Prognosis and survival chances for children with neuroblastoma. Neuroblastoma in children: symptoms and reliable signs. Therapeutic monoclonal antibodies

Neuroblastoma is one of the most common malignant tumors that occurs in childhood. This tumor ranks fourth among neoplasms in children after leukemia, central nervous system tumors, malignant lymphomas and sarcomas. The tumor is detected at a fairly early age, most often from 1 to 3 years. Neuroblastoma in children can be localized in any part of the body, but practical significance It is localized behind the peritoneum and in the posterior mediastinum.

The causes of the disease are not fully understood. The only reliable factor is heredity.

Forms of neuroblastoma

There are 4 forms of neuroblastoma in children depending on the type of cells and location.

Melulloblastoma - the tumor is localized quite deep in the cerebellum, which makes it impossible to remove it through surgery. It is characterized by the rapid appearance of metastases, which leads to the rapid death of the child. The very first symptoms of tumor development manifest themselves in impaired coordination of movement and balance.

Retinoblastoma is a malignant tumor that affects the retina of the eye in children. The development of retinoblastoma leads to blindness and metastases to the brain.

Neurofibrosarcoma is a tumor of the sympathetic nervous system. Localized in the abdominal cavity, gives metastases to the bones and The lymph nodes.

Sympathoblastoma is a malignant tumor that is localized in the sympathetic nervous system and adrenal glands. Formed in the fetus during the formation of the nervous system. Sympathoblastoma can also be localized in chest cavity. Due to the enlargement of the adrenal gland tumor, the spinal cord can be affected, leading to paralysis of the limbs.

Stages of neuroblastoma

There are 4 stages of development of neuroblastoma in children:

Stage I - characterized by localization of neuroblastoma in the area primary focus;

Stage II – the tumor extends beyond the primary focus, but is not localized on the other side of the spine;

Stage III - the tumor spreads to both sides of the spine with damage to the lymph nodes;

Stage IV – neuroblastoma gives distant metastases to soft tissues, skeletal bones, lymph nodes, etc.

Symptoms of neuroblastoma

The first signs of neuroblastoma development in children are pallor skin, decreased apatite, vomiting, causeless whims, constipation. Possible appearance low-grade fever, increased heart rate, excessive sweating, hair loss. As the tumor develops and grows, symptoms increase. A pulse oximeter may be needed to measure your heart rate.

With retroperitoneal neuroblastoma, an increase in abdominal circumference is observed; upon palpation, a sedentary and lumpy tumor is palpable. The costal arch may be everted or a bulge may be visible in the lumbar region on the side of the tumor formation. You can often notice vascular network in the upper abdomen and chest. Retroperitoneal neuroblastoma is characterized by very early changes in a blood test, namely a decrease in hemoglobin levels.

Neuroblastoma in the mediastinum is initially asymptomatic. As the tumor progresses, its growth into spinal canal, which leads to paralysis of the upper and lower limbs, as well as urinary and stool incontinence.

Often parents notice signs of the disease that are caused not by the tumor, but by metastases. This is due to the fact that the tumor metastasizes quite early. Symptoms depend on the location of the metastases. When the bones of the skull are affected, tumor tubercles can be seen on the scalp, as well as protrusion of the eyes and hemorrhages. With metastases to the liver area, its enlargement and tuberosity are observed. Bone damage is characterized by pathological fractures.

Diagnosis of neuroblastoma

To diagnose a tumor, the child is examined with palpation of the abdomen, through which the tumor can be identified. They also prescribe blood and urine tests, perform ultrasound and X-ray examination, computer and magnetic resonance therapy. Final diagnosis diagnosed on the basis of a tumor biopsy and histological examination.

Treatment of neuroblastoma

Treatment of neuroblastoma includes three main areas: the use of chemotherapy, radiation therapy and operating method.

Usually carried out complex treatment neuroblastoma using several methods. The first and second stages of neuroblastoma are successfully treated with surgery. Although removal of retroperitoneal neuroblastoma is not always possible due to tumor growth into adjacent tissues and connection with main vessels. Sometimes it's possible partial removal tumors. Good effect has radiation therapy.

At proper treatment the probability of recovery in children with neuroblastoma of the first and second stages is quite high. The fourth stage has a poor prognosis of the disease; the five-year survival rate, even when using the most modern treatment programs, does not exceed 20%.

