Among the malignant neoplasms of the sympathetic nervous system, ganglionicoblasts account for more than 40% of cases, which puts them at the second position in this group of tumors. Most often ganglioneuroblastoma affects children, whose age is within the age range of four to ten years.
A special conversation deserves a combined ganglioneuroblastoma, the structure of which( almost identical to the ganglioneuromus structure) contains areas consisting of germinal nerve cells( neuroblasts).
Neoplasms of this type are found in children of middle and senior school age, as well as in young people under the age of twenty.
What is Ganglioneuroblastoma?
Ganglioneuroblastoma is a malignant neurogenic neoplasm consisting of neuroblasts and ganglion cells characterized by different levels of differentiation.
Externally, this malignant neoplasm, which does not have a capsule, resembles a node of gray or gray-pink color with necrotic areas and multiple foci of hemorrhages.
Causes and Symptoms of the
The specialists in the field of neurology still do not have accurate data on the causes that give impetus to the development of ganglioneuroblastoma.
It is known only that it appears as a result of malignant ganglioneuroma.
Ganglioneuroblastoma is an intermediate position between this benign neoplasm and an extremely malignant and aggressive neuroblastoma.
The clinical features of ganglionic fibroids are determined by the localization of the tumor process:
- Ganglioneuroblastoma, located in the retroperitoneal space of , manifests itself as a whole complex of common symptoms:values exceeding 38 degrees and a reduced amount of red blood cells and hemoglobin in the blood. Another characteristic manifestation of malignant tumoral process is the presence of periorbital edema. Due to the violation of the outflow of the lymphatic fluid, it is most often localized between the eyes, provoking the appearance of the so-called symptom of the eyeglasses - bruises under the eyes. Excessive content of physiologically active substances in the blood - noradrenaline, adrenaline and dopamine - entails the development of tachycardia( rapid heartbeat), arterial hypertension and hyperhidrosis( increased sweating).Patients often experience hyperemia of the skin, leading to reddening of the face, as well as chronic diarrhea.
- For ganglioneuroblastoma of the mediastinum, the respiratory function is abnormal: patients suffer from shortness of breath, severe cough, periodic attacks of asphyxia( suffocation).Their breath( doctors call it stenotic, or stridor) becomes very noisy: wheezing, rife with wheezing. The complex of the above symptoms usually arises either as a result of the operation of the reflex mechanisms, or because of the neuropathic paresis of the larynx, which develops as a result of compression of the vagus nerve and its branches with the tissues of the growing tumor. The symptomatology of the disturbance of respiratory function appears suddenly and develops extremely rapidly - in a few days. Neoplasm in this case, as a rule, is small in size, does not affect the lungs and does not lead to the displacement of mediastinal tissues. If the tumor compresses the structures of the upper chest sympathetic nodes, the clinical picture is supplemented by a violation of the function of sweating( dyshidrosis), the appearance of anisocoria( pupils of different sizes) and Horner's syndrome, manifested by a whole complex of symptoms: enophthalmos( displacement of the eyeball inside the orbit), ptosis) and miosis( narrowing of the pupil).
- Ganglioneuroblastoma, localized in the brain tissues, manifests itself as a symptom of an intracerebral tumor. Focal symptoms are reduced to the violation of precisely those functions for which control was responsible for the affected area of the brain. Memory, attention, hearing, vision, smell, sensitivity and motor functions of the body can be affected. The degree of expression of these manifestations can be different.
Ganglioneuroblastomas have a high capacity for the formation of metastases. Metastasis of gangliononeblastoma of the brain is limited to the limits of the central nervous system, that is, cancer cells can affect only the spinal cord and brain.
Ganglioneuroblastoma mediastinum and retroperitoneal space metastasize to the tissues of bones, liver( and a number of other internal organs) and lymph nodes.
In the clinical course of ganglioneuroblastoma, the following stages are distinguished:
- Malignant neoplasm of stage I is limited to the limits of the affected organ.
- Tumor II stage leaves the body that became the focus of primary lesion, but does not have time to spread to the lymph nodes.
- Ganglioneuroblastoma III stage, breaking the median line, involves in the tumor process lymph nodes located on both sides of the spinal column.
- Tumor of the IV stage leads to significant damage to the body due to the appearance of metastatic foci in the tissues of bones and internal organs.
Diagnosis of a tumor
- Diagnosis of Ganglioneuroblastoma begins with a laboratory examination of blood and urine. The presence of a tumor is indicated by a high level of prostaglandins and catecholamines( norepinephrine, dopamine), as well as products formed as a result of their disintegration in the delivered samples.
