One important feature of pheos is that they always grow. The growth speed of pheo, however, is only known for patients with von Hippel Lindau disease. For people with true sporadic pheo (~70% of all pheos), we have little knowledge on the growth speed of their pheos. The reason is that familial pheos can be monitored but sporadic pheos are usually resected once they are diagnosed. Only under two conditions, patient's choice of delaying surgery and omission of pheo testing of incidentally-identified adrenal mass, the growth speed of sporadic pheos can be assessed.
I recently took care of a patient with likely sporadic pheo. This patient's adrenal pheo increased from 0.6 x 1.2 cm to 3.0 x 3.6 cm in 6 years. This patient is among the few I know with recorded growth speed of a sporadic pheo.
The information on pheo growth speed is much needed to tell patients what they will expect of their tumor. I would like to do a survey of the growth speed of pheo, whether with genetic mutations or not. If you have a pheo that was imaged multiple times, please let me know the size of the tumor, the time interval in-between imaging, and whether you have a pheo syndrome (with mutations) or not. I thank you in advance.
Dr. Pheo
Tuesday, June 16, 2009
Tuesday, May 26, 2009
A familial pheo risk calculator
Two papers on risks of familial pheo appear in the May, 2009 issue of the Journal of Clinical Endocrinology and Metabolism. One Paper describes experience in about 500 Italian patients, the other over 200 Spanish patients. The paper on Italian patients is particularly informative. With the three large studies now available (the original landmark study in 2002 published in the New England Journal of Medicine and these two new papers), I feel it is time to quantify the probability of having familial pheo. I have prepared a calculator of risks for familial pheo. The calculator is based on the 3 above large studies. The calculator has only five input variables:
1) Primary location of your pheo/paraganglioma
2) Age at diagnosis
3) Family history of pheo or paraganglioma (Yes/No)
4) Single or multiple pheo/paraganglioma (Single/Multiple)
5) Do you have any of the following diseases in addition to pheo/paraganglioma (Yes/No): medullary thyroid cancer, hyperparathyrodism, hirschsprung's disease, mucosa neuroma, hemangioblastoma, renal cell carcinoma, pancreatic neuroendocrine tumor, and skin neurofibroma?
As this is a blog format, I can not post the calculator on line. If you tell me the answer to the 5 variables, I will tell you your risk of having a familial pheo. The calculator has its intrinsic limitations and perhaps even errors; and the results are only as good as the accuracy of the 5 variables. Also I can not tell you the chances of any particular mutated genes.
Of course, the calculator only gives you an estimate of risk and only your doctor and you shall decide whether to do genetic testing. You should use the results of the calculator responsibly and at your own risk; I can not be held liable for any adverse consequences.
Dr. Pheo
1) Primary location of your pheo/paraganglioma
2) Age at diagnosis
3) Family history of pheo or paraganglioma (Yes/No)
4) Single or multiple pheo/paraganglioma (Single/Multiple)
5) Do you have any of the following diseases in addition to pheo/paraganglioma (Yes/No): medullary thyroid cancer, hyperparathyrodism, hirschsprung's disease, mucosa neuroma, hemangioblastoma, renal cell carcinoma, pancreatic neuroendocrine tumor, and skin neurofibroma?
As this is a blog format, I can not post the calculator on line. If you tell me the answer to the 5 variables, I will tell you your risk of having a familial pheo. The calculator has its intrinsic limitations and perhaps even errors; and the results are only as good as the accuracy of the 5 variables. Also I can not tell you the chances of any particular mutated genes.
Of course, the calculator only gives you an estimate of risk and only your doctor and you shall decide whether to do genetic testing. You should use the results of the calculator responsibly and at your own risk; I can not be held liable for any adverse consequences.
Dr. Pheo
Monday, May 11, 2009
Silent? No way!
You may have heard the term “silent pheo”. The sole purpose of this post is to convince you that “silent pheo” is a dangerous term and should be avoided at all cost. So next time you hear someone mentioning “silent pheo”, you tell the person there is no such a thing. Every time I hear the term, I get goose bumps and try to correct the person.