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Every parent needs to know what neuroblastoma is and how it progresses in childhood, because the health of children is the most important thing that can happen. Neuroblastoma is cancer, a tumor that begins its formation in the sympathetic nervous system. This disease is of embryonic origin, that is, the prerequisites for it arise even before the birth of the child.

When neuroblastoma occurs in children, the main signs are pain, weight loss and flushing. Metastases in such oncology can spread to any organs, blood, bones, as well as the lymphatic system. Diagnosis of neuroblastoma includes many examination methods. Treatment for this type of cancer involves surgery, radiation methods, as well as chemotherapy with stem cell transplantation.

When we're talking about about oncology, many parents are interested in what neuroblastoma is, because this type of cancer affects exclusively children. Neuroblastoma is the fourth most common tumor among oncological diseases in children, it affects eight out of a million children per year under the age of fifteen. Most patients with neuroblastoma are among two-year-old children, however, sometimes it can also be diagnosed in newborns, occurring together with congenital anomalies.

Tumor tissue develops from immature sympathetic cells of the nervous system (neuroblasts), which were supposed to become neurons. Due to certain disturbances in the prenatal period, the development of these cells stops, but their growth and division do not stop. The uniqueness of this pathology lies in the fact that the immature neuroblasts found in early age(up to three months) sometimes spontaneously mature, turning into full-fledged neurons.

The most common place for the primary development of a tumor is the adrenal glands, where it affects the nerve trunks, followed by the retroperitoneal space, and slightly less often cancer affects posterior mediastinum, pelvic area, neck. The disease spreads very quickly, primarily metastasizing to the bones and bone marrow, and distant lymph nodes. Rarely, the metastatic type of neuroblastoma can be localized in the kidneys. Neuroblastoma also occurs on the skin, but is rare.

A distinctive feature of this type of cancer is that neuroblasts can grow, divide, form a tumor that metastasizes, and then suddenly stops growing and disappears, no matter what size it was or how much it metastasized. That is, an independent regression occurs, a cure occurs without any intervention. Neuroblastoma can also mature into benign ganlioneuroma. Other neoplasms do not have this ability. Despite this, neuroblastoma requires mandatory treatment.

Causes of the disease

The exact causes of this form of cancer have not been identified. Hereditary factor is determined only in twenty percent of sick children. A tumor can arise before the birth of a child, or it can develop during growth, arising from immature neuroblasts. The reason for their immaturity is often genetic DNA mutations that arise for unknown reasons. The causes of neuroblastoma are being studied, but so far medicine has not made any progress in this regard.

Classification

This type of tumor is classified according to the location where it is most often observed, as well as according to the degree of differentiation it has. Based on location, the neoplasm is of four main types:

1. Melolloblastoma – localization of tumors – head. It is located deep in the cerebellum and is almost always not operable. This type characterized by aggressiveness, rapid metastasis and a high percentage of early mortality. In patients with neuroblastoma of this type, already at the beginning of its development, movement coordination is impaired. Neuroblastoma of the brain, like other types of neuroblastomas, does not occur in adults.
2. Retinoblastoma is found in the retina of the eye. The manifestation of the disease is a violation visual functions, up to complete blindness. Metastases with this type of tumor go to the brain.
3. Neurofibrosarcoma is a retroperitoneal neuroblastoma that metastasizes to lymph nodes and distant bones.
4. Sympathoblastoma is a neuroblastoma of the adrenal glands in children. It can also occur in the chest cavity and peritoneum. If the adrenal glands enlarge, paralysis occurs.

Depending on the degree of differentiation, the tumor can be of the following types:

• ganglioneuroma is a mature tumor arising from ganglion cells and has the most favorable prognosis, since it has a benign course;
• ganglioneuroblastoma - consists of cells that can be benign on one side of the tumor and malignant on the other;
• undifferentiated form – is completely malignant. The cells that make it up have round shape and dark spotted kernels.

No matter what kind they wear malignant diseases, they should be diagnosed as early as possible. Only in this case does the child have a better chance of life.

Stages of the disease

Neuroblastoma is, in most cases, a rapidly progressing tumor. In total, it has four stages of disease development, but some of them are divided into substages:

1. In the first stage, the tumor is small (no more than five centimeters) and there is only one. Spread to other organs and lymph nodes does not occur.
2. The second degree also does not metastasize, but it already becomes up to ten centimeters in size.
3. The third stage, depending on the size, is divided into 3A and 3B degrees. With grade 3A, the tumor size is no more than 10 centimeters, metastases do not spread to organs, but there is damage to nearby lymph nodes. With grade 3B, the tumor size is more than 10 centimeters, but neither the lymph nodes nor other organs are affected by metastases.
4. Stage 4 neuroblastoma is also divided into two subgrades. IV-A degree size may be different sizes, the condition of the lymph nodes is not determined, but metastasis spreads to distant organs. In grades IV-B, multiple tumors are observed that grow synchronously. It is impossible to assess the condition of the lymph nodes and distant organs.