- X-ray survey in this case is not an informative method, as the data obtained from this study can be only an indirect evidence of the presence of a tumor in the patient's body. An X-ray in ganglioneuroblastoma of the retroperitoneal space will only indicate the presence of paravertebral calcifications and some stretching of the intestinal loops. A tumor localized in the mediastinum appears on the roentgenogram as a dense rounded shadow.
In order to visualize the tumor, modern diagnostic methods are used, performed on a very complex and expensive medical equipment:
- multispiral computed tomography of internal organs( in this study, the tomograph table moves continuously and its tube rotates all the time);
- ultrasound examination of the abdominal cavity;
- pneumomediastinography - the method of radiographic examination of the mediastinum, involving the introduction of nitrous oxide or oxygen into the mediastinal fiber;
- computed tomography of organs localized in the thorax;
- for suspected brain tumor perform magnetic resonance imaging of this organ.
To exclude the presence of metastases or confirm their presence, resort to:
- scintigraphy of the skeleton;
- ultrasound examination of the liver;
- puncture biopsy of lymph nodes.
The most reliable, undisputed method for detecting ganglioneuroblastoma is a laboratory study of tissues taken from the patient during the biopsy.
During the stereotactic biopsy, the skin and soft tissues of the head are cut, and a tiny hole is inserted into the bones of the skull, designed to insert a special needle. Bringing it to a strictly defined place, take a sample of the brain tissue. After the needle is removed, the wound is sutured.
Taken sample is subjected to a thorough histological examination.
The presence of immature small neurocytes with different degrees of differentiation, large multinuclear neurocytes( their cytoplasm contains vacuoles), small cysts and necrotic areas is a confirmation of the fact that the brain of the patient is ganglionic fibroblasts.
Ganglioneuroblastoma must be differentiated from malignant tumors with a small-cell structure: for example, from metastatic tumors, lymphomas and glioblastomas.
Treatment of gangloneuroblastoma
For the treatment of ganglionioroblastoma, complex therapy is used that provides for surgical intervention, radiation therapy and the use of medication.
The leading value is given to surgical treatment:
- The operation to remove gangliononeblastoma of the brain is performed by a neurosurgeon. The procedure of surgical intervention in this case fully corresponds to the principles used in the course of operations on the brain.
- The operative removal of ganglioniformoblast, localized in the retroperitoneal space and mediastinum, is performed by a thoracic surgeon. If the tumor does not have time to germinate in the tissues of surrounding structures, an operation of its radical removal is performed, consisting in excision of the malignant neoplasm. In this case, resection of adjacent tissues is not carried out. If the tumor process has already spread to surrounding tissues, the tactics of advanced removal of ganglionioroblastoma, involving resection of affected lymph nodes and fatty tissue, are applied. If for any reason a complete removal of the tumor is not possible, to reduce the pressure exerted by its expanding tissues on adjacent anatomical structures, perform a palliative operation consisting in partial resection of ganglionioroblastoma.
To improve the results of surgical treatment, chemotherapy is used:
- In the process of searching for the most effective therapeutic regimens, monohimotherapy was first used, consisting of taking cyclophosphamide, doxorubicin, vincristine, and a number of other drugs that suppress the activity of tumor cells.
- More recent practice has proven the effectiveness of such cytotoxic agents as ifosfamide and carboplatin.
- Today( in the most severe cases), chemotherapy courses carried out with high doses of cytotoxic drugs precede an operation to transplant the bone marrow.
For treatment of adolescents and patients of adolescence, radiotherapy sessions are sometimes used, although the progressive development of antitumoral drug treatment has almost brought their meaning to naught.
Low demand for radiotherapy is also due to the fact that ganglioneuroblastomas most often develop in the body of young children whose irradiation can lead to extremely dangerous distant radiation lesions.
Developing treatment tactics for each specific case, a team of treating specialists( consisting of a surgeon, chemotherapist and radiologist) must take into account two extremely important factors:
- prognostic signs found in small patients in the process of diagnosis;
- the response of a malignant neoplasm to the treatment being undertaken.
Forecast
- The 2-year survival rate of patients with ganglioneuroblastoma on average does not exceed 70%.
- The most favorable prognosis are tumors localized in the region of the posterior mediastinum. Survival of such patients is at the level of 80%.
- Survival in ganglioneuroblastoma located in the retroperitoneal space is no more than 60%.
- In the case of early detection of a tumor, and also after its radical removal, the two-year survival rate of patients is at least 95%.
- Ganglioneuroblastoma of a widespread nature, as well as giving metastases leaves a chance for two years of survival to only half of the patients.