Neuroblastoma is a tumor formation of the sympathetic nervous system, which is of a malignant nature and develops mainly in young children. Neuroblastoma is characterized by unusual features. On a number of occasions, it manifests itself as an aggressive neoplasm, which has the ability to rapidly grow and metastasize. But there are cases when the tumor opposite, spontaneously regresses,( eventually disappears without treatment).Sometimes its cells mature themselves, as a result of which, malignant formation turns into a benign - ganglioneuroma.
Neuroblastoma in children
Neoplasm is detected mainly in toddlers from 1-3 years old, as well as in newborns. Neuroblastoma can sometimes make itself felt, even before the birth of a child, in the womb of the mother.
In this case, it can be detected only on ultrasound.
It is able to develop in any part of the child's body, in the neck, sternum, hitting the middle part of the chest. However, it often manifests itself in one of the adrenal glands, both organs are infrequent.
For newborns, as well as children, whose age does not exceed 1 year, malignant neoplasm has the most favorable prognosis than in cases with older children, regardless of the severity of the disease. In children, the tumor can regress itself, without treatment.
The main thing is that the child should be diagnosed correctly, as a result of which, he will be sent for treatment to the department of oncology-hematology, under the supervision of specialists who have experience in this field.
Stages and forms of the disease
There are 6 stages of this disease, due to which the prognosis is determined, as well as the disease management strategy:
- I - localized formation, lymph nodes from 2 sides of the body are not affected;
- IIA - localized formation, lymph node involvement is absent;
- IIB - one-sided formation, lymph nodes are present, on the same side of the body;
- III - the formation moves to the other side of the body, metastatically affects nearby lymph nodes, as well as the formation of one side, the manifestation of metastases in the lymph nodes from the opposite side of the body, or the median neoplasm caused by metastases from one and the other side of the lymph nodes;
- IV is a neoplasm that spreads with distant metastasis in the organs( bone marrow, bones and lymph nodes), in addition to the cases that refer to the stage described below;
- IVS is a primary localized neoplasm corresponding to the definitions of stages I and II.
At this stage, there is a metastatic spread in the liver, bone marrow and skin. The child at the same time arrives at an age that does not exceed 1 year.
There are several forms of the disease, it directly depends on the type, as well as the location of the cells:
- Medulloblastoma - this form of neoplasm is localized in the depth of the cerebellum, so it can not be surgically removed. It quickly grows, metastasizes and the child quickly dies in this case. Determine the emergence of medulloblastoma can be based on signs of a violation in the child's balance and loss of coordination of motion;
- Retinoblastoma is a malignant formation that affects the eye retina. As a result, the child may completely lose sight. Metastases give their roots to the brain;
- Neurofibrosarcoma - this kind of neoplasm appears in the sympathetic nervous system. His metastases affect the bones, as well as lymph nodes;
- Sympatoblastoma - this kind of formation is manifested in the adrenal glands, as well as the sympathetic nervous system. It can develop, at the time of the formation of the nervous system in the embryo.
Sympatoblastoma is able to express itself in the area of the sternum. If the neoplasm in the adrenal glands is added in size, it can speak of a spinal cord injury followed by paralysis of the limbs.
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What are the causes of the appearance of neuroblastoma
To date, the exact reasons for the appearance of this type of malignant tumor have not been determined. However, there is a fact that it is somehow interconnected with changes in the pathological nature of DNA cells, as well as with congenital pathologies.
When a child has a defect in embryonic cells that are the precursors of nerve cells, they are not able to become full and instead, tumor foci are formed.
Symptoms and signs of the disease
Initial signs of a neoplasm may be similar to the manifestation of simple pediatric diseases of various kinds.
This is closely related to the fact that the tumor with its metastases can sometimes affect several areas of the child's body at once. Disorders of metabolism, is explained by the growth of the lesions of the neoplasm.
The clinical picture of malignant formation mainly depends on where it appeared, as well as the localization of metastases and the content of vasoactive substances in it.
The neoplasm in the region of the neck, sternum, pelvis and abdominal cavity, at the time of germination, squeezes around the underlying organs, which is characterized by a corresponding symptomatology.
To which, the following phenomena are attributed:
- There are palpable nodules;
- There is a syndrome "Horner";
- Respiratory functions are impaired;
- Veins are contracted.
The appearance of malignant formation in the abdominal region may be indicative of tumor masses that have arisen. If the disease struck the pelvic area, then a micturition process is manifested.
Clinical symptoms are based on the following manifestations:
- Fever;
- Malignant neoplasm in the peritoneum;
- Anemia;
- Painful sensations in the bones,( cause of this metastasis);
- Significant weight loss;
- Puffiness;
As a result of tumor growth in the region of the posterior mediastinum, children complain of constant regurgitation, constant cough, respiratory distress, dysphagia. Sometimes there is deformity of the sternum. If a malignant lesion affects the bone marrow, anemia appears, as well as a hemorrhagic syndrome.
Symptoms of tumor metastasis
If the tumor has released its metastases, then in newborns, the liver begins to grow at a fast pace, with the formation in rare cases of nodules on the skin with a bluish-bluish hue, as well as bone marrow damage.
