Pheochromocytoma (dye) is a tumor that originates in the medulla of the adrenal gland. It is called a hormonally active tumor because it secretes catecholamines, which include adrenaline and norepinephrine. Pheochromocytoma most often affects people over 40. what are the causes and symptoms of phaeochromocytoma? What is the treatment of pigmentosa?
Pheochromocytoma( dye , Latinphaeochromocytoma ) is an active paraganglioma. The term phaeochromocytoma is used only for hormonally active tumors located in the adrenal medulla, while if they are located outside the adrenal glands, the following terms are used: sympathetic paragangioma (hormonally active; formerly pheochromocytoma) and parasympathetic (hormonally inactive) paraganglial neuroma. These types of lesions are most often located in the adrenal glands, but they can also affect the bladder, prostate gland, pericardium, or certain areas of the neck.
The size of the phaeochromocytoma most often does not exceed a few centimeters (tumors ranging in size from a few millimeters to 20-30 centimeters have been described), and the average weight is about 100 g. It can occur bilaterally and be malignant. Due to their histological structure, pheochromocytomas are divided by pathomorphologists into:
- epithelial tumors - made of epithelial cells, containing granular, eosinophilic cytoplasm and hyperchromatic nuclei; cells are arranged in strings and clusters
- polymorphic tumors - made of epithelial cells, but with a greater degree of differentiation (even giant cells present) and greater hyperchromaticity
- fusiform tumors - fusiform cells predominate; this is the rarest character
Pheochromocytoma: causes
Yes, the cause of a tumor in the adrenal medulla is unknown. A tumor appears because its cells secrete catecholamines, hormones produced in the medulla of the adrenal gland. These hormones, through the central nervous system, influence the work of the heart, blood pressure and the increase of glucose in the blood. In some patients, the occurrence of phaeochromocytoma is genetically determined. Sometimes I am accompanied by cancers of other organs - thyroid cancer, cancer of the parathyroid gland, and a pituitary tumorcerebral.
Pheochromocytoma: symptoms
The most characteristic symptom of phaeochromocytoma is arterial hypertension - paroxysmal or permanent. Additionally, patients report:
- paroxysmal headaches
- felt a violent heartbeat
- sweating
- pallor
- pupils widely dilated
- lowering blood pressure after standing up
- shaking hands
- feeling anxious
Sometimes the tumor is accompanied by chest pain, nausea and vomiting, constipation, abdominal pain or an increase in blood pressure during general anesthesia.
In pregnancy, a phaeochromocytoma can cause premature separation of the placenta and miscarriage.
Diagnosis of pheochromocytoma
The first step is the determination of free catecholamines or their metabolites (VMA vanillinmandelic acid and methoxycatecholamines) in the 24-hour urine collection (methoxycatecholamines can also be determined in serum). The test also measures urinary excretion of adrenaline, noradrenaline, and dopamine.
Imaging tests are also very important in tumor diagnosis:
- ultrasonography
- computed tomography
- magnetic resonance imaging
- adrenal scintigraphy
In the differential diagnosis of phaeochromocytoma, the following should be taken into account :
- primary hypertension
- diabetes with concomitant hypertension
- hyperthyroidism
- paroxysmal tachycardia
- ischemic heart disease
- brain tumors, especially those located in the posterior cranial cavity
- migraine, cluster or vasomotor headaches
- menopause in women
- acute intermittent porphyria
- pseudopheochromocytoma
- renovascular hypertension
- chaperones
- anxiety disorders
Treatment of pheochromocytoma
Cancer treatment consists of surgical removal of the adrenal gland. Before the procedure, patients are given drugs that block adrenergic receptors (e.g. labetalol, prazosin or phenoxybenzamine).
Prognosis and prophylaxis of pheochromocytoma
The prognosis after surgical removal of the tumor is favorable, provided that the tumor has not become malignant and is not located beyond the adrenal glands. The genetic occurrence of the disease gives a worse prognosis.
Families with cases of phaeochromocytoma should be subject to ongoing genetic counseling.
For a benign tumor, the 5-year survival rate is 96 percent, and for a tumormalignant disease is reduced to 44 percent. The risk of benign tumor recurrence is about 10 percent. An untreated malignant phaeochromocytoma has approximately 50 percent five-year survival.
The perioperative mortality does not exceed 3 percent. Blood pressure returns to normal in approximately 60 percent of patients after surgery. Normalization is more common in patients with paroxysmal hypertension (around 80 percent) than in persistent hypertension (around 40 percent).
