Pheochromocytoma refers to an adrenal medullary tumor. It is capable of producing hormones.
What is a pheochromocytoma?
A pheochromocytoma is a tumor in the adrenal medulla. In most cases, the hormone-producing tumor is benign. Hormones produced include mostly epinephrine and norepinephrine. In 85 percent of all cases, the tumor is located on the adrenal gland. While 85 percent of all pheochromocytomas are benign in nature, 15 percent take a malignant course. In 90 percent of cases, the tumor is unilateral. In the remaining 10 percent, it settles on both sides. A rare form of pheochromocytoma is paraganglioma. In this case, the tumor runs along the sympathetic boundary cord outside the adrenal glands. This is located in the abdominal and thoracic region in the spinal area. Pheochromocytoma rarely occurs. Two to eight cases of the disease occur in about one million people. Adults are particularly affected by the disease, but sometimes children also suffer from the tumor.
Causes
The cause for the development of a pheochromocytoma cannot always be found out. In medicine, the term sporadic pheochromocytoma is then used. This form of tumor mainly appears at the age of 40 to 50 years. Some forms of pheochromocytoma are hereditary. They are caused by mutations within the genetic material and occur mostly in younger people under the age of 40. About 10 percent of these pheochromocytomas show up in families. In some cases, pheochromocytoma occurs in association with other inherited diseases. These include Von Hippel-Lindau syndrome, multiple endocrine neoplasia type 2, and neurofibromatosis type 1. A typical feature of pheochromocytoma is increased production of stress hormones such as adrenaline and noradrenaline. Similarly, the tumor can produce dopamine, although this is less common.
Symptoms, complaints, and signs
The increased release of epinephrine and norepinephrine causes the symptoms of pheochromocytoma. Individually, however, the symptoms vary. In some patients there are even no symptoms at all, which means that the tumor is only discovered by chance. A typical symptom of pheochromocytoma is high blood pressure. The blood pressure rises abruptly and suddenly, and can even become life-threatening. Doctors then speak of a hypertensive crisis or a blood pressure crisis. About 50 percent of all patients are affected by this symptom. Sometimes blood pressure can also be chronically elevated, which is called persistent hypertension. Around half of all adult patients and around 90 percent of all children suffer from this condition. However, other symptoms of pheochromocytoma are possible. These include weight loss, angina pectoris, headaches, sweating, palpitations, tremors, anxiety, and pallor of the face. The high adrenaline level also results in a rise in blood sugar levels. Thus, there is an increased risk of developing diabetes mellitus as the disease progresses.
Diagnosis and course of the disease
Diagnosis of pheochromocytoma is mostly based on typical symptoms. First and foremost, the occasional attacks of high blood pressure, in which the usual treatments with medication have no effect, are considered suspicious. It is also important to measure the stress hormones adrenaline and noradrenaline. For a reliable measurement, either a urine sample collected 24 hours or an examination of the blood plasma within 30 minutes is performed. If there is an elevated hormone level, a clonidine inhibition test is performed. Clonidine is one of the active substances against elevated blood pressure. While clonidine inhibits the release of adrenaline in healthy individuals, the level remains high in the case of a pheochromocytoma. Imaging techniques such as computed tomography (CT) or magnetic resonance imaging (MRI) are used to detect a pheochromocytoma. To localize a tumor that is located outside the adrenal gland, a special MIBG scintigraphy is performed. The course of disease in pheochromocytoma varies. In about 80 percent of all patients, blood pressure returns to normal after successful removal of the tumor.In the remaining patients, however, hypertension persists because other causes play a role. In about 15 percent of all those affected, pheochromocytoma recurs after a considerable time.
Complications
The symptoms of pheochromocytoma can vary widely. For this reason, complications and symptoms usually cannot be universally predicted. However, in most cases, this disease results in high blood pressure. Sufferers can also suffer a heart attack in the process, which in the worst case leads to the death of the affected person. 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. Furthermore, pallor of the face and heart palpitations also occur. If pheochromocytoma is not treated, diabetes may also develop. In the treatment of pheochromocytoma, the tumor that is responsible for the symptoms is usually removed. It cannot be universally predicted whether complications will occur. Furthermore, chemotherapy is usually also necessary. It is also possible that the pheochromocytoma will reduce the life expectancy of the affected person.
When should you see a doctor?
