Ganglioneuroblastoma is a malignant neurogenic tumor containing neuroblasts and ganglion cells at various stages of differentiation. Disease is characterized by anorexia, weight loss, fever, symptoms of excess catecholamines (increased blood pressure, sweating, facial hyperemia, tachycardia). Depending on the localization of the tumor, symptoms of compression of the abdominal cavity or mediastinum, focal neurological symptoms may be observed. The diagnosis is based on the visualization of the tumor using ultrasound, CT, MRI, MSCT and its morphological verification in the study of biopsy material. The most effective way to treat ganglioneuroblastoma is radical or extended removal of the tumor.
ICD 10
C47 Malignant neoplasm of peripheral nerves and autonomic nervous system
General information
Ganglioneuroblastoma is a gray-pink or gray tumor node devoid of a capsule, having foci of hemorrhages and necrotic areas. Among the tumors of the sympathetic nervous system, ganglioneuroblastoma ranks second in frequency of occurrence. It accounts for about 40% of tumors in this group. In half of the cases, ganglioneuroblastoma is located in the retroperitoneal space, 40% of its localization is in the posterior mediastinum. Ganglioneuroblastoma of the central nervous system is a rare type of tumor and accounts for only 0.5% of cases.
Ganglioneuroblastoma is the result of a malignant transformation of ganglioneuroma and occupies an intermediate position between this benign tumor and an extremely malignant neuroblastoma. To date, neurology and medicine in general do not have accurate information about the causes leading to the development of ganglioneuroblastoma. Disease occurs most often in children, mainly in the age range from 4 to 10 years. Separately, there is a so-called combined ganglioneuroblastoma, which has the structure of a ganglioneuroma with sections of neuroblasts. Such ganglioneuroblastoma occurs mainly among patients older than 10 years, and the average age of the patients is 20 years.
Symptoms
Retroperitoneal ganglioneuroblastoma is manifested by common symptoms: weight loss, anorexia, anemia, fever. Periorbital edema may be observed. Since ganglioneuroblastoma is accompanied by an increase in the blood of cotecholamines (adrenaline, dopamine, norepinephrine), clinically, symptoms caused by their excess are often observed: increased sweating, hypertension, tachycardia, redness of the face, sometimes chronic diarrhea.
Mediastinal ganglioneuroblastoma is characterized primarily by a violation of respiratory function: shortness of breath, cough, stenotic breathing or stridor, attacks of asphyxia. These symptoms are more often caused by reflex mechanisms and neuropathic laryngeal paresis, which occur when the vagus nerve or its branches are compressed by a tumor. They are distinguished by the sudden appearance and rapid development of the clinic within a few days. In this case, the tumor may be small in size, not cause displacement of mediastinal structures and not spread to the lungs. If ganglioneuroblastoma exerts pressure on the upper thoracic sympathetic nodes, then in the clinical picture there is a violation of sweating, anisocoria (different pupil size), Gorner syndrome: drooping of the upper eyelid (ptosis), pupil dilation (miosis), enophthalmos.
Ganglioneuroblastoma of the brain has a clinical picture of an intracerebral tumor with focal symptoms corresponding to its location. As a rule, it metastasizes within the central nervous system. Ganglioneuroblastoma of extra-cerebral localization gives metastases to lymph nodes, bones, liver and other internal organs.
Diagnostics
In the laboratory diagnosis, it is important to determine the content of catecholamines in the blood and their metabolites (vanillylmindal acid and homovanilinic acid) in the urine.
X-ray examination allows you to obtain only indirect data for the tumor. When it is localized in the retroperitoneal space, an overview radiography of the abdominal organs reveals stretching of the intestinal loops and paravertebral calcifications, when the tumor is located in the mediastinum, a rounded dense shadow, local expansion of intercostal spaces, the presence of “usur” on the ribs at the places where they adjoin the tumor can be detected on the chest radiography.
In order to visualize ganglioneuroblastoma, MSCT or ultrasound of the abdominal cavity, CT of the chest, pneumomediastinography, MRI of the brain are used. Radiography and scintigraphy of the skeleton, ultrasound of the liver, puncture biopsy of the lymph node, etc. are used to detect tumor metastases.
Finally, ganglioneuroblastoma can be diagnosed only by microscopic examination of a tumor sample obtained intraoperatively or during a biopsy. Stereotactic biopsy can be used in the diagnosis of the brain. Histological examination in the case in the preparation reveals small immature neurocytes with varying degrees of differentiation, large multinucleated neurocytes with vacuoles in the cytoplasm, areas of necrosis and small cysts. Combined ganglioneuroblastoma is characterized by a structure similar to ganglioneuroma, a distinctive feature is the presence of foci consisting of neuroblasts, which may or may not have signs of differentiation.
Ganglioneuroblastoma needs to be differentiated from other malignant neoplasms with a small-cell structure, for example, from lymphoma, glioblastoma and metastatic tumors.
Treatment
Removal of ganglioneuroblastoma of the brain is performed by neurosurgeons in accordance with the principles of operations for brain tumors. Surgical intervention in ganglioneuroblastoma of the mediastinum and retroperitoneal space is performed by surgeons and thoracic surgeons. At the same time, if the ganglioneuroblastoma does not germinate into the surrounding tissues, then its radical removal is carried out. However, more often it is necessary to perform an extended removal of the tumor, including resection of adipose tissue and regional lymph nodes. In cases where the ganglioneuroblastoma cannot be completely removed, palliative removal of the tumor, i.e. its partial resection, is performed to decompress the anatomical structures surrounding it.
Surgical treatment is combined with chemotherapy with vincristine and cyclophosphamide. In older children, radiation therapy may be used. In severe cases, a bone marrow transplant may be required.
Forecast
The average 2-year survival rate of patients with ganglioneuroblastoma is 70%. Clinical observations have shown that ganglioneuroblastoma localized in the posterior mediastinum has a more favorable prognosis. With it, the 2-year survival rate is approaching 80%, while with retroperitoneal ganglioneuroblastoma, it is at the level of 60%. Patients with nonproliferated ganglioneuroblastoma and after radical surgery live longer than 2 years in more than 95% of cases. With the widespread nature of the tumor and the presence of metastases, the 2-year survival rate is only 50%.
