Benign, as well as malignant tumors of the adrenal hormone-active type is commonly called pheochromocytoma, but sometimes this term is used to designate neoplasms outside the adrenal glands in chromaffin tissue.
Adrenal pheochromocytoma is a difficult to diagnose disease, therefore it is assigned a separate position among other diseases associated with endocrine glands. And although official data only 0.0001% of the population suffer from this disease, that is, it occurs in every ten thousandth person, but in reality these figures are somewhat different. So in the course of pathological studies, the tumor of pheochromocytoma is detected at a frequency of 0.3%, and in hypertensive patients - in 1% of patients. According to different countries, pheochromocytoma was diagnosed in 40-76% of cases only at autopsy.
In 3/4 the disease occurs only in one adrenal gland - in the right, in 1/10 - in both glands, in the remaining cases, outside glands, in the abdominal cavity. Malignant pheochromocytoma is not found more often than in every tenth case, and metastases are even less frequent.
Symptoms of
Symptoms of pheochromocytoma rarely show themselves to the fullest. The main thing that is guided by is arterial hypertension, the clinic of which can be episodic or permanent. If a pheochromocytoma clinic episodic, then most often it will be provoked by stress, overeating and increased physical exertion.
In a pheochromocytoma, a medical history consists of bursts of symptoms, including high blood pressure, a sudden disappearance or a transition to the opposite. For example, hypertension after an attack can go into hypotension.
Clinic of pheochromocytoma, like adrenal neoplasms, during an exacerbation consists of such symptoms as:
- Cramps in the legs;
- Discomfort in the abdomen;
- Increased sweating;
- Nausea;
- Vomiting;
- Pulsating headache;
- Pale skin.
In addition, the tumor of the adrenal gland can manifest as bouts of anxiety, loss of consciousness, seizures, a constant need for caffeine, a syndrome of hyperventilation.
In severe cases, releases of catecholamines lead to neuropsychiatric, cardiovascular, endocrine, hematologic and gastrointestinal disorders, such as:
- Salivation;
- High blood ESR;
- Hyperglycaemia;
- Vascular injury of the fundus;
- Hypogonadism;
- Heart failure;
- Neurasthenia;
- Psychoses.
Pathogenesis
From the point of view of medicine, pheochromocytoma, the causes of which are still unknown, but analyzing the pheochromocytoma of the adrenal glands, doctors hypothesized the existence of genetic prerequisites for this disease. The clinic of the disease provides its development at any age, but the greatest number of cases is fixed in the interval from 25 to 50 years. If the localization of the pheochromocytoma tumor is in the adrenal cortex, then 90% that it is benign, if out of the glands, then the chance of malignant formation grows to 30%.
Pathogenesis is largely characterized by the location of the tumor. If the formation is outside the adrenal glands, then the body releases norepinephrine, leading to such manifestations as high blood pressure, bradycardia. If the tumor is in the gland, adrenaline is thrown into the blood, an excess of which leads to tremor, hyperexcitability, dilated pupils, hyperglycemia, tachycardia, high blood pressure.
Diagnosis
Because the tumor intensively produces catecholamines, the assays are aimed at detecting this substance in the adrenals, bronchi and tissues of the urinary system. The detection of catecholamines is most often performed using the silvering method of Grimalius and Gamperl-Masson.
In addition, laboratory diagnosis of pheochromocytoma is aimed at assessing the catecholamine content in the secreted urine throughout the day, in which vanillandelic acid and epinephrine can also be present.
The second part of the diagnosis is monitoring of fluctuations in blood pressure in the patient. It is also necessary to monitor the activity of the CAS, paying special attention to persistent cardiac rhythm disturbances and cardiopathies. A very important part of the diagnosis of neoplasm in the adrenal glands is given to topical diagnosis, for which scintigraphy is used in combination with metaiodobenzylguanidine, a radioactive substance. If the size of the lesion is more than 2 mm, it is possible to use a method such as ultrasound. Also for these purposes, CT and MRI are effectively used. No less important in the diagnosis of neoplasm is the definition of a malignant one or benign, and in the case of cancer try to identify metastases.
