Neuroblastoma is a malignant tumor that is growing of the fetal neuroblasts of the sympathetic nervous system. In 99% it affects children before the age of 15 years. Middle age of the patients is 2 years old.
90% of patients are children before the age of 5 years. Neuroblastoma is the most common oncological illness among the kids before the age of 1 year.
It remains unknown what are the causes of neuroblastoma. External factors that can increase or decrease the risk of this illnessare not found.
There is a familial form of neuroblastoma observed among 2 % of patients. Familial neuroblastoma is characterized by the primary occurrence of the tumor in two or more locations. Hereditary background impacting the development of other forms of neuroblastoma is not identified.
Neuroblastoma can be located in different parts of the body. Therefore, it is able to cause various clinical symptoms. The most common location of the neoformation are retroperitoneal space (adrenal glands) and the abdominal cavity.
Basic groups of neuroblastoma symptoms are:
Pain. Usually, the tumor is not painful by itself, including on the palpation. But it can reach large sizes and compress the surrounding tissues. As a result, pain syndrome occurs.
Sympathicotonia. Irrespective of the neuroblastoma location, child can have seizures, associated with the increased influence of the sympathetic nervous system on the body. Due to the release of catecholamines and vasoactive intestinal peptidesthe increase in blood pressure, paleness of skin, diarrhea with liquid stools, fever are observed.
Symptoms of vascular compression. Depending on the location of the tumor, child may experience swelling in the legs, scrotum, face, hands, neck, and upper chest. Fluid can accumulate in the pericardium and there will be heart failure symptoms.
Symptoms of nerve compressioncan damage different functions such as:breathing, swallowing, limbs movements (if neuroblastoma is located close to the spine).
Other symptoms depend on the tumor location and the disease prevalence. It can be:
Early diagnosis is difficult because symptoms do not appear at the beginning of the disease.Age of the patient often does not allow him to express complaints of feeling unwell, and even more so to specify them.At the time of the visit to the doctor two-thirds of patients already have metastasis in the lymph nodes.
Following methods are used for the neuroblastoma diagnostics:
Laboratory methods. 85% of patients have the increased levels of catecholamines (to the greatest extent dopamine) in the blood. Diagnostics is held by determining the daily urinary excretion of hormones and their metabolites.
There are also other biochemical markers of neuroblastoma:
Imaging methods. Ultrasound, computed tomography,magnetic resonance imaging, and X-rays are used for the neuroblastoma diagnostics. These methods of diagnostics help to detect the neuroblastoma and its metastasis.
Biopsy is done on the final stage of neuroblastoma diagnostics. With the needle a doctor takes a sample of the tumor mass for cytological and immunohistochemical studies.
Bone marrow puncture is doneto assess the prevalence of the tumor process. Usually, it is done simultaneously with neuroblastoma biopsy while a child is under general anesthesia.
Scintigraphy. Radioactive isotopes are injected into the body. If neuroblastoma is suspected, the MIBG (meta-iodobenzyl guanidine) is used. This material in the chemical structure resembles catecholamines. It accumulates in the tumor cells irrespective of where they are situated. In a few days or hours child’s body is scanned and neuroblastoma location is identified with all its metastasis.
Neuroblastoma treatment includes:
Alternatively to the radiation therapy, in addition to remote irradiation, the MIBG-therapy is used. It presupposes the intravenous injection of the meta-iodobenzyl guanidine. It is a radioactive chemical that accumulates in the tumor cells irrespectively of their location and destroys them.
The procedure is done in the special shielded room. Patient remains completely isolated, as he is radioactive and hazardousduring the treatment period. Isolation lastscouple days.
Patients with the prevalent neuroblastoma forms can be indicated the highly intensive chemotherapy that presupposes the use of high doses of the medications. Taking into consideration the stage of the disease, good results of treatment can be achieved.
Disease-free period is:
Four-year disease-free period can be observed among 30% of patients after the intensive chemotherapy and stem cells transplantation (bone marrow).
New ways of neuroblastoma treatment are constantly developed. The medical achievements of the last years are:
Retinoids. The use of retinoids (isotretinoin) can significantly reduce the risk of tumor recurrence.
Targeted therapy is based on the implementation of medications (crizotinib) that influence just the cells with the changed ALK gene. As a result, the tumor is reduced in sizes. ALK gene changes are characteristic for the 15% of neuroblastomas.
Immunotherapy.Monoclonal antibodies (dinutuximab) are used for the neuroblastoma treatment. They stimulate the immune system activity against the tumor cells. Research showed that monoclonal antibodies are effective for some children, who are resistant to chemotherapy.
Prognosis depends on the risk group. Risk group can be low, moderate or high and is determined by the combination of parameters:
Five-year survival prognosis, depending on the risk group:
If neuroblastoma was established on the stage 4, the five-year survival is about 20%. In case of the early diagnosis establishment (stage 1) five-year survival is about 100%.
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