Dr. Pheo's Pheo Dr.

I have two criteria for entering doctors into this list: 1) great clinicians, and 2) clinical researchers with >=3 papers on pheo. Both criteria have to be met to be on this list. You will see that the list is pretty short. Pheo is a rare disease, and a doctor usually has to practice at a metropolitan area to see enough patients to become an expert. I need the readers to send me more names and I will check them and select those who meet my criteria. These doctors are mostly endocrinologists and I know personally quite a few of them. They are real experts on pheo.

The list is by no means comprehensive and will be updated frequently based on reader feedback. For example, at least one or two doctors in the list are semi-retired and I don’t know how long they will practice. In addition, if I have missed a great clinician who also does clinical research on pheo, please let me know.

California
Paul Fitzgerald, University of California San Francisco
Run Yu, University of California Los Angeles

Iowa
Thomas O'Dorisio, University of Iowa

Massachusetts
Robert Dluhy, Brighams & Womans Hospital

Michigan
Richard Auchus, University of Michigan

Minnesota
William Young, Mayo Clinic

Missouri
Ruth Decker, St. Lukes Hospital

Ohio
Charis Eng, Cleveland Clinic

Pennsylvania
Raymond Townsend, University of Pennsylvania

Texas
Camilo Jimenez, MD Anderson Cancer Center

DC
Karel Pacak, National Institute of Health
Constantine Stratakis, National Institute of Health

Canada
Shereen Ezzat, University of Toronto

UK
Ashley B. Grossman, St. Bartholomew’s Hospital

France
Pierre-Francois Plouin, Hopital Europeen Georges Pompidou

Germany
Hartmut Neumann, University of Freiburg
Graeme Eisenhofer, University of Dresden

Netherlands
Jacques Lenders, St Radboud University

Sweden
Barbro Eriksson, Uppsala University

China
Zhengpei Zeng, Peking Union Medical College

Japan
Yukio Hirata, Tokyo Medical and Dental University
Mitsuhide Naruse, Kyoto Medical Center

Australia
Bruce Robinson, Sir Charles Gairdner Hospital
Roderick Clifton-Bligh, Royal North Shore Hospital

Name a good pheo doctor

I am compiling a list of doctors and medical centers that are experienced in pheo. I hope readers will share their PERSONAL experience with their own doctors and medical centers that they feel do a great job with pheo. Please indicate only the doctor's name and state, and the medical center's name. No other information please. I don't want any physician ad here at all.

I would ask you to only list a doctor who actually diagnosed or treated you. Name the doctor or center as comments to this post. Please only list if you are a pheo patient yourself. I hope we can come up with an accurate list of good pheo doctors voted by patients.

I may or may not list the doctors or centers in my own list, depending on the investigative work I will do to check. I have my own angle: from a fellow physician.

Dr. Pheo

Pheo imaging: now you see it, now you don't

Today I will go over some intricacies of pheo imaging. The post is suggested by readers and I will answer some particular questions they have.

For most patients with real pheo, finding the tumor is rather straightforward. Most pheos are about 4-6 cm in diameter and have unique features. CT or MRI describes pheo well. An MIBG scan is then done to see whether there are other pheos lurking in the dark somewhere in the body. Unfortunately for some patients with real pheo and for a lot of patients who do not really have a pheo, imaging can be confusing.

First, for Peep, let's discuss how radiologists make a diagnosis. I work closely with quite a few great radiologists and begin to know their mindset. Radiologists are great at seeing things most other doctors don't see. But they will never be completely sure what the things are because many things can appear the same way. They need clinical information to make a judgment. So Peep, when your urine test results were very high, they will search your body from neck to pelvis trying to find something that might be pheo. A lymph node, or an uncommon normal variation of the shape of a normal organ, can be interpreted as potential pheo. Now the urine results are normal, radiologists will think you probably don't have pheo and take the same findings as what they normally are: lymph node or normal variations. So the same radiological finding in two patients can be read by radiologists as two different diseases. Think this way, the same red liquid in a fancy glass in an expensive restaurant probably is some great wine, while it must be melt lollipop if you see it on the pavement with a stick in the middle. Therefore, Peep, you may have some structures that are not exactly common but can be normal. What they are depends on the risk of your really having a pheo.