Treatment of
Treatment of the disease consists of two stages, and often includes both medicamental and surgical effects. To surgical influence on a pancreatic tumor it is possible to proceed only after normalization of pressure in admissible norms. As a drug preoperative treatment, such A-adrenoblockers as tropafen, phentolamine and phenoxybenzamine are used.
Depending on what features adrenal gland has, various methods of surgical intervention can be used. To provide the necessary manipulation in the process of surgical intervention, use extraperitoneal, transthoracic, transperitoneal or combined access.
If the neoplasm is a single benign or malignant tumor without metastases, then this treatment is successful in 85% of cases and does not lead to a relapse.
For the treatment of pheochromocytoma with multiple adrenal tumors, the most appropriate option is to remove all neoplasms, but since such surgery has a high level of risk to the patient's health, surgical interventions are performed in several stages.
Pheochromocytoma crisis
Both malignant and benign tumors are often detected in patients with hypertension, while arterial hypertension can be constant with pheochromocytoma, and can manifest itself in the form of periodic crises. Hypertensive crisis with pheochromocytoma is manifested by tachycardia, headache, unconscious fear, fever, frequent urination, hearing or vision impairment, sweating.
During a crisis caused by a tumor of the adrenal glands, the skin becomes sharply pale in patients, and sweating becomes profuse, the limbs become colder, and the pupils dilate, the blood pressure rises to 240/140.After such an attack a person usually feels a strong weakness.
Pheochromocytoma in children
This neoplasm in children is rare, and has a non-studied etiology. The main reason for the development of adrenal tumors is seen in the genetic factor. The most common pheochromocytoma in children occurs outside the adrenal gland or has a two-sided shape. An interesting factor of neoplasms in children is that although in older people the tumor is more common in women, then in childhood this type of education is more likely for boys. At the same time, there is absolutely no difference between which parent the child inherited predisposition to neoplasms in the adrenal glands. Often in children, such neoplasms are combined with tumors of the mucous membranes, intestines and thyroid cancer. Children's pheochromocytoma is often associated with Hippel-Lindau, Sippl and Recklinghausen diseases.
Malignant pheochromocytoma
Malignant pheochromocytoma is a hormonally active tumor. It is formed by the adrenal medulla. Quite often it is two-sided. For today in medicine there is no unequivocal answer concerning a character of an origin of a similar neoplasm.
The disease can develop at any age. However, most often such a diagnosis is made for patients aged 20 to 50 years. The percentage of patients with different sexes is almost identical. Slightly less often a tumor develops in children. The most dangerous age in this case is the period from 10 to 11 years.
Of the total number of malignant tumors, pheochromocytoma accounts for 15% of cases. According to medical statistics, a third of patients with this diagnosis die before the disease was diagnosed. The reason for this is the similarity of symptoms with a variety of other ailments. This specificity is due to the amount of adrenaline secreted by the tumor. Therefore, only highly skilled specialists who can use the differential diagnosis can establish the disease.
Another feature of malignant pheochromocytoma is the different character of the symptomatology in children and adults.
The predominant part of the neoplasm is a single tumor of the adrenal medulla. Of all the reported cases, 10% of tumors are located outside the adrenal glands. About 3% are formed in the cervical region, as well as the thorax. Twenty percent of tumors are plural.
Classification of pheochromocytoma according to ICD 10
According to the international classification of diseases, malignant pheochromocytoma is assigned code D35, which denotes the neoplasm of other and unspecified endocrine glands.
Separately, according to the location of the tumor, ICD 10 classifies diagnoses:
- Code D35.0 indicates adrenal neoplasm;
- D35.1 refers to the tumor of the parathyroid [parathyroid] gland;
- D35.2 - so the new formation of the pituitary gland is ciphered.
And so on. In total, the ICD 10 includes 9 different types of tumors.
Separated coded according to the international classification of diseases, Sipple Syndrome. This ailment is characterized by multiple pheochromocytomas.
Often this tumor is combined with neurofibromatosis. With this diagnosis, a separate code is also used.