Ganglioneuroblastoma
Ganglioneuroblastoma is characterized by a gray or gray-pink tumor node, devoid of a shell, has necrotic areas and hemorrhages. It is considered to be the result of an inferior modification of ganglioneuroma and is in an intermediate position between a malignant neuroblastoma and a benign tumor. Modern medicine and neurology do not have accurate information about the causes that lead to the appearance of ganglioneuroblastoma. A significant number of morbidity is observed in children 4-10 years old. Separately, there is a combined ganglioneuroblastoma, which has the structure of a ganglioneuroma with spaces of neuroblasts, which occurs in children older than 10 years.
The content of the article:
Symptoms of ganglioneuroblastoma
Diagnosis of ganglioneuroblastoma
Treatment of ganglioneuroblastoma
Prognosis of ganglioneuroblastoma
Ganglioneuroblastoma
Taking into account the degree of organ damage and localization, such stages of ganglioneuroblastoma are distinguished:
Stage I — the neoplasm does not cross the boundaries of the organ in which it was formed;
Stage II — the tumor goes beyond the boundaries of the primary organ of the lesion, but does not extend to the lymph nodes;
Stage III — the neoplasm crosses the middle line and expands to the lymph nodes on both sides of the spine;
Stage IV — the tumor significantly damages the body, which is manifested by metastases in the liver, bones and other organs.
Symptoms of ganglioneuroblastoma
Ganglioneuroblastoma most often affects the mediastinum, brain and retroperitoneal space. Therefore, patients with this pathology will develop various symptoms.
Symptoms of retroperitoneal ganglioneuroblastoma
Retroperitoneal ganglioneuroblastoma has no specific signs and is manifested by common symptoms — anorexia, weight loss, fever, anemia, periorbital edema may also be noticed. Since ganglioneuroblastoma is accompanied by an increase in catecholamines in the blood, their excessive amount leads to the formation of such clinical manifestations as hypertension, redness of the face, increased sweating and tachycardia.
Symptoms of mediastinal ganglioneuroblastoma
Ganglioneuroblastoma of the mediastinal organs is characterized by a violation of the respiratory system, which is manifested by coughing, shortness of breath, an attack of asphyxia, stridor or stenotic breathing. These symptoms are caused by a reflex mechanism and neuropathic weakening of the larynx, which occurs when the tumor compresses the nerve or its branches. These signs appear unexpectedly and develop rapidly within a few days. At the same time, the neoplasm does not provoke a displacement of the structure of the mediastinal organs, has a small size and does not extend to the lungs. In the case of pressure of ganglioneuroblastoma on the upper thoracic nodes, the following clinical signs may appear:
different pupil size (anisocoria);
ptosis of the upper eyelid (Gorner’s symptom);
violation of sweating;
miosis (pupil dilation);
deep position in the orbit of the eyeball.
Symptoms of ganglioneuroblastoma of the brain
Ganglioneuroblastoma of the brain is manifested by a violation of those functions that are controlled by the damaged area. This may be a violation of hearing, vision, attention, memory, sense of smell, tactile and motor functions of the body of various levels of severity. Regardless of the location, ganglioneuroblastoma contributes to the deterioration of the entire body, which is accompanied by sweating, fever, weight loss, bone pain and loss of appetite. Ganglioneuroblastoma often metastasizes. When the tumor is located in the brain, metastases expand within the boundaries of the nervous tissue, with extra-cerebral damage they pass into lymph nodes, bones and other organs.
Diagnosis of ganglioneuroblastoma
First of all, a laboratory method is used — the determination of the content of catecholamines in the blood and the products of their cleavage in the urine. X-ray examination methods make it possible to obtain only indirect information about the tumor, therefore, chest CT, abdominal ultrasound, MRI of the brain, pneumomediastinography or multisection spiral tomography are performed to visualize the neoplasm, which make it possible not only to identify ganglioneuroblastoma, but also to determine its size, location and the nature of its effect on other tissues and organs.
Due to the dynamic metastasis of the neoplasm, it is necessary to examine the surrounding organs to detect tumor metastases. For this purpose, bone scintigraphy and radiography, lymph node biopsy and liver ultrasound are used.
The final diagnostic method for determining ganglioneuroblastoma will be a microscopic examination of a sample of the neoplasm obtained during a biopsy or intraoperatively. Stereotactic biopsy is used to determine ganglioneuroblastoma of the brain. During histological examination, small and large neurocytes of different differentiation, small cysts and areas of necrosis are detected in the preparation. Combined ganglioneuroblastoma has a structure similar to ganglioneuroma and is characterized by the presence of foci that consist of neuroblasts. Ganglioneuroblastoma should be distinguished from such substandard neoplasms with a small cell structure as metastatic tumors, glioblastoma, and lymphoma.
Treatment of ganglioneuroblastoma
Treatment of ganglioneuroblastoma consists in surgical excision of the neoplasm. Elimination of ganglioneuroblastoma of the brain is performed by a neurosurgeon according to the rules of surgery for brain tumors. Surgical intervention for tumors of the retroperitoneal space and mediastinum is performed by a surgeon. In the case of the spread of ganglioneuroblastoma to the surrounding tissues, its radical removal is performed. Most often, an extended removal is performed, which includes excision of lymph nodes and regional fatty tissue. If it is impossible to completely remove the ganglioneuroblastoma, partial resection (palliative removal) is performed in order to decompress its anatomical structure.
Surgical treatment of ganglioneuroblastoma is combined with an individually selected combination of chemotherapy drugs. The course of chemotherapy is prescribed to each patient of different duration and dose, which is due to the severity of the tumor.
Prognosis of ganglioneuroblastoma
According to clinical studies, the survival rate of patients with a tumor for two years is 70%. A tumor that is located in the mediastinum has a favorable prognosis, with a survival rate close to 80%, and with retroperitoneal ganglioneuroblastoma — at the level of 60%. After radical surgery and with a non-spread tumor, patients live for more than two years in 95% of cases. In the presence of metastases and the widespread nature of ganglioneuroblastoma, the survival rate for several years is only 50%. Since the causes of the formation of this pathology have not been established, there are no special preventive measures that could prevent its occurrence. The most important thing will be timely diagnosis and therapy of pathology.
