenox hill hospital. Dr Grasso removed the my left adrenal glance and everything is fine today.But I went thourgh hell and back and if I can help someome out my name is Joe my number 973-3092590 please call if you think you have a pheo. Doctors have

  5. Anonymous says:

    I also had a pheo on my right adrenal gland. Had it for 2 years. Had bouts of BP when I had the episodes, but normal after and in between. On heart monitor 3 times, Cardio doctor said I needed a pacemaker, so implanted, missed the diagnosis, two weeks later, they removed gland and tumor. question is, do I really need this pacemaker, another doctor says no, Now Im stuck with it.
    Pheo’s are misdiagnosed 50% of the time according to some reported articles. Find an endo doctor if you can.

  6. Tehneka says:

    I’m a 17 year old female and am currently being tested for a pheochromocytoma and for it to be ruled out. I have been really sick for 7 months.. Some symptoms that I’ve had for years but never really thought much of it until I got sick and others had only arised the 7 months ago and along the way. I live in Australia and came across this site, even though it’s American I thought this can help so I would love replies, advice and things like that. Okay, 7 months ago the first day the 1st symptom happened. I was in bed and woke up to what I had though was a shiver (when you get cold and your teeth chatter) I wasn’t cold though, I had a high temperature. I ended up going back to bed hoping it would go away, it didn’t. It last almost a week, my mum took me to the doctors the 3rd day I think and all he said was I think you’re getting a bug, you will start getting worse before you get better just take panadol and whatnot. At the time we were like okay… I actually did get sick and that lasted a couple of days, then the shiver (which was actually an essential tremor of the jaw) went away and I got better. 2 weeks later, I was on the bus on the way to school (it was a cold day and at the time I was cold) the tremor was coming back but I was unsure because I was cold. The tremor I had the two weeks before this was a really really fast tremor. 2nd period my jaw was going crazy and so I called my mum and she said get a note so you can go home and I’ll take you to the hospital. I did that and as my teacher was writing my note my heart rate went up heaps, it was beating like crazy. I told my teacher and she took my heart rate it was almost 115 for my age it should be 50-60 I think. We got to the hospital and took me in straight away, they had first thought I was on ice or something but I don’t do drugs, smoke or drink. Anyway they still thought that for a few hours until the 2 drug tests they took came back obviously clean. I was on the drip, and magnesium I think it was called.. Which at that point I don’t remember much because of what they had me on. An hour or so later it had worn off, everything was the same.. My heart, my jaw, they ran loads and loads of blood tests, MRIs, scans, ultrasounds and even tests I’ve never heard of. I was in hospital for a week and 1 day.. When I left everything was still the same, they kept me in until my unexplained tachycardia was well enough for me to go home being in the high hundreds. Thoughout the 8 days being in hospital they found from tests I had a high adrenaline level and increased bserum normetadrenaline. And I had gotten more symptoms: obviously the tremor (which caused exrutiating pain) and unexplained tachycardia, dilated pupils, (few months before this 7 months I started feeling nuaseated after eating anything) since I was born, I have always been soo soo tired all the time, no matter how much sleep I get. Flushing of the face and palpitations of the knuckles.. Within these 7 months, I’ve had 3 lots of botox to stop the tremor which it did for a while, but everything else has stayed. No solution no nothing. 3-4 weeks ago my tremor has slowly stared coming back slowly with even more excruciating pain. When I was in hospital I was on 4-5 different medications and am now on 4 types of pain killers because none of them are working. Please help? 🙁

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