There is another type of fourth grade tumor, which is classified with the letter “S”. This neoplasm is of neurogenic origin and has biological characteristics that are absent in similar neoplasias. The prognosis for such a tumor is favorable, with timely treatment it gives a good survival rate.

Symptoms of the disease

With neuroblastoma, symptoms can be very diverse, since the tumor can be localized in different organs. Signs of neuroblastoma in children depend on the location of the main tumor and metastases. At the first stage, the neoplasm is not expressed specific signs. Thirty percent of children experience symptoms such as pain at the site of the tumor and hyperthermia. Twenty percent of babies are underweight or starting to lose weight.

If the neoplasm is located in the retroperitoneal space, then sometimes it is possible to determine the presence of nodes in the abdomen by palpation. A little later, the tumor can metastasize through the intervertebral foramina, causing compression of the spinal cord. This leads to the development of compression myeloptia, which provokes functional disorders of the pelvic organs, for example, disturbances in the process of urination or defecation.

When the child’s breathing becomes difficult, a cough occurs, the baby often spits up, and his chest becomes deformed as the tumor enlarges. If the tumor is located in the neck, then the tumor can not only be felt, but also seen. Gorgen's syndrome often occurs, in which the following symptoms occur:

• drooping upper eyelid;
• pupil reduction;
• a deeper position of the eyeball in the orbit than normal;
• weak reaction of the pupil to a light stimulus;
• sweating disorder;
• redness of the skin on the face and conjunctiva.

All these signs are noted on the side on which the tumor is located.

With distant metastases, the signs are also varied. If the metastases have passed through the lymph flow, then there will be an enlargement of the lymph nodes. If the skeleton is affected, then the bones hurt. If the metastatic tumor is located in the liver, the organ becomes greatly enlarged and jaundice develops. If the bone marrow is affected, the following occurs:

• anemia;
• leukopenia;
• thrombocytopenia;
• lethargy;
• weakness;
• increased bleeding;
• susceptibility to infectious diseases;
• other symptoms that resemble acute leukemia.

If the skin is affected, dense nodular formations of blue, red or bluish color appear on their surface. Esthesioneuroblastoma (tumor in the nose) is accompanied by rhinitis and nasal bleeding. Also, during neuroblastoma, metabolic disturbances occur - the skin turns pale, diarrhea occurs, and intracranial pressure increases. After it has been carried out successful treatment, all signs lose their severity and gradually disappear completely.

Diagnostics

In pathological cases that are classified as cancer, it is necessary to diagnose and begin therapy as soon as possible. Mandatory laboratory test blood, including tumor markers (proteins produced by certain organs). Examination methods:

• bone marrow aspiration biopsy;
• trephine biopsy of the thoracic and ilium bones;
• biopsy of areas where metastases are suspected.

The material collected during the biopsy is sent for cytology, histology, as well as immunological and immunohistochemical analyses. In addition, the following is carried out:

• ultrasound diagnostics;
• chest x-ray;
• CT scan;
• Magnetic resonance imaging.

Rest diagnostic methods depend on the location of the tumor.

Treatment methods

Doctors develop a treatment plan only after full examination child and diagnosis. Basically, treatment is carried out comprehensively, including surgery, chemical and radiation therapy. Sometimes a child needs to take various drugs– antibiotics, antivirals, immunomodulators and other drugs, depending on clinical picture and complications of oncology.

Surgical treatment

Surgical removal of the tumor is most beneficial for the child’s body, but it is only effective for initial stage development of neuroblastoma and partially helps with the second stage. If multiple metastases occur, surgery will be ineffective. Sometimes during surgery, the organ on which the tumor is located, as well as surrounding tissues and lymph nodes, is partially or completely removed. Before and after surgery, especially in the later stages, chemotherapy is administered.

Chemotherapy

Medications chemical composition are very strong and can stop the growth of the tumor or kill it completely. However, such drugs, passing through the general bloodstream, have side effects. As a result of chemotherapy, a child may experience nausea, vomiting, and fever. The baby becomes weak and susceptible to infectious and viral diseases. After stopping therapy, all symptoms of side effects disappear.