Because a pheochromocytoma is a tumor, it must be treated immediately by a doctor. The earlier treatment and diagnosis occur, the higher the chances of a complete cure. Often, the tumor itself is discovered only by chance, as it does not lead to any clear symptoms. After diagnosis, however, it must be removed quickly. In many cases, pheochromocytoma is manifested by high blood pressure. If the high blood pressure occurs without a particular reason and, above all, permanently, a doctor should be consulted. The blood pressure itself is often chronically elevated. Likewise, the pheochromocytoma is noticeable by a loss of weight, facial pallor or by heavy sweating. A physician should be consulted for these symptoms as well. Usually, the initial examination can be performed by the primary care physician. However, a more detailed examination and possibly treatment requires a specialist. Whether pheochromocytoma will result in a decreased life expectancy for the patient cannot generally be predicted.
Treatment and therapy
Surgical removal of the tumor is considered the best method of treatment for pheochromocytoma. In most patients, minimally invasive laparoscopy is performed for this purpose. In this procedure, only three small incisions need to be made on the abdominal wall. After inserting the surgical instruments, the surgeon finally removes the tumor. If the tumor is larger or difficult to remove, a more extensive laparotomy may be necessary. If both adrenal glands are affected by a pheochromocytoma, this results in a steroid hormone deficiency after surgery. To compensate for this, the missing hormones are replaced with drugs. Further treatment measures depend on whether the pheochromocytoma is benign or malignant. Thus, a malignant tumor can lead to the development of metastases (daughter tumors). Possible therapeutic measures include chemotherapy or radio-iodine therapy. Sometimes it is not possible to treat a pheochromocytoma surgically. In such cases, symptomatic therapy takes place. This involves treating the symptoms that occur as a result of the excessive production of stress hormones. For example, alpha-blockers can attenuate the effects of adrenaline by blocking the docking sites.
Outlook and prognosis
The prognosis for pheochromocytoma varies and depends on several factors. If it is possible to remove the tumor early and the patient does not suffer from additional diseases, the outlook is usually favorable. The symptoms often disappear again. In about 50 to 80 percent of all affected persons, blood pressure returns to normal after surgery, provided that the pheochromocytoma is benign. If the patient has been suffering from the pheochromocytoma for a long time, there is a risk of secondary symptoms such as heart failure due to the increased blood pressure.Around 15 percent of all those affected must expect a recurrence of pheochromocytoma after therapy. Doctors then refer to this as a recurrence. For this reason, the patient should attend control examinations at regular intervals. They serve to detect a possible recurrence in time to enable effective treatment. If a benign phaechromocytoma is surgically removed, essential hypertension still exists. This means that blood pressure will continue to be high because other triggers are still present. If a benign pheochromocytoma is present, 95 percent of all patients survive the next five years. However, if the tumor is malignant and has already metastasized, the 5-year survival rate drops to about 44 percent.
Prevention
Preventing pheochromocytoma is not possible. However, genetic counseling may be useful in predisposed families.
Follow-up care
In most cases, the person affected by pheochromocytoma has only very limited measures and options for aftercare. Since this is a tumor on the kidneys, a doctor should be contacted very early in the process to prevent complications or other complaints from arising in the further course. The earlier a doctor is consulted, the better the further course of the disease usually is, since no spontaneous healing can occur. Most of the affected persons are dependent on a surgical intervention. In any case, the affected person should rest and take care of his body after such an operation. Efforts or stressful and physical activities should be refrained from in order to avoid further strain on the body. Most patients with pheochromocytoma are also dependent on the support of friends and relatives in everyday life. It is not uncommon for psychological support to be very important in order to prevent psychological upsets or depression. Regular checks and examinations by a doctor are also very important in order to detect and remove further tumors at an early stage. In some cases, pheochromocytoma reduces the life expectancy of the affected person.
Here’s what you can do yourself
A pheochromocytoma is usually removed surgically. The measures the patient can take afterward to recover quickly depend on the location of the tumor and other factors. In principle, nothing should be eaten in the first hours after the operation. After general anesthesia, drinking must also be avoided. After one day, the patient is allowed to eat light food again. The doctor will give precise instructions regarding the diet and, if necessary, also involve a nutritionist. In order to compensate for the loss of nutrients and fluids, sufficient food and drink should be consumed in the days and weeks following the pheochomocytoma operation. Accompanying nutritional supplements as well as infusion solutions are recommended. Alcohol should be avoided in the first few weeks, as it has a negative effect on blood clotting. Physical rest is also always recommended. The operated part of the body is particularly sensitive to pain and must not be subjected to strong vibrations. Instead, careful cooling is recommended. After the pheochomocytoma has been removed, further symptoms may initially occur. These should have subsided after a few weeks at the latest. If symptoms persist, it is best to inform the physician.