Neuroblastoma
Neuroblastoma is a malignant tumor of the sympathetic nervous system.
It is an undifferentiated malignancy consisting of small blood cells with dark-spotted nuclei.
Neuroblastoma has a number of properties that are absent in other malignant neoplasms, including:
• Ability to spontaneously regress – a malignant tumor can spontaneously regress, even in cases where it is large in size with a generalized spread of metastases.
• Ability to mature /differentiate/, rarely transformation into a benign tumor /ganglioneuroma/.
• Ability to rapidly progress aggressively and form metastases.
What are the symptoms?
Most of the tumor is found in the retroperitoneal space, most often in the adrenal glands. It is less common in the mediastinum and neck.
The clinical picture depends on the site of tumor formation, the localization of metastases, the amount of tumor tissue from which vasoactive substances are formed.
The main clinical manifestations of neuroblastoma include:
• Tumor in the abdominal cavity;
• Edema;
• Bone pain due to metastases;
• Anemia;
• Fever;
• Loss of body weight;
The most common complaints are pain – 30-35% of cases, fever – 25-30% of cases, unusual and unexplained weight loss – 20%.
When the tumor grows in the posterior mediastinum in children, constant cough, respiratory disorders, dysphagia and constant regurgitation are observed; deformation of the chest wall is observed.
Involvement of the bone marrow causes anemia and hemorrhagic syndrome. When the malignant tumor grows in the retrobulbar space, a characteristic “eye syndrome” occurs with exophthalmos, that is, protrusion of the eyeball.
If the neuroblastoma arises in the retroperitoneal space, it grows rapidly, reaching the spinal canal. On palpation, it is felt as a nodular motionless tumor mass with a stone consistency.
When the tumor spreads from the chest to the retroperitoneum through the diaphragmatic openings, it takes on the appearance of an hourglass or a dumbbell. Metastases of neuroblastoma in the skin have the appearance of blue-purple nodules with a dense consistency.
Neuroblastoma metastases most often form in the bones, bone marrow, and lymph nodes. Less often, they are localized in the skin and liver, and very rarely in the brain. The symptoms in this case are determined by which organ is affected by the metastases:
• Rapid increase in the size of the liver;
• Formation of light blue nodules on the skin;
• Pain in the bones;
• Swelling of the lymph nodes;
• Anemia, hemorrhages and other manifestations characteristic of leukemia.
What are the causes?
Neuroblastoma only occurs in children. The incidence is 0.85-1.1 cases per 100 thousand children aged up to 15 years. The average age is 2 years.
This disease is the most common malignant neoplasm in early childhood – 14%, in many cases it is detected at birth and can be accompanied by birth defects. As children grow, the probability of the appearance of this type of tumor decreases.
Treatment of neuroblastoma
The following methods are used as therapeutic methods to destroy and remove the tumor:
• Surgical method;
• Chemotherapy;
• Radiation therapy;
• Bone marrow transplantation;
In case of modern detection of the tumor, removal of the primary tumor node is performed. Usually, the malignant entity does not recur with remaining tumor cells, which also distinguishes neuroblastoma from other malignant tumors.
Chemotherapy of neuroblastoma includes the use of vincristine, cyclophosphamide, cisplatin, etc.
Radiation therapy is not prescribed in all cases, considering its danger to the body of small children.
Nevertheless, it is indicated for non-resectable tumors or metastases, poor effectiveness of the applied chemotherapy, and also for the destruction of possible future relapses.