Second, for Dennis, let's discuss about MIBG scan. This is the single most misinterpreted imaging for pheo. MIBG scan itself is a great test. It is just some doctors do not know what to make of it. Technically, as Dennis points out, there is an I-131 MIBG, and there is an I-123 MIBG. In a nutshell, don't use the I-131 MIBG for diagnosis (it is used for treatment of pheo). Only use the I-123 MIBG which has a much better signal/background ratio. I-123 just gives much sharper pictures. Second, MIBG scan does not diagnose anyone with pheo, it just shows where the pheo is. As I discussed in a comment before, normal folks often have one adrenal taking more MIBG than the other but they don't have pheo. Nuclear medicine doctors, like radiologists (and any other doctors), will try to find a pheo if they are told there is really a pheo in a patient. If you use photoshop, Dennis, you will know that you can find something you like by adjusting the threshold, the contrast, or the color. If they know the patient has low risk, they will think a little spot might be just a fluke.

The key thing is communication between the endocrinologist and the radiologist. I used to routinely discuss with radiologists on every single case. Now that I know a little about pheo imaging, I still read every patient's images and I will call the radiologists when I feel something is not right. A pheo specialist should be a mini-radiologist on the adrenal gland, at least. My radiologist friends will call me as well if they feel something is not right.

Back to Dennis' point, the most likely place where endocrinologists and radiologists communicate to each other is an academic center with experience on pheo.

Dr. Pheo

Pheo and heart

Endocrine diseases seem to always affect the heart in some ways. Thyroid diseases certainly cause abnormal heart functions and diabetes causes atherosclerosis. The other day, I joked with a cardiologist that they should "steal one more disease from endocrinologists." This disease is pheochromocytoma.

The heart of a pheo patient is bombarded with catecholamines. Although most patients with pheo do not have obvious heart symptoms except for palpitation, ECG reveals subtle abnormalities in about half of the patients with pheo. In patients with large pheos, all kinds of severe heart problems can show up. I have seen myocardial infarction, congestive heart failure, and life-threatening arrhythmia in patients with pheo.

As patients with heart problems usually go to see cardiologists, and because pheo is so rare and not on the radar screen of cardiologists in the initial work-ups, delay in diagnosis is rather common. I am not here to say that all patients with common heart diseases should test for pheo. That would be impractical and common things are common. But if a relatively young patient without clear risk factors develops heart disease, pheo should be considered.

Surprisingly, little is known on why catecholamines cause heart damage. Another disease called Takotsubo cardiomyopathy has a lot in common with pheo. Takotsubo cardiomyopathy is a heart muscle diseases in patients with sudden and severe emotional or physical stress. If any colleagues have insights in heart diseases in pheo, please comment here.

The morals are: 1) if you have pheo but don't have heart symptoms, you should at least do an ECG to make sure you don't have a significant heart problem; 2) if you have heart problems and nobody knows why, consider pheo, at least as a long shot.

Dr. Pheo

Follow-up after pheochromocytoma resection

OK. Now you are diagnosed with pheochromocytoma, carefully prepared before resection, and operated by a great surgeon. Are you out of the loop yet? What shall you do next?

For patients with truly sporadic pheochromocytoma, 90% is the chance that they are cured. The remaining 10% may have recurrence or metastasis in the future. The problem is that the doctors can NOT predict who will be the lucky 90% and who will be the unlucky 10%. Therefore no patients should think that they are absolutely cured and all patients should have follow-up.

My own approach to patients with apparently sporadic pheochromocytoma is to measure plasma metanephrines 1 month after surgical resection to make sure there is no hidden tumor somewhere. Too early measurements can be false positive because I suspect based on my experience that there is some kind of depot of metanephrines in the body that is only depleted in a month. If the metanephrines are normal, I then measure them yearly or whenever they have symptoms suggestive of pheochromocytoma or when an adrenal or other mass is identified incidentally.

If a patient has familial form of pheochromocytoma or a gene mutation is identified, then the patient will have a much higher chance of recurrence or metastasis. Yearly plasma metanephrines are definitely needed. Imaging studies may be indicated depending on which form of familial pheochromocytoma or which gene mutations. Other components of the syndromes need to be screened; patients' family members need to be tested and carriers need to be regularly screened.

Now I have finished the basic topics on pheochromocytoma. I will write more on my personal experience and more interesting topics. If the contents of my blog are mostly based on my own experience and there is not much existing study on the topic, I will clearly state that at the beginning of the post. I welcome colleagues and patients to critique and to suggest. I will also just use "pheo", instead of the full name.