Radiation therapy

Depending on the stage of the disease, doctors can carry out both external and internal exposure. This type of therapy is very aggressive towards children's body, but on late stages Without it, it is impossible to defeat the tumor. After irradiation, the baby loses hair not only on his head, but also on his entire body, including eyelashes and eyebrows. The baby becomes weakened, lethargic, loses his appetite, and may lose weight. Unfortunately, at stage 4B, radiation therapy does not always help either.

Forecast

The overall five-year survival rate for children with neuroblastoma is seventy percent. The higher the stage of oncology, the less chance of successful treatment. With the fourth stage of cancer in a child, the survival rate for five or more years is only forty percent, but in children under one year of age this figure reaches sixty. How younger child, the more chances he has even at high stages.

If treatment was carried out at the first or second stage, then neuroblastoma extremely rarely recurs. Relapses occur more often after treatment of the third stage of the tumor, but not with less frequency than with other forms of cancer. In children, stage 4 very often leads to relapse, which makes the prognosis unfavorable if the pathology is detected at a later stage.

Prevention

Unfortunately, there are no specific recommendations that, if followed, could prevent the development of neuroblastoma in a child, since the exact reasons for its occurrence are still unknown. If someone in the family has had cases of cancer, then when planning a pregnancy, parents should contact a geneticist, who, after conducting a series of studies, can, with some degree of probability, determine the risk of pathology in the unborn baby. During pregnancy, a woman should undergo screening every trimester.

Neuroblastoma in children most often affects renal tissue. Neuroblastoma of the right adrenal gland, as well as the left, can metastasize blood vessels, affect lymph nodes, internal organs (for example, liver) and affect skin cells. This type of malignant neoplasm has unique characteristics and an aggressive form of development.

Adrenal neuroblastoma in children is a malignant tumor that tends to grow rapidly.

Description and features

An adrenal tumor occurs during embryonic development and, as a rule, manifests itself at an early age. Development happens because pathological changes immature embryonic nerve cells. The cells mutate and do not mature to a full level, while continuing to divide and form a tumor.

Adrenal neuroblastoma in children has unique feature- in some cases disappears on its own.

A negative characteristic of neuroblastoma of the left and right adrenal glands in children is its aggressive and rapid development. This pathology of the adrenal glands in childhood is extremely rare and manifests itself before the age of 5. Ranks in abdominal area the child is quite a large space. In the first stages of development, it is quite difficult to detect a malignant formation; this is only possible through a series of studies.

Children with adrenal neuroblastoma do not have any symptoms of the disease. More often, a tumor is discovered by chance during an examination by a pediatrician or during X-ray and ultrasound examinations. Malignant formation is characterized rapid growth and for a short time may affect the spinal cord canal.

The development of adrenal neuroblastoma in the fetus is extremely rare. When performing an ultrasound examination of the fetus, neuroblastoma can be detected at the end of the 2nd trimester of pregnancy, most early diagnosis tumors were recorded at 26 weeks. In the fetus, the tumor is usually localized on one side. It is almost impossible to predict the further development of the tumor after diagnosis - the course ranges from independent regression to active spread of metastases.

Causes

The etiology and causes of the formation of adrenal neuroblastoma in embryonic and childhood have not yet been clarified. In the vast majority of recorded cases, the tumor occurs without visible reasons. In rarer cases, this is hereditary disease. Adrenal neuroblastoma, a hereditary form of cancer, is found in newborns and infants under 1 year of age.

Pathology occurs when the healthy maturation of embryonic cells changes. Mutation to cellular level leads to defective maturation of nerve cells, but they continue to divide, forming a malignant tumor. If the disease occurs before the age of one year, there is a chance that the tumor will spontaneously transform into a benign form and disappear over time.

Symptoms of the disease

At the first stages of development of adrenal neuroblastoma in children, no characteristic symptoms. For childhood minor ailments are considered as signs of other diseases characteristic of this period of life. The primary location of the tumor is usually located in the adrenal glands. The child may have bluish or red spots on the skin. This indicates damage to skin cells by metastases. The main characteristic symptoms in the presence of adrenal neuroblastoma in children:

• constant fatigue, drowsiness;
• increased sweating;
• increased body temperature for no apparent reason;
• enlarged lymph nodes, lumps in the neck and abdomen;
• abdominal pain, stool upset;
• poor appetite constant nausea, weight loss;
• pain in the bones.

