[New Latin, from International Scientific Vocabulary pheochromocyte chromaffin cell + New Latin -oma]
First appeared circa 1929: A tumor that is derived from chromaffin cells, usually associated with paroxysmal or sustained hypertension
First of all, I need to state that I am not a medical doctor. My name is Mary Peebels, and I've had extensive background in physiology, chemistry, and physical scienced. My main knowledge of Pheochromocytoma and the associated illnesses comes from first hand experience, which is a form of knowledge that cannot be learned in a college classroom or from a textbook. I have seen the pain and distress of others, including my family members. Because I am concerned for others who suffer from the mental and physical cruelty of this illness, I began this site. Until recently, I didn't want to know any medical details about the illness even though I had an extensive background in physiology. Any time I would read or talk about tumors and surgery, I would begin to feel a sense of anxiety or doom. Even now, there are times when I am working on this site, and I have to completely stop and focus on something less disturbing. If this discussion upsets you in any way, I understand and sympathize.
Pheochromocytoma in my family's case (MEN2A) is a disease in which malignant cancer cells are found in special cells in the body called chromaffin cells. In non-MENS or non-inherited types of Pheochromocytoma, the tumor is most often not cancer. With MENS, Pheochromocytomas start inside the adrenal gland, in the medulla section, where most chromaffin cells are located. There are two adrenal glands, one above each kidney in the back of the upper abdomen. Cells in the adrenal glands make important hormones that help the body work properly. The Pheochromocytoma usually is found in the adrenal, as stated. However, Pheochromocytoma may also start in other parts of the body, such as the area around the heart or bladder. 1
Some tumors which start in the chromaffin cells do not spread to other parts of the body and are not cancer. These are called benign tumors. (I've always thought that "benign" was such a strange word for a tumor. Does any tumor show kindness and gentleness? As far as I'm concerned, this misnomer is as silly as calling a dictator benign.) Anyway, if a tumor is found, the doctor will need to determine whether it is cancer or "benign".
Pheochromocytomas often cause the adrenal glands to make too many hormones called catecholamines. The extra catecholamines cause high blood pressure, called hypertension, which can cause headaches, sweating, pounding of the heart, pain in the chest, and a feeling of anxiety. High blood pressure that goes on for a long time without treatment can lead to heart disease, stroke, and other major health problems such as kidney, pancreas and liver illness.
If there are symptoms, your doctor should order blood and urine tests to see if there are extra hormones in the body. A patient may also have a special nuclear medicine scan. A CT scan, an x-ray that uses a computer to make a picture of the inside of a part of the body, or an MRI scan, which uses magnetic waves to make a picture of the abdomen, may also be done. I do need to CAUTION that some patients have had blood and urine tests which came back negative in the beginning stages, yet a Pheochromocytoma was found present later on in time. Also, a tiny tumor sometimes can't be found with a CT scan or an MRI.
Pheochromocytoma is so rare, and the technology has not arrived to a point where we can always receive help in the first stages. There are medicines which can control the anxiety, pain, blood pressure, and other symptoms. I want to emphasize the importance of these medicines, because without them, I wouldn't be alive.
Pheochromocytoma can make you think that you are, or will go crazy. It causes severe hormonal imbalances, anxiety and panic attacks that can be overwhelming. There are other forms of anxiety and panic attacks, other than Pheochromocytoma which can be helped with medications. They are also caused by hormonal imbalances, and they need to be controlled so that you can enjoy life.
Pheochromocytoma is often part of a condition called Multiple Endocrine Neoplasia (MEN), which has also been called Sipple's Syndrome. People with MEN often have other cancers, such as Thyroid cancer, and other hormonal problems. Often a condition called Hyperparathyroidism exists. The Parathyroids are tiny but mighty, and they reside on the Thyroid. They have also been found in other places in some cases, such as the Thymus. The Parathyroid tumors are called adenoma, and are "benign". I want to comment on the benign, kind, and gentle way that my Parathyroid tumors took calcium out of my bones and teeth, and rapidly made numerous kidney stones, one right after another. The "benign" tumors also left me with a bone condition called Osteoporosis.
