A pheochromocytoma is an adrenal gland tumor. He is able to produce hormones.
What is a pheochromocytoma?
A pheochromocytoma is a tumor in the adrenal medulla. In most cases, the hormone-producing tumor is benign. The hormones produced mostly include adrenaline and noradrenaline. In 85 percent of all cases, the tumor is located in the adrenal gland. While 85 percent of all pheochromocytomas are benign, 15 percent are malignant. The tumor is unilateral in 90 percent. In the remaining 10 percent, it settles on both sides. See dictionaryforall for Hemangioma (or Blood Sponge) in Dictionary.
A rare form of pheochromocytoma is the paraganglioma. The tumor runs along the sympathetic trunk outside the adrenal glands. This is located in the abdomen and chest region in the spinal column area. A pheochromocytoma is rare. Two to eight cases of the disease occur in around one million people. Adults are particularly affected by the disease, but sometimes children also suffer from the tumor.
Causes
The cause of the development of a pheochromocytoma cannot always be found out. In medicine, this is then referred to as a sporadic pheochromocytoma. This type of tumor occurs mainly between the ages of 40 and 50. Some forms of pheochromocytoma are hereditary. They are caused by mutations within the genome and mostly occur in younger people under the age of 40. About 10 percent of these pheochromocytomas are familial.
In some cases, pheochromocytoma occurs in connection with other hereditary diseases. These include von Hippel-Lindau syndrome, multiple endocrine neoplasia type 2 and neurofibromatosis type 1. A typical feature of pheochromocytoma is the increased production of stress hormones such as adrenaline and noradrenaline. The tumor can also produce dopamine, but this is less common.
Symptoms, Ailments & Signs
The increased release of adrenaline and noradrenaline leads to the symptoms of pheochromocytoma. However, the symptoms vary from person to person. Some patients even show no symptoms at all, which means that the tumor is only discovered by chance. High blood pressure is a typical symptom of pheochromocytoma. This leads to an abrupt and paroxysmal rise in blood pressure, which can even take on life-threatening proportions.
Doctors then speak of a hypertensive crisis or a blood pressure crisis. About 50 percent of all patients are affected by these symptoms. Sometimes blood pressure can also be chronically elevated, which is referred to as persistent hypertension. Around half of all adult patients and around 90 percent of all children suffer from it.
However, other symptoms are also possible with a pheochromocytoma. These include weight loss, angina pectoris, headaches, sweating, tachycardia, tremors, anxiety and pallor of the face. The high level of adrenaline also causes an increase in blood sugar levels. There is an increased risk of developing diabetes mellitus as the disease progresses.
Diagnosis & course of disease
The diagnosis of a pheochromocytoma is usually based on the typical symptoms. The most suspicious are the occasional attacks of high blood pressure, in which the usual medication treatments have no effect. It is also important to measure the stress hormones adrenaline and noradrenaline. For a reliable measurement, either a urine sample is taken, which is collected over a 24-hour period, or the blood plasma is analyzed within 30 minutes.
If there is an increased hormone content, a clonidine inhibition test is carried out. Clonidine is one of the active ingredients against high blood pressure. While clonidine inhibits the release of adrenaline in healthy people, the value remains high in the case of a pheochromocytoma. Imaging methods such as computed tomography (CT) or magnetic resonance imaging (MRI) are used to detect a pheochromocytoma.
A special MIBG scintigraphy is performed to localize a tumor that is located outside the adrenal gland. The course of the disease in a pheochromocytoma varies. In about 80 percent of all patients, blood pressure returns to normal after successful tumor removal. In the remaining patients, however, the high blood pressure persists because other causes also play a role. In about 15 percent of all those affected, a pheochromocytoma reappears after a while.
Complications
The symptoms of pheochromocytoma can be very different. Because of this, the complications and symptoms usually cannot be universally predicted. In most cases, however, this disease leads to high blood pressure. Those affected can also suffer a heart attack, which in the worst case can lead to the death of the person concerned.
General malaise and hypertension can also occur and usually have a very negative effect on the patient’s everyday life. It is not uncommon for those affected to suffer from anxiety or sweating. A racing heart or severe headaches can also occur due to the disease. There is also paleness in the face and tachycardia. If the pheochromocytoma is not treated, it can also lead to diabetes.
When treating pheochromocytoma, the tumor that is causing the symptoms is usually removed. Complications cannot be predicted universally. Chemotherapy is usually also necessary. The life expectancy of the person affected may also be reduced by the pheochromocytoma.
When should you go to the doctor?