Cancer cells produce hormones and cause pressure on organs. An expanded malignant formation in the retroperitoneal space can negatively affect work gastrointestinal tract. If metastases reach the bone marrow, the child becomes sick and weak. Cuts, even minor ones, cause heavy bleeding which is difficult to stop.

The course of development of adrenal neuroblastoma has conventional stages. This division of the development of the disease makes it possible to determine the most effective methods treatment. Stage 1 is characterized by a single tumor, the size of which (no more than 5 centimeters) allows surgery. There are no metastases or lesions of the lymph nodes. Stage 2A - the malignant neoplasm is localized, part of it is operable. There are no metastases at all or no signs of distant metastases. Stage 2 B - the development of metastases that affect the lymph nodes.

Stage 3 - a bilateral tumor appears. Stage 3 neuroblastoma is in turn divided into several classifications: with degrees T1 and T2 - single tumors no more than 5 centimeters and from 5 to 10 centimeters. N1 - lymph nodes are affected by metastases. M0 - no distant metastases. When diagnosing N, it is impossible to determine the presence or absence of metastases.

Stage 4 - the malignant tumor increases in size, metastasizes to the bone marrow, internal organs and lymph nodes. Stage 4A has a tumor of no more than 10 centimeters; sometimes it is impossible to determine the presence or absence of metastases. Stage 4 B has many synchronous tumors. It is impossible to determine the involvement of lymph nodes, as well as to assess the presence of distant metastases.

Oncological diseases, which include neuroblastoma, are among the most severe. Special meaning Such pathologies occur in pediatric practice.

What it is?

Neuroblastoma is one of the neoplasms. By classification oncological pathologies This disease is classified as malignant. This suggests that the course of the disease is very dangerous. Most often, these malignant tumors are recorded in newborns and children under three years of age.

The course of neuroblastoma is quite aggressive. It is impossible to predict the outcome and prognosis of the disease. Neuroblastomas - malignant neoplasms, which belong to embryonal tumors. They affect the sympathetic nervous system. Neuroblastomas usually grow rapidly.

However, doctors note one thing amazing property- ability to regression. According to statistics, neuroblastoma of the retroperitoneal space is the most common. It occurs, on average, in 85% of cases. Damage to the ganglia of the sympathetic nervous system due to intensive growth of the tumor is less common. Adrenal neuroblastoma occurs in 15-18% of cases.

The incidence in the general population is relatively low. Only one baby out of a hundred thousand gets neuroblastoma. The spontaneous transition of a malignant tumor to a benign one is also another mystery of this disease.

Over time, even with an initially unfavorable prognosis, the disease can develop into a more light form- ganglioneuroma.

Ganglioneuroma

Most cases, unfortunately, proceed rather quickly and unfavorably. The tumor is characterized by the appearance large quantity metastases that occur in a variety of internal organs. In this case, the prognosis of the disease worsens significantly. Various types of intensive treatments are used to eliminate metastatic cells.

Causes

Scientists have not reached a consensus on what causes neuroblastoma in children. There are currently several scientific theories, which provide justification for the mechanism and causes of occurrence malignant tumors in kids. Thus, according to the hereditary hypothesis, in families where there are cases of neuroblastoma, the risk of having babies who subsequently develop this disease increases significantly. However, the likelihood of this is quite low. It does not exceed 2-3%.

Some experts say that intrauterine infections may contribute to the appearance of various genetic abnormalities. They cause mutations in genes that lead to disruption of the coding of basic characteristics. Impact of mutagenic and carcinogenic factors environment only increases the possibility of having a child with neuroblastoma. Usually this condition is associated with a violation of the division and proliferation of adrenal cells during the period of intrauterine growth. “Immature” cellular elements are simply not able to perform their functions, which causes pronounced disturbances and deviations in the functioning of the adrenal glands and kidneys.

European researchers believe that the cause of neuroblastoma in children may be a genetic breakdown that occurs during the formation of the embryo. During the conception of a child, two genetic apparatuses merge - maternal and paternal. If any mutagenic factors act at this time, then mutations appear in the newly formed daughter cell. Ultimately, this process leads to impaired cell differentiation and the development of neuroblastoma.

Mutagenic or carcinogenic factors during pregnancy have a significant impact.

Thus, scientists have found that maternal smoking while pregnant increases the risk of having a child suffering from neuroblastoma.

Living in environmentally unfavorable areas or the strongest constant stress provides negative impact on the body of a pregnant woman. In some cases, this can also lead to the birth of a child with neuroblastoma.