The early diagnosis of Pheochromocytoma is important, not only because it offers the possibility of curing hypertension but also because unrecognized Pheochromocytoma is a potentially lethal condition. Hypertensive crisis or shock, or both leading to death have been precipitated by drugs, anesthesia, or surgery for totally unrelated conditions. Persons with Pheochromocytoma do not react to certain medications the same way that other people do. Some medications cause a reverse effect in those of us with this illness.
Moreover, many of the tumors are malignant. Pheochromocytoma most commonly arises from chromaffin cells of the adrenal medulla but may be found wherever chromaffin tissue is located. Familial (inherited) occurrence is common. It can be transmitted as an autosomal dominant trait. The tumor may be associated with such neurocutaneous syndromes as Neurofibromatosis, von Hippel-Lindau disease, Tuberous Sclerosis, Sturge-Weber syndrome, and as a component of the Multiple Endocrine Neoplasia mentioned earlier. 2
(When I was first diagnosed, the illness MEN was then called MEA, or Multiple Endocrine Adenomatosis.)
Some doctors feel that the most common symptoms of Pheochromocytoma are headache, palpitations, and excessive and inappropriate perspiration. I feel that in this case, the doctors are ignoring the anxiety part of the illness which I personally feel is the most frightening one. Along with the anxiety, I experience tremors and uncontrollable shaking, nausea, weakness, chest pain like angina, and abdominal pain. Sometime I am flushed, other times I am extremely pale. I generally find myself looking at my hands, because they feel numb, and my palms are usually strangely mottled and sweaty.
I have been told that these symptoms of nervousness and anxiety, nausea and chest pain, et cetera are rare. Yet every "Pheochromocytoma" friend I have talked with has these symptoms. They also say that after an "episode" or "attack", the feel extreme weakness, exhaustion, and just want to curl up and sleep.
My experience with Pheochromocytoma consists of symptoms which are paroxysmal. This means that the attacks are sudden and without any warning. I can go for months without an attack, and almost reach a point where I feel I am "cured". Then out of the blue,I feel the familiar pain, anxiety, nausea, and I want so badly for it to stop.
I'd like to emphasize again that familial Pheochromocytoma is associated with medullary carcinoma of the thyroid, and adenoma or hyperplasia of the parathyroid glands and has been designated Multiple Endocrine Neoplasia Type II or Sipple's Syndrome. The coexistance of Pheochromocytoma, thickened cranial nerves, alimentary track ganglioneuromatosis, and marfanoid habitus constitutes Multiple Endocrine Neoplasia Type III. The diagnosis of any Pheochromocytoma requires the demonstration of excessive catecholamine secretion. The most widely used procedure includes measurement of urinary catecholamines or their metabolites, vanillomandelic acid and total metanephrines. Of these, the urinary metanephrines provide a more sensitive clue to the presence of Pheochromocytoma. Furthermore, plasma catecholamine measurements may be as reliable as the measurement of urinary metabolites.
In the early stages of Pheochromocytoma, the urine test must be taken DURING the attack and and for a short specified time afterwards. This urine test is called VMA, or Vanillomandelic Acid. The VMA is usually performed during a 24 hour period, but for an attack "urine test", the test may be done over a two hour period, and then multiplied by 12. As noted above, keep the test refrigerated, and remind the laboratory handlers to keep it refrigerated. 3
As with many blood tests, determination of plasma catecholamines requires that the patient be in a fasting state. The amounts of excreted free catecholamines and their metabolites vary depending on the levels of synthesising and metabolizing enzymes within the tumor. Additional provocative testing may be done with a glucagon stimulation test.
A suppression test uses the ability of clonidine, a centrally acting alpha-adrenergic agonist, to suppress the release of neurogenically mediated catecholamines. Plasma catecholamines in patients with essential hypertension are suppressed by clonidine, whereas they are unaltered in patients with Pheochromocytoma. A normal clonidine suppression tests consists of a fall of norepinephrine and epinephrine to a level below 500Pg/ml 2- 3 hours after the administration of 0.3mg of clonidine. 4
Concentrations of total plasma catecholamines are measured while the patient kept rested in a supine position for at least 30 minutes. Values over 2,000Pg/ml are considered pathognomonic of Pheochromocytoma. Patients with values between 1,000 and 2,000Pg/ml receive a clonidine suppression test. An abdominal CT scan is then performed on patients with clinical and biochemical features suggestive of Pheochromocytoma. 4
A recently developed technique for localization of neoplastic chromaffin tissues uses I-131-metaiodobenzyl guanidine, a radioactive compound selectively taken up by adrenergic cells. 5
Pheochromocytoma is cured by surgical removal of the tumor, but I want to say that my family has the inherited type of the illness, and removal of tumors cured the illness only temporarily. More "Pheos" developed later on in their lives.