What to do if pheochromocytoma is diagnosed

This topic is suggested by Dennis.

First of all, making the diagnosis of pheochromocytoma is not a trivial decision. It means more imaging studies and the eventual surgical operation. The patient has to ask two questions: 1) whether the diagnosis is correct, and 2) if the diagnosis is correct, how to proceed with further workups and management.

The experience of the doctor who makes the diagnosis is probably the most critical indicator of whether the diagnosis is correct. The average physicians see few patients with pheochromocytoma in their entire professional life, and they make the diagnosis themselves for even fewer patients. A reasonable question the patient needs to ask the physician is "how many patients with pheochromocytoma have you taken care of?" Most doctors won't be offended by this question. If the doctor has seen fewer than 3 patients, the doctor's experience on pheochromocytoma is too little to make an accurate diagnosis.

The other factor is the doctor's practice environment. Doctors affiliated with an academic center with a large experience on pheochromocytoma have more resources on pheochromocytoma. They know physicians with more experience, they know which radiologists to refer the patients to, and they know which surgeons have the best reputation on pheochromocytoma resecton.

The best doctors on pheochromocytoma are those working in an academic center and doing clinical research on pheochromocytoma. These doctors deal with this disease as part of their career and they have accumulated a large experience on this tumor. They also usually have a program on systemically follow patients after surgical resection.

If the diagnosis is made by an experienced physician, the diagnosis is probably correct. If the diagnosis is made by an inexperienced physician, it may not be necessarily correct, and to have a second opinion from an experienced physician is very prudent. If the patient has the means, it is always a good idea to have a second opinion from another experienced physician even if the diagnosis was already made by an experienced physician, especially when the first physician was not very sure.

To select a surgeon follows the same logic. The best surgeons are those working in an academic center with a large experience on pheochromocytoma and they have operated on more than a few patients with pheochromocytoma. They have seen the tricky perioperative course and work closely with endocrinologists.

As Dennis puts it, a patient should not settle for mere convenience after diagnosed with pheochromocytoma for the first time. Seeking opinions from experienced physicians is very important.

Screening for mutations: in whom, and how?

Thank you again, Foxy, for asking an intriguing question on screening for gene mutations that cause pheochromocytoma. As this is a question of general interest, I put it as a new topic.

The chemicals a tumor produces do not tell much about whether the tumor is likely caused by a known mutation. Pheochromocytomas in MENII usually produce epinephrine, while those in VHL usually make norepinephrine. And in general, most pheochromocytomas tend to make norepinephrine, with or without mutations. Extra-adrenal pheochromocytomas (called paragangliomas) in familial paraganglioma (SDH mutations) and in Carney's triad (unknown gene mutations) may not produce catecholamines. Although these patterns of catecholamine secretion exist, they do not help in determining whether a patient should be screened.

Neither does tumor size or patient sex.

Some other clinical parameters do help. 1) Positive family history certainly suggests familial pheochromocytoma. 2) Age. The younger (<50), the more likely a patient has mutations causing pheochromocytoma. 3) Multifocal pheochromocytomas suggest mutations. 4) Signs of genetic syndromes. For example, a patient may already have meduallary thyroid carcinoma. If a patient has any of these, then genetic testing has higher yield.

Should any patient with pheochromocytoma be screened? At last year's International Symposium on Pheochromocytoma (ISP 2008, Cambridge, UK) which I attended, this topic was lively debated and the arguments were divided at the Atlantic Ocean. The European doctors seem to root for testing everyone as the tests are covered by either insurance or research centers. American doctors are more hesitant. No insurance companies in America I know cover genetic testing of pheochromocytoma in a patient without family history. A few American centers do offer testing in a research setting but the patients have to enter a trial of some sort. It can cost a patient thousands of dollars to do all the commercially available tests.

The other issue is which gene(s) to test first. Six (6) genes are known to cause pheochromocytoma: RET, VHL, NF1, SDHB, SDHD, and SDHC. Clinical history provides some help. Bilateral pheos: RET and VHL first. Extra-adrenal pheos: SDHB, SDHD, and VHL first. The hit rate is not very high even with these clinical history-based strategy. Above all, only 30% of patients without family history really have a mutation known to cause pheochromocytoma.

In my own practice, I highly encourage patients with family history to test for mutations. In those without family history, I explain the above and let them decide. Most patients without family history elect not to do genetic testing. They are followed clinically and by tests and imaging. It is unfortunate that genetic testing even in patients with family history are still not covered by insurance in many instances.