Stages

Neuroblastoma can be localized in many organs. If the tumor has developed in the adrenal glands or in the chest, then they speak of sympathoblastoma. This form of the disease can cause dangerous complications. When the adrenal glands become too large in size, paralysis develops. If a tumor is present in the retroperitoneal space, they speak of neurofibrosarcoma. It causes numerous metastases, which are mainly localized in the lymphatic system and bone tissue.

Adrenal neuroblastoma

Neuroblastoma of the retroperitoneum

There are several stages of the disease:

• Stage 1. Characterized by the presence of a neoplasm up to ½ cm in size. At this stage of the disease there is no lesion lymphatic system and distant metastases. The prognosis at this stage is favorable. When conducting radical treatment The survival rate of babies is quite high.
• Stage 2. It is divided into two substages - A and B. It is characterized by the appearance of a neoplasm, measuring from ½ to 1 cm. There are no lesions of the lymphatic system or distant metastases. It can be eliminated by surgery. Stage 2B also requires chemotherapy.
• Stage 3. It is characterized by the appearance of a neoplasm larger or smaller than a centimeter in combination with other signs. At this stage, the regional lymph nodes are already involved in the process. There are no distant metastases. In some cases, the disease occurs without involvement of the lymph nodes.
• Stage 4. It is characterized by multiple foci or one solid large neoplasm. Lymph nodes may be involved in the tumor process. At this stage, metastases appear. There is practically no chance of recovery in this case. The prognosis is extremely unfavorable.

The course and development of the disease depends on many initial factors.

The prognosis of the disease is influenced by the localization of the neoplasm, its clinical form, baby’s age, cellular histological proliferation, stage of tumor growth and more.

It is very difficult to predict how the disease will progress in each specific case. Even experienced oncologists often make mistakes when talking about the prognosis of the disease and further outcome.

Symptoms

Signs of neuroblastoma may not be typical. The course of the disease alternates with periods of remission and relapse. During a period of complete calm, the severity of adverse symptoms is insignificant. The child can lead a normal lifestyle. During a relapse, the baby's condition deteriorates sharply. In this case, an emergency consultation with an oncologist and possible hospitalization is required. oncology department for treatment.

Clinical manifestations of neuroblastoma are numerous and varied. Typically, an extremely unfavorable course is accompanied by the appearance of several symptoms simultaneously. At the fourth stage of the disease, characterized by the appearance of metastases, clinical signs can be expressed significantly and significantly disrupt the child’s behavior.

Symptoms of neuroblastoma include:

• Difficulty swallowing. This sign is characteristic of neuroblastoma, which is located in the mediastinum. Difficulty swallowing leads to decreased appetite. Children begin to lag behind in physical development.
• The appearance of wheezing during breathing. They are detected by auscultation (listening) of the lungs. In the last stages of the disease, wheezing and difficulty breathing in the baby can be heard even without a stethoscope. This manifestation occurs if neuroblastoma develops in the chest.
• Severe and hacking cough. Usually nothing precedes its appearance. Cough appears independently, without connection with a viral or bacterial infection. Most often it is dry. Sputum is not separated.
• Chest pain. Appears when tumor tissue grows. This clinical sign characteristic of large neoplasms located in the mediastinum. The pain syndrome is of the same intensity both during inhalation and exhalation.

• Impaired intestinal motility. An actively growing tumor puts pressure on the organs of the gastrointestinal tract, disrupting their function. Impaired movement of the bolus of food through the intestines leads to constipation in the child, which practically cannot be treated with laxatives.
• Abdominal pain. The pain syndrome has nothing to do with eating. The baby may notice that there is a “pulling” or pain in the tummy. Active growth neoplasms only contribute to the progression of this symptom. In the final stages pain syndrome becomes unbearable.
• Appearance of a lump in the abdomen. A growing tumor can be felt. This symptom usually reveals pediatrician during a clinical examination of the baby and during palpation of the abdomen.

• Swelling of the limbs. On early stages it is manifested by excessive pastosity. Tumor growth and involvement of the lymphatic system in the process contribute to the progression of edema. They are loose in density and easily palpable.
• Increased or jumping blood pressure. This sign is characteristic of adrenal neuroblastoma. Normally, various hormones are formed in the cortex of this organ, which keep arterial pressure at the level normal values. When neuroblastoma appears, the function of the adrenal glands is disrupted, which is manifested by this clinical symptom.
• Increased heart rate. Tachycardia can be very pronounced. This sign also occurs in adrenal neuroblastoma. For normalization heart rate the prescription of b-blockers and other drugs for long-term use is required.