In older children, the growth of metastases causes a sensation of pain in the bones, as well as an increase in the size of the lymph nodes. The disease in rare cases can manifest signs, the same as in the case of leukemia.
Symptoms of tumor modification
Changes occur with growth of the tumor and lead to the appearance of similar symptoms. When the level of catecholamines, and occasionally also of the vasoactive peptides rises, children may have attacks of excessive sweating, the skin becomes pale and this is accompanied by hypertension or a loose stool.
Symptoms may occur regardless of the site in which the tumor was formed. Its intensity can decrease as a result of the correct approach to treatment.
Diagnosis of neuroblastoma
Diagnosis is performed to identify this disease, as well as its degree.
For this it is required:
- To pass the daily volume of urine;
- Carry out a bone marrow puncture;
- To make a reanobiopsy of the wing of the ileum or sternum;
- ultrasound;
- Ultrasound;
- Computed tomography;
- Radiography;
- Abdominal aortography and veinography.
All these activities are appointed by the doctor as necessary for him to discretion.
With the help of these all studies, it is possible to accurately diagnose the disease and identify the stages of its development. After this, the doctor can prescribe the appropriate treatment.
Methods for treating neuroblastoma
There are many ways to treat this terrible disease. Before appointing one of them, a team of specialists in this field, hold a meeting, as a result of which, they come to a common opinion and prescribe a course of treatment.
Medication Therapy
For the treatment of this disease, the following drugs may be prescribed by the doctor;
- Cytoxan;
- Holoxan;
- Temodal;
- Nidran;
- Icebreaker;
- Vincristine;
- Bleocin, etc.
Surgical method
The surgical method for the treatment of neuroblastoma is used in most cases, depending on the location of the tumor and the extent of its prevalence. The surgeon during the operation removes the maximum tumor formation. If you can not remove it, for some reason, a biopsy is performed.
Chemotherapy
Chemotherapy is performed with the help of special cytotoxic agents. By their action they contribute to the destruction of the neoplasm or slow down the process of its growth.
There are several ways to penetrate chemo drugs into the bloodstream:
- Taking drugs inside, in the form of medicines;
- Introduction to the vein;
- Drug administration by intramuscular injection.
After getting the drug into the body, tumor formations are subject to destruction. If you enter the drug into the spinal canal, the organ that is affected by the neoplasm, or into any of the body cavities, then this chemotherapy will have a name - regional.
When choosing a method of chemotherapy, take into account the type of tumor, as well as the stage of its development. Sometimes it is necessary to use several chemotherapeutic drugs at once, such chemotherapy is called - combined.
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Radiotherapy and high dose chemotherapy with stem cell transplantation
Chemotherapy is prescribed in such doses in large doses. As a result, hematopoietic stem cells are destroyed, which eventually replace with new ones. They are taken from the bone marrow of the child( donor cells can also be used), after which they are preserved by freezing.
At the end of chemotherapy, the cells are returned back to the patient, by the intravenous route. The introduced cells mature and contribute to the formation of new ones.
Radiotherapy
Radiotherapy is used to treat a tumor using X-ray high-frequency radiation. With the help of this method it is possible to achieve complete disposal of malignant cells, as well as to stop their further growth.
Radiation therapy is of two types:
- The is a procedure in which the device's rays are directed directly to the tumor;
- Internal view of - for carrying out the procedure, radioactive substances are filled with needles, tubes and catheters, they are injected into tissues that are near the neoplasm, or immediately into it.
The method of this therapy is chosen by the doctor, depending on the stage and type of malignant process.
Treatment with the help of the monoclonal antibodies
For this type of treatment, antibodies are used that are made in laboratories from one kind of cell type. They are designed to recognize specific formations on tumor cells and other substances. They have the ability to define specific types of tumor in tumor cells.
Antibodies have properties intertwined with neoplasms, killing malignant cells and blocking their growth in the future. Monoclinic antibodies enter the body of the child by means of indium.
Physiotherapy
Physiotherapy - performed to achieve good results, as a result of the main treatment. This method is the most innocuous of all. Treatment in this way is prescribed by the doctor, to each patient separately, depending on the severity of the disease.
Methods of treatment can be the following:
- Treatment with laser therapy;
- Low-frequency laser therapy;
- Cryotherapy;
- Thermal radiation and other mechanical effects;
- Myostimulation;
These are the most commonly used methods, there are a number of others.
Prognosis and consequences
Prognosis of the disease depends on timely diagnosis of the tumor, from its stage and age of the child. Important are the genetic characteristics of tumor cells, as well as the area of localization of the primary neoplasm.
The consequence of treatment may be the secondary formation of neuroblastoma. It can appear many years after successful treatment. A consequence of this kind is called a belated side effect. Therefore, at the end of treatment activities, the patient should be observed at the doctor constantly.
In our time, the development of neuroblastoma can not be prevented, because, risk factors that could be avoided, are not defined. If there were any cases associated with this in the family, then the child can only develop a tumor in rare cases.
In the video case, the progression of neuroblastoma: