Pheochromocytoma figuratively called in medical circles and "the great imitator", and the "true pharmacological bomb" and "impressionistic tumor." What lies behind "pheochromocytoma" term?
Modern scientists do not know the real causeoccurrence of this tumor. They have only assumptions that this disease is genetic in nature. However, medicine has accumulated a lot of information about pheochromocytoma. Here are the major historical milestones of knowledge of this disease:
- In 1886, German pathologist Frankel first described pheochromocytoma found in the adrenal glands have two 18-year-old girl, who died suddenly from collapsing.
- In 1893 Manasseh reported a patient with pheochromocytoma, and in 1896 demonstrated that these tumors become yellow-brown color when stained with chromium salts.
- In 1904, Marchetti described adrenal pheochromocytoma, which can be considered hereditary, but the first documented case of hereditary pheochromocytomas was described in 1943
- In 1912, L. Rush coined the term "pheochromocytoma" (from the Greek phaios - dark color;. Chroma - chromium salts; cytoma - tumor).
- 1926 Roux (Switzerland) and C.H. Mayo (US) successfully removed pheochromocytoma. Both patients had occult tumor with paroxysmal episodes of hypertension, who underwent surgery.
- 1952 marked an increase in the number of thyroid carcinomas associated with pheochromocytoma.
The prevalence of pheochromocytoma
Pheochromocytoma occurs in 1% of patients withconstantly elevated diastolic blood pressure, being one of the causes of hypertension. In a population of pheochromocytoma are relatively rare, with a frequency of greater than 1:. 200 thousand a year, and the incidence is less than 1 person in 2 million people.
The swelling may occur in persons of any age,but most frequently it is observed between 20 and 40 years. The prevalence of pheochromocytomas among adult men and women is the same, but in children is more common in boys - 60%. Multiple tumors often found in children than in adults (respectively 35 and 8% of cases).
Currently not seem possibletalk about the absolute lifetime diagnosis of chromaffin adrenal tumors. Often remains undiagnosed pheochromocytoma, in such cases, the high risk of serious cardiovascular events, or death. American researchers claim that, despite the perfection of methods of diagnosis, approximately 1/3 of the patients with the diagnosis of pheochromocytoma is not possible to establish in life.
The mechanism of development of pheochromocytoma
The mechanism of development of pheochromocytoma due toexcessive blood entering the so-called catecholamines - natural physiologically active substances called adrenaline and noradrenaline. In most cases, both types of pheochromocytoma allocates catecholamines.
Some tumors produce only one of these catecholamines; rarely prevails dopamine.
Furthermore catecholamines, pheochromocytoma can produce serotonin and other hormones.
Concordance between the size of the tumor, the levelcatecholamines in blood and the clinical picture does not exist. Small tumors can produce and release into the blood large amounts of catecholamines, while large tumors metabolize catecholamines in its own tissue and produce only a small part of them.
Another figurative name pheochromocytoma -"Ten percent tumor" - due to the fact that 10% of cases it is malignant, at 10% - localized is the adrenal 10% - found bilateral involvement, 10% - combined with a hereditary disease and a 10% - found in children .
In 90% of cases occur in the medullary pheochromocytomaadrenal substance. Much rarer tumors are located outside the adrenal glands. Sporadic single pheochromocytomas are more often localized in the right adrenal gland, while the familial forms are bilateral multicentric. When bilateral tumors of the adrenal gland increases the probability of hereditary syndromes.
With timely radical surgerytreatment of pheochromocytoma is completely cured. The effectiveness of radical surgical treatment of pheochromocytoma in benign tumors is high and amounts to 95%. In cases of malignancy five-year survival reached 44%.
In the absence of severe hypertensive complicationscrises (coronary heart disease and heart failure, cerebrovascular accident, retinal detachment), according to the advanced cases of the disease, disabled patients after surgical treatment is completely preserved.