The term “silent pheo” or “subclinical pheo” apparently refers to a pheo in a patient who does not have hypertension or paroxysmal attacks. There are quite a few situations where a patient indeed has a pheo or paraganglimoa but does not have classical symptoms of pheo. Most paragangliomas in the neck and chest do not produce significant amounts of catecholamines. I would rather call these tumors "nonfunctional" rather than "silent". Very small pheos produce only small amounts of catecholamines so that they do not cause clinical symptoms. In the above two situations, blood tests for pheo show normal results. Most pheos in adrenal glands, retroperitoneal space, and bladder do produce catecholamines and are therefore "functional". The majority of patients with pheo have at least subtle symptoms that can be elicited by experienced physicians or realized in retrospect after removal of the tumor.
Some patients indeed have no apparent hypertension in spite of very functional pheos. These patients tend to be mismanaged and are at the highest risk of complications around surgery. Here are the reasons. These patients tend to be young and healthy before they have pheo. Their bodies try very hard to accommodate the bombardment of catecholamines by shrinking their blood volume. My experience is that their blood pressure may be normal while sitting or standing but rises when they lie down. An inexperienced doctor may think they have so-called "desensitization" of catecholamine receptors and can go ahead with surgery without preparation. Disaster happens if they do. They will have very high blood pressure while the tumor is manipulated and profound hypotension after the tumor is resected. Then they will get a lot of intravenous fluid their hearts can not handle. It will be a mess!
The bottomline is that if a patient has normal blood test results, whatever tumor the patient has may not be sufficiently functional. If the patient has elevated blood markers for pheo, the patient should be treated as having a functional pheo and undergo careful preoperative preparation, regardless of having hypertension or not. A young, otherwise healthy patient with a large pheo and high levels of markers but without hypertension is the most vulnerable one. That patient particularly deserves careful preoperative preparation.
Dr. Pheo
The term “silent pheo” or “subclinical pheo” apparently refers to a pheo in a patient who does not have hypertension or paroxysmal attacks. There are quite a few situations where a patient indeed has a pheo or paraganglimoa but does not have classical symptoms of pheo. Most paragangliomas in the neck and chest do not produce significant amounts of catecholamines. I would rather call these tumors "nonfunctional" rather than "silent". Very small pheos produce only small amounts of catecholamines so that they do not cause clinical symptoms. In the above two situations, blood tests for pheo show normal results. Most pheos in adrenal glands, retroperitoneal space, and bladder do produce catecholamines and are therefore "functional". The majority of patients with pheo have at least subtle symptoms that can be elicited by experienced physicians or realized in retrospect after removal of the tumor.
Some patients indeed have no apparent hypertension in spite of very functional pheos. These patients tend to be mismanaged and are at the highest risk of complications around surgery. Here are the reasons. These patients tend to be young and healthy before they have pheo. Their bodies try very hard to accommodate the bombardment of catecholamines by shrinking their blood volume. My experience is that their blood pressure may be normal while sitting or standing but rises when they lie down. An inexperienced doctor may think they have so-called "desensitization" of catecholamine receptors and can go ahead with surgery without preparation. Disaster happens if they do. They will have very high blood pressure while the tumor is manipulated and profound hypotension after the tumor is resected. Then they will get a lot of intravenous fluid their hearts can not handle. It will be a mess!
The bottomline is that if a patient has normal blood test results, whatever tumor the patient has may not be sufficiently functional. If the patient has elevated blood markers for pheo, the patient should be treated as having a functional pheo and undergo careful preoperative preparation, regardless of having hypertension or not. A young, otherwise healthy patient with a large pheo and high levels of markers but without hypertension is the most vulnerable one. That patient particularly deserves careful preoperative preparation.
Dr. Pheo
Tuesday, April 28, 2009
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
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
Sunday, April 19, 2009
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
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
Friday, April 17, 2009
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
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
Saturday, April 11, 2009
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
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
Subscribe to:
Posts (Atom)