Neuroblastoma in children – causes, symptoms, diagnosis, treatment

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Unusual abdominal pain in a child may be a symptom of a dangerous disease – a developing cancer. It can be detected by ultrasound of the abdominal cavity. The problem, however, is that this test is performed very rarely for children.

– Prophylactic ultrasound examinations in children would allow for early detection of congenital defects and tumors developing in the abdominal cavity in the initial stage – says Prof. Danuta Perek, head of the Oncology Clinic at the Children’s Memorial Health Institute in Warsaw. Unfortunately, this is not done due to the high cost.

Neoplastic diseases in children

Cancer in children is rare, in Poland it is about 1200 new cases a year. However, they are very dangerous. In infants, it is the third cause of death, after birth defects and infections, and in children over 5 years of age, the second cause of death after injuries and accidents.

Little patients develop the most malignant types of cancer. Therefore, in children, the course of the disease is very rapid. – There are cancers that can double their mass within 17 hours – says prof. Perek. – Because children develop cancer rarely and pediatricians may not have to deal with them throughout their practice, early diagnosis of the disease is a huge problem – he adds.

Neuroblastoma – causes

Neuroblastoma is a cancer that only occurs in children. At the time of diagnosis, 50 percent. patients are under the age of 2, 75 percent. under 4 and 90% under 10 years of age.

Neuroblastoma develops from immature cells in the nervous system, more precisely from a part of it called the sympathetic nervous system. It works without our will and is responsible for preparing the body for the “fight or flight” reaction in stressful situations. It regulates the heartbeat, dilates the pupil, can release adrenaline or inhibit the secretion of gastric acid, saliva and sweat.

The ganglia of the sympathetic nervous system run along the spine and are also found in the medullary adrenal glands. It is not known why the cells of this system turn into cancer in some children. The primary tumor may be localized in the paraspinal area along the axis of the body and in the adrenal gland, i.e. in the abdominal cavity, mediastinum, neck area and pelvis.

The cause of neuroblastoma in most cases is the spontaneous mutation of cells that begin to divide uncontrollably. In addition, sometimes changes are also found in the structure of chromosome 1, i.e. a structure composed of DNA strands wound on proteins (histones), which are associated with a more aggressive course of the disease and a worse prognosis.

When speech after the formation of neuroblastoma is assumed the so-called “Theories of two” strokes, according to which, in addition to the congenital mutation, another must be acquired in order for the tumor to develop. In the case of neuroblastoma, the n-MYC gene is multiplied, which is responsible for the maturation of cells, their division and the formation of new vessels. At this point, it should be noted that such a mutation has a worse prognosis.

In addition, it should be added that according to statistics, the risk of developing the disease increases when a pregnant woman, for example: is abusing alcohol, uses sex hormones or is treated with diuretics (water tablets). In turn, folic acid taken by a woman during pregnancy and before conception may reduce the risk of developing neuroblastoma.

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Neuroblastoma – stages

The Evans tumor advancement system is adopted, in which there are five stages:

1. Stadium I neuroblastomy – the tumor is limited to one organ, where its complete removal is possible.
2. Stadium II neuroblastomy – in this case, the tumor infiltrates beyond the organ in which it developed, but does not exceed the midline of the body. Lymph nodes on the side where the cancer grows may or may not be involved.
3. Stadium III neuroblastomy – at this stage, the tumor already crosses the midline of the body, and there may or may not be metastases in the ipsilateral lymph nodes.
4. Stadium IV neuroblastomy – distant metastases through the bloodstream to internal organs: lungs, liver, brain, soft tissues and distant lymph nodes.
5. Stadium IVS neuroblastomy – a small tumor of the adrenal gland (as in stages I and II), metastases to the liver, bone marrow or skin, without damage to the bone structure.

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Symptoms of neuroblastoma

The symptoms of neuroblastoma can resemble those of other medical conditions. These can include low-grade fever, weight loss, abdominal pain, cough, shortness of breath, chest pain, and anemia. This delays the diagnosis of the disease. Meanwhile, the detection of the disease at an early stage gives almost 95 percent. chances of recovery.