I also want to add that one of my doctors informed me that beta-blockers such as Atenolol should not be given as a first medicine in treating the blood pressure and angina pains. Atenolol is a great medicine for some people, but an alpha-blocker, such as Hytrin, should be given instead, in the case of Pheochromocytoma. 6
Lastly, while on the subject of tumors, I want to make a suggestion to the medical community:
1.) I think that a tumor which is dormant or truly kind and gentle can be called a "Benign" tumor.
2.) I think that a tumor which is "Malignant" should be called exactly that.
3.) I think that "Benign" tumors which are doing as much or even more damage to a person's body as a "Malignant" tumor should be called an "Insidious" tumor.
Index of Pages
Additional Information and Credits
1 This noted information came from AOL Health Channel, but I would like to point out that several group members and a number of medical doctors feel that AOL needs to greatly update some of their information and definitions. (An immediate example of this need for updating is seen in the unstated fact that many Pheochromocytoma are not cancerous):
Information from AOL Health Channel, quotes, "Pheochromocytoma, a rare cancer, is a disease in which cancer (malignant) cells are found in special cells in the body called chromaffin cells. Most pheochromocytomas start in the inside of the adrenal gland (the adrenal medulla) where most chromaffin cells are located. You have two adrenal glands, one above each kidney in the back of your upper abdomen. The adrenal glands are also called the suprarenal glands. Cells in the adrenal glands make important hormones that help your body work properly. Usually pheochromocytoma affects only one adrenal gland. Pheochromocytoma may also start in other parts of the body, such as the area around the heart or bladder.
Most tumors that start in the chromaffin cells do not spread to other parts of the body and are not cancer. These are called benign tumors. If you have a tumor, your doctor will need to determine whether it is cancer or benign.
Like most cancers, pheochromocytoma is best treated when it is found (diagnosed) early. Pheochromocytomas often cause your adrenal glands to make too many hormones called catecholamines. The extra catecholamines cause high blood pressure (hypertension), which can cause headaches, sweating, pounding of the heart, pain in the chest, and a feeling of anxiety. High blood pressure that goes on for a long time without treatment can lead to heart disease, stroke, and other major health problems.
If you have symptoms, your doctor may order blood and urine tests to see if you have extra hormones in your body. You may also have a special nuclear medicine scan. A CT scan, an x-ray that uses a computer to make a picture of the inside of a part of your body or an MRI scan, which uses magnetic waves to make a picture of your abdomen, may also be done.
Pheochromocytoma is sometimes part of a condition called multiple endocrine neoplasia syndrome (MEN). People with MEN often have other cancers (such as thyroid cancer) and other hormonal problems.
Your chance of recovery (prognosis) depends on how far the cancer has spread, your age, and your general health.
Transmitted: 6/22/98 (a2002494) Pheochromocytoma
2 Information from Encarta Encyclopedia, Endocrine Tumors and Diseases. Version 1998, Division of MicroSoft, Inc. Link: Encarta Online Home & Pheochromocytoma in von Hippel-Lindau Disease& VHL Handbook, Part 2
3 Information from research library at University of California Medical Center, San Franciso CA, Francis S. Greenspan FACP, noted Endocrinologist
Links: Journal of the AMA and Laparoscopic Adrenalectomy and also
Consequences of Direct Genetic Testing for Germline Mutations in the Clinical Management of Families With Multiple Endocrine Neoplasia, Type II
4Information from research by Mary Peebels and the wonderful helpers at the library at Stanford University, Stanford CA Complete Link: Medical Research Lane Library
5 Information from Stanford University Libraries and Academic Information Resources Web Site, Stanford CA Complete Link: Education Solutions: Case Studies
6 Information from Ash Jain, MD, Cardiology Specialist at Washington Hospital, Fremont CA; Lynn A. Kohlmeier, M.D, Private Practice, Fremont CA