It is usually a benign neoplasm originating in cells that are found mainly in the medulla of the adrenal glands. A characteristic feature of such tumors is the secretion of catecholamines.
Pheochromocytomas most often develop in the adrenal medulla, but can also appear in the abdominal cavity and sometimes in the chest. This type of tumor is called a 10 percent tumor, according to medical statistics describing it. In 10 percent of cases it occurs outside the adrenal glands, in 10%. cases is malignant, in 10 percent. attacks both adrenal glands, 10 percent affects children, in 10 percent is plural, in 10 percent. patients do not have hypertension.
Characteristics of tumors
After years of clinical observations, the nomenclature was clarified and the term pheochromocytoma is used when it occurs in the adrenal medulla, i.e. it is hormonally active. If it is localized elsewhere in the body, the correct term is parasympathetic (non-functioning) paragaginal neuroma.
Pheochromocytomas usually develop in the 4th and 5th decade of life, but can appear in both older and younger people. About 25 percent. cases of the disease are associated with mutations in many genes. The diameters of the formed tumors are very different – from a few millimeters to 20-30 centimeters. Sometimes they are a several-kilogram structure that occupies a large part of the abdominal cavity. The average tumor, however, weighs about 100 g. Pheochromocytomas are richly vascularized. Inside there are visible bleeding, necrotic foci and calcium deposits.
Symptoms
The main symptom of phaeochromocytoma is arterial hypertension, which may be paroxysmal or permanent. Seizures or spikes of high pressure last differently long – from several minutes to even several hours. The frequency of the pressure surges also varies. During an attack, there is a severe headache, drenching sweats, tachycardia, significant pale skin, a feeling of anxiety, sensory disturbances and visible hand tremors. Sometimes there is a visible dilatation of the pupils. After each episode of hypertension, the patient is exhausted, weary and eagerly falls asleep.
The cause of an attack of high blood pressure can be a lot of physical exertion, stress, abdominal pressure, heavy meals, long standing, sexual intercourse or even a bowel movement. Another reason for an attack may be medication. This group includes, among others some cytostatics, tricyclic antidepressants, histamine, glucagon, etc. People with phaeochromocytoma are usually thin because their metabolism is accelerated.
Many symptoms of a phaeochromocytoma result from its location. For example, when a tumor develops in the bladder, it may interfere with urination or cause bleeding. When placed in the abdominal cavity, it will cause nausea, vomiting, and constipation.
A symptom of the disease may also be a fever without chills. Usually it is associated with accelerated metabolism and disturbed heat regulation. Others may experience numbness and tingling in the extremities, visual disturbances, fits, loss of consciousness.
The characteristic symptoms of the tumor are also cardiovascular disorders, which is related to the influence of catecholamines on the heart muscle. For this reason, there may be cardiomyopathy, arrhythmias, atrial fibrillation, supraventricular tachycardia, and ventricular fibrillation.
Diagnosis and treatment
To make a diagnosis, your doctor recommends that you measure free catecholamine in your XNUMX-hour urine collection. It is also necessary to perform imaging tests that allow to locate the tumor location. Typically, computed tomography or magnetic resonance imaging is performed. When the result is not clear, a contrast scintigraphy or a PET scan is ordered. Determining the location of the tumor is extremely important because it determines the choice of the surgical technique.
Before the planned surgery, which is the treatment of choice in patients with phaeochromocytoma, appropriate pharmacological preparation is necessary in order to restore the correct pressure and protect the patient from its sudden jumps. Typically, medications are given for two weeks or even longer. An important element of preparation for surgery is to replenish the deficiency of blood volume. Sometimes it is necessary to roll it.
The only effective method of treatment is surgical removal of the tumor. The type and scope of the surgery always depends on the location of the tumor. If, most often, the tumor is located in the adrenal glands, the adrenal glands are removed. When operating on pheochromocytomas, the laparoscopic or laparotomy methods are increasingly used.
After surgery, it is necessary to monitor blood pressure, pulse and frequent ECGs. Persistent high blood pressure may indicate the presence of a second tumor.
Malignant phaeochromocytomas (about 50% of cases) are treated surgically or with radiotherapy, chemotherapy, cryoablation or embolization.
The prognosis is good for benign phaeochromocytomas. 96 percent patients survive 5 years. Malignant tumors have a worse prognosis. Only 44 percent. survives 5 years. The risk of tumor recurrence is approximately 10%. In over 60 percent. patients, after tumor removal, blood pressure is normalized.
Tekst: Anna Jarosz