Since a pheochromocytoma is a tumor, it must be treated immediately by a doctor. The sooner treatment and diagnosis occurs, the higher the chances of a complete cure. The tumor itself is often only discovered by chance, as it does not lead to any clear symptoms. After the diagnosis, however, this must be removed quickly. In many cases, pheochromocytoma is manifested by high blood pressure. If the high blood pressure occurs for no particular reason and above all permanently, a doctor should be consulted. The blood pressure itself is often chronically elevated. The pheochromocytoma is also noticeable through a loss of weight, facial pallor or heavy sweating. If you have these symptoms, you should consult a doctor.
As a rule, the first examination can be carried out by the family doctor. However, a more detailed examination and possibly treatment requires a specialist doctor. Whether the patient’s life expectancy will be reduced as a result of the pheochromocytoma cannot generally be predicted.
Treatment & Therapy
The best treatment for pheochromocytoma is surgical removal of the tumor. Minimally invasive laparoscopy is performed in most patients for this purpose. Only three smaller incisions need to be made on the abdominal wall. Finally, after inserting the surgical instruments, the surgeon removes the tumor. If the tumor is larger or difficult to remove, a more extensive laparotomy may also be necessary.
If both adrenal glands are affected by a pheochromocytoma, this results in a steroid hormone deficiency after an operation. To compensate for this, the missing hormones are replaced with drugs. Further treatment measures depend on whether the pheochromocytoma is benign or malignant. A malignant tumor can lead to the development of metastases (secondary tumors). Chemotherapy or radioiodine therapy are conceivable as possible therapeutic measures.
Sometimes it is not possible to treat a pheochromocytoma surgically. In such cases, symptomatic therapy takes place. It treats the symptoms that occur as a result of the excessive production of stress hormones. For example, alpha blockers can reduce the effects of adrenaline by blocking the docking sites.
Outlook & Forecast
The prognosis for a pheochromocytoma varies and depends on several factors. If it is possible to operate on the tumor early and if the patient does not suffer from additional diseases, the prospects are usually good. The symptoms then often go away. In around 50 to 80 percent of all affected persons, blood pressure returns to normal after surgery, provided the pheochromocytoma is benign.
If the patient has been suffering from the pheochromocytoma for a long time, there is a risk of side effects such as heart failure due to the increased blood pressure. Around 15 percent of all those affected must expect a recurrence of a pheochromocytoma after the therapy. The doctors then speak of a recurrence. For this reason, the patient should have check-ups at regular intervals. They serve to detect a possible recurrence in good time in order to enable effective treatment.
If a benign phaechromocytoma is surgically removed, essential hypertension still exists. This means that blood pressure continues to be high because other triggers are present. If a benign pheochromocytoma is present, 95 percent of all patients survive the next five years. However, if the tumor is malignant and metastases have already formed, the 5-year survival rate drops to around 44 percent.
Prevention
Preventing a pheochromocytoma is not possible. However, genetic counseling can be useful for families with previous problems.
Aftercare
In most cases, those affected with a pheochromocytoma only have very limited measures and options for aftercare. Since this is a tumor on the kidneys, a doctor should be contacted very early on so that there are no complications or other symptoms later on. The earlier a doctor is consulted, the better the further course of the disease, since spontaneous healing cannot occur.
Most of those affected require surgical intervention. The person concerned should definitely rest after such an operation and protect his body. Efforts or stressful and physical activities should be avoided in order not to continue to strain the body. Most of those affected with pheochromocytoma also depend on the support of friends and relatives in everyday life.
It is not uncommon for psychological support to be very important so that mental upsets or depression do not occur. Regular checks and examinations by a doctor are also very important in order to identify and remove further tumors at an early stage. In some cases, pheochromocytoma reduces the life expectancy of the person affected.
You can do that yourself
A pheohomocytoma is usually surgically removed. What measures the patient can take afterwards to recover quickly depends on the location of the tumor and other factors.
Basically, nothing should be eaten in the first few hours after the procedure. After a general anesthetic you also have to refrain from drinking. After one day, the patient can eat bland food again. The doctor will make precise specifications regarding the diet and, if necessary, also involve a nutritionist. In order to compensate for the loss of nutrients and fluids, you should eat and drink enough in the days and weeks after the pheohomocytoma operation. Dietary supplements and infusion solutions are also available to accompany this. Alcohol should be avoided in the first few weeks as it has a negative effect on blood clotting.
In addition, physical protection always applies. The operated part of the body is particularly sensitive to pain and must not be exposed to strong vibrations. Instead, careful cooling is recommended. After the pheohomocytoma has been removed, other symptoms may initially appear. These should subside after a few weeks at the latest. If symptoms persist, it is best to inform the doctor.