• Redness of the skin. This sign is very specific, however, it does not occur in all cases. Excessive redness of the skin may be the first sign of a tumor located in the adrenal glands.
• Slight increase in body temperature. Prolonged low-grade fever is often the reason why parents seek advice from a doctor. In this case, they do not even suspect that the child has neuroblastoma. An examination allows you to identify the disease and determine treatment tactics.
• Loss of coordination and gait. This symptom may indicate the appearance of metastases in bone tissue. To identify them, it is necessary to carry out additional diagnostics by using highly informative research. These include: computed and magnetic resonance imaging, ultrasound using Doppler scanning, and in some cases even radiography.
• Losing weight. Losing weight in a child over a short period of time should always be of great concern to parents.

If the baby eats well and fully, then there should always be a good reason for losing weight.

In some cases, this is caused by rapid growth of neoplasms and malignant tumors. To identify them, it is imperative to conduct a set of diagnostic studies.

Treatment

The choice of treatment tactics for neuroblastoma depends on many initial factors. Pediatric oncologists treat this disease. These specialists select required course therapy, which is discussed with parents. Often, to eliminate a tumor it is necessary surgery and chemotherapy. This combination treatment at certain stages it helps to stabilize the baby’s well-being and reduces the possibility further development diseases.

Neuroblastoma is a type of cancer that develops from postganglionic sympathetic neurons - nerve cells located in different parts of the body.

Neuroblastoma often occurs in the adrenal glands, glands that are similar in origin to nerve cells.

Neuroblastomas also appear in the chest, neck, abdomen, and pelvis—anywhere clusters of nerve cells exist.

According to American experts, neuroblastoma accounts for 8-10% solid tumors in children. This is the third most common type childhood cancer in the USA (after leukemia and brain tumors). The incidence is 1 case in 10,000 children under 15 years of age. Girls get neuroblastoma 30% more often than boys. In the United States, about 75% of neuroblastoma cases occur in children under 5 years of age; about 97% of cases occur in children under 10 years of age. In rare cases, neuroblastoma is detected during fetal ultrasound.

Some forms of neuroblastoma may go away on their own, others require complex and long-term treatment. The choice of treatment for neuroblastoma in each specific case will depend on many factors.

Causes of neuroblastoma

Neuroblastoma originates in neuroblasts - immature nerve cells, which are formed in the fetus as a normal part of its development process. Subsequently, neuroblasts transform into nerve fibers and the cells that form the adrenal glands. Most neuroblasts mature by the time the baby is born, but some studies show that newborns have a small amount of immature neuroblasts. Subsequently, neuroblasts will mature or disappear. But some of them can form a tumor - neuroblastoma.

It is not entirely clear what causes genetic mutation leading to neuroblastoma. Researchers believe that this mutation occurs already during intrauterine development or soon after birth, because the vast majority of patients are children in the first years of life.

Risk factors for neuroblastoma

Signs of neuroblastoma

1. Neuroblastoma in the abdominal cavity (the most common form):

Pain and discomfort in the abdomen.
. Palpable formation under the skin.
. Changes in intestinal motility.
. Edema of the lower extremities.

2. Neuroblastoma in the chest:

Wheezing.
. Chest pain.
. Chronic cough.
. Swallowing disorder.

Other possible symptoms of neuroblastoma include:

Suspicious nodules under the skin.
. Unexplained weight loss.
. Temperature increase.
. Dark circles around eyes.
. Horner's syndrome (ptosis, miosis and anhidrosis).
. Retention of stool and (or) urine.
. Lower back pain.
. Paraplegia.
. Ataxia.

If you notice any suspicious symptoms or changes in your child’s behavior, show him to a specialist!

Complications of neuroblastoma

Diagnosis of neuroblastoma

Urine and blood tests. Test results will help rule out other causes of your child's symptoms. A urine test may reveal high level catecholamines, which are produced by neuroblastoma cells.
. Visualization. X-rays, ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) make it possible to scan the patient's body and see the tumor.
. Taking a tumor sample. If a formation is found, the doctor will need to take a piece of tissue for testing. This procedure is called a biopsy. Special lab tests make it possible to determine the type of cells that make up the tumor, as well as the specific genetic characteristics of cancer cells. Using this data, the doctor will develop the optimal treatment plan.
. Bone marrow biopsy. This procedure is performed to detect tumor metastases in the bone marrow. Bone marrow is a spongy tissue found inside large bones. It takes part in the formation of blood cells. Neuroblastoma sometimes metastasizes to the bones and bone marrow. A bone marrow biopsy is performed using a thick needle that is inserted into the bone.