Suggestions on posting questions

As readers begin to post comments or questions, I note that it may be hard for me to find questions posted under earlier topics. I would like to ask readers to post specific questions on pheochromocytoma under the most recent topic, whatever it is. For example, if you want to ask about interpretation of a specific chromogranin A test, please post the question under this topic simply because it is the most recent one and the question is easier for me to find.

Please still post comments or editorial questions under the specific topic you like to comment on.

I am not really savvy on the technical aspects of blogging. If there are better ways, please let me know.

Thanks.

Dr. Pheo

Doctor-patient interaction: in response to Foxy's question

Thank you, Foxy, for your comments and question. You have just touched upon an important but overlooked issue in physician-patient relationship. You astutely observe that some doctors can be nervous and defensive when they are seeing patients with rare diseases (including PHEOCHROMOCYTOMA) that they know little about. There is no simple solution to the problem but I can provide some background here so that we can think of creative ways to solve the problem.


Physicians are used to be viewed as knowledgeable experts. They are supposed to help patients, to guide, and to cure. Many physicians also have a large ego as well and enjoy the role of a healer and its associated prestige. When they see a patient who suffers from a disease they know almost nothing, all the pretext of their psyche suddenly does not exist any more. A good number of physicians take this as an opportunity to learn with the patient and acknowledge their ignorance and willingness to learn, while some others will act out and use defensiveness to hide their embarrassment.

Although I don't think patients are to blamed for the problem, patients are indeed also part of the issue. Patients sometimes unknowingly encourage bad physician behaviors. For example, some patients may take arrogance as confidence of a physician. Some patients like black-and-white decisions and take discussion of nuances as indecisiveness of a physician.

At the end of the day, I guess the physician-patient relationship is like any other relationships--you relate with the one you like, and you separate from the one you dislike, and face the consequences.


Finding a good physician should aim at different goals at different disease stages. For definitive diagnosis, you need an expert on pheochromocytoma even though the expert may not be a perfect lady/gentleman. For surgical resection, you need an expert surgeon who has many cases before yours. For follow up, the character of the doctor is more important than knowledge and above all, you may not have a local expert in the first place.

As many of my own patients are from out-of-town, they often ask me how to pick a local doctor to coordinate the care for pheochromocytoma. The advice I give to my own patients is the follows: 1) you trust and like the doctor, 2) the doctor has an open mind on diseases she/he is not familiar with. The specialty of the doctor is not so important.


Let's not even start mentioning the hurdles on choosing physicians placed by HMO, the insurances, and other societal beings.

Dr. Pheo

My credential

One reader suggests that I add my credential. Here is a bit about my credential:

I have an MD degree and a PhD degree (in one of the biological sciences). I am board-certified in Internal Medicine and Endocrinology. I practice as a specialist in pheochromocytoma and other neuroendocrine tumors in a large US hospital. I do clinical research in pheochromocytoma.

I never copy and paste any sentences to my blog. In other words, I type every letter in my blog.

The initial blogs are common situations in pheochromocytoma and I say the same words many times to my patients. Therefore I write them down here so that any one interested can read them. I anticipate I will answer more questions than write on general topics in the near future.

The real testament of my credential will be and should be whether my answers to specific questions make sense, help patients and colleagues, and are later confirmed to be correct.

Surgical resection of pheochromocytoma

There are 3 surgical approaches to remove a pheochromocytoma: 1) open adrenalectomy, 2) laparoscopic adrenalectomy, and 3) cortex-preserving partial adrenalectomy.

The open adrenalectomy used to be the only approach but is now usually reserved for large (>10 cm) tumors. The whole adrenal gland along with the tumor is removed.

Laparoscopic adrenalectomy is the main procedure nowadays and is performed for most patients with pheochromocytoma. Again, the whole adrenal gland along with the tumor is removed.

Cortex-preserving partial adrenalectomy only removes the tumor but preserves the adrenal cortex (at least in theory). It is possible only in some patients. It is appealing to young patients with familial pheochromocytoma. These patients will have multiple pheochromocytomas on both adrenal glands and have to take medications for many many years if they have both adrenal glands removed at a young age.

The most important factors for a successful operation are experienced surgeon and anesthesiologist, and of course, careful preoperative preparation. The specific approach a surgeon adopts is less a factor. The surgeon has to be proficient in the specific approach she/he uses.