Determining the stage of neuroblastoma

To clarify the stage of the disease, X-rays, isotope bone scans, CT and MRI, as well as a number of other methods are used.

Based on the results, the stage of the disease is determined:

Stage I: In the first stage, neuroblastoma is limited to a specific area. In this case, the tumor can be easily removed surgically.
. Stage IIA. At this stage, the neuroblastoma is still limited to a specific area, but cannot be completely removed by surgery.
. Stage IIB. Neuroblastoma is limited to a specific area and may (or may not) be removed surgically. Neighboring lymph nodes already contain cancer cells.
. Stage III. At the third stage, the tumor reaches a significant size and cannot be removed surgically. There are no metastases.
. Stage IV. Most late stage cancer. At this stage, neuroblastoma metastasizes to distant parts of the body.
. Stage IVS. This stage is a special category for neuroblastoma, which behaves differently from other forms of tumor. Stage IVS neuroblastoma occurs in children younger than 1 year of age. It metastasizes to various organs - mainly the liver, skin and bone marrow. Despite the many organs affected, the chance of recovery is high. Stage IVS neuroblastoma sometimes goes away on its own without treatment.

Neuroblastoma Treatment Methods

1. Surgical treatment of neuroblastoma.

Surgical removal is the main treatment for neuroblastoma. For low-risk neuroblastoma, surgery alone may be sufficient. Opportunity complete removal tumor depends on its size and location. The tumor may be small, but its location, say, near the spinal cord, makes surgery very risky. For moderate to high-risk neuroblastoma, the surgeon will try to remove most of the tumor. Chemotherapy or radiation is then prescribed to kill any remaining tumor.

2. Chemotherapy for neuroblastoma.

Used in chemotherapy special drugs that kill cancer cells. For the treatment of neuroblastoma, carboplatin, cisplatin, etoposide, adriamycin, and cyclophosphamide are prescribed. Chemotherapy drugs have a detrimental effect on cells that multiply quickly - cancer cells. But they suffer along with them hair follicles and cells of the gastrointestinal tract, which leads to common side effects - hair loss, nausea, diarrhea, etc.

Children with low-risk neuroblastoma that cannot be removed American doctors It is recommended to prescribe chemotherapy. Sometimes chemotherapy may be given before surgery (neoadjuvant) to shrink the tumor and make surgery easier. In other cases, chemotherapy remains the only treatment option.

For neuroblastoma medium degree risk is often recommended surgery in combination with a course of chemotherapy. At high risk, patients usually receive large doses of chemotherapy to kill any cancer cells that may metastasize.

3. Radiation therapy for neuroblastoma.

Radiation therapy uses high-energy rays to destabilize and destroy cancer cells. The rays can damage any cells they hit, so your child will be protected from surrounding tissue during the procedure. But it is impossible to completely avoid the consequences of radiation. Side effects radiation exposure can be quite serious, including the risk of future cancer.

Patients with low- and intermediate-risk neuroblastoma may be given radiation if surgery and chemotherapy do not help. If the risk is high, radiation may be used after chemotherapy and surgery to reduce the chance of cancer coming back.

4. Stem cell therapy.

Western experts recommend stem cell transplantation (autogenous stem cell transplantation) for children at high risk. Every person's bone marrow produces stem cells that mature and develop into full cells blood - erythrocytes, leukocytes and platelets.

The child undergoes a procedure in which the blood is filtered to collect stem cells. After this, high doses of chemotherapy kill the remaining cancer cells in the child's body. Stem cells are injected into the blood, where they migrate into the bones and begin to produce new, healthy blood cells.

5. The latest methods of treating neuroblastoma.

Newer methods include the use of monoclonal antibodies and vaccines, which trigger the body's immune response against the tumor. Modern targeted drugs are able to selectively act on cancer cells by recognizing their unique proteins.

Immunotherapy using ch14.18, a monoclonal antibody against the cancer-associated disialoganglioside GD2, is one the latest methods fight against neuroblastoma. Recent studies have shown that immunotherapy using ch14.18, GM-CSF and interleukin-2 has significant benefits compared with standard therapy.

Konstantin Mokanov