However, it should be added that the symptoms of neuroblastoma are different and depend on the location of the primary focus.

If the tumor is present in the abdominal cavity, the characteristic symptoms will be abdominal pain, loss of appetite or vomiting, mentioned earlier.

In the case of a tumor in the area of ​​the spine, this will put pressure on the spinal cord and cause symptoms such as back pain, muscle atrophy, dysfunction of the bladder and anal sphincter, or an increase in reflexes.

On the other hand, a tumor in the neck area may give symptoms of Horner’s syndrome (pupil narrowing, the drop of the eyeball into the eye socket, drooping eyelid), and exophthalmos, swelling of the eyelids and optic disc, strabismus, hematomas in the area of ​​the eye sockets (so-called hematomas) glasses).

The tumor that develops in the posterior mediastinum has a long latency period and symptoms include shortness of breath, coughing, wheezing, difficulty swallowing, and recurrent pneumonia.

A tumor in the small pelvis is associated with symptoms such as difficulty passing stools and urine.

In addition to the symptoms resulting from the primary location of the tumor, there are also symptoms associated with the excessive production of catecholamines at the neuroblastoma. We are talking here about e.g. accelerated heart rate, headaches, increase in blood pressure, excessive sweating, redness of the face, often associated with excessive production of VIP (a substance that in excessive amounts causes abdominal pain or diarrhea).

– If the accidentally detected tumor is, for example, 2,5 cm in diameter, only surgical intervention solves the problem. If there is already the development of the tumor found at the medical examination and there are bone metastases, the chance drops to 15%. – says prof. Perek. Unfortunately, half of the children who come to us for treatment already have metastases – he adds. The first symptoms of neuroblastoma may appear after the cancer has attacked the bones. – If a two-and-a-half-year-old child who has already walked suddenly stops, curls his legs and does not want to get up and has no other symptoms, it may be a symptom of this disease. The “spectacled” hematomas around the eyes are also specific for this tumor.

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Neuroblastoma – diagnosis

– 75 percent the primary focus of neuroblastoma is located in the abdominal cavity. This means that it can be detected in an ultrasound scan – explains Prof. Perek. A tumor in the abdominal cavity may manifest itself, inter alia, in constipation, vomiting or diarrhea. – That is why a child who looks sick and behaves like a sick for no apparent reason, has unusual abdominal pain, anemia – an ultrasound of the abdomen should be performed. Prophylactically, it would be best to do these tests for all babies, right after birth and then every 4-6 months. In fact, every pediatrician should be able to perform this test without restrictions, emphasizes prof. Perek.

In addition to abdominal ultrasound, in the case of neuroblastoma, a number of other tests are also carried out, including: urine tests (especially for increased concentration of catecholamine metabolites), blood tests, computed and positron tomography, magnetic resonance imaging, biopsy and scintigraphy (i.e. which introduces radioactive isotopes into the body).

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Treatment of the neuroblastoma

Treatment of neuroblastoma in the first and second stage of the disease is primarily the removal of the tumor. In the case of inoperable tumors, chemotherapy is used first to reduce its size. It also prevents or treats metastases. In stage III of the disease, metastases to bones, liver or other organs occur. At this stage, stronger chemotherapy is used, as well as radiotherapy and possibly surgery. It should be added, however, that in over 50% of cases, the tumor metastasizes.

In the fourth stage of the disease, no treatment is undertaken, but the patient is closely monitored. This is the case in infants who have a small tumor in the adrenal gland and metastases to the liver, skin or bone marrow (data show that spontaneous regression of the disease may occur, but this is very rare).

It should be added that in the treatment of neuroblastoma (especially in the high-risk group), retinoid therapy (13-cis-retinoic acid) is also used. According to the data, the experimental anti-GD2 immunotherapy, whose task is to stimulate anti-cancer activities in the cells of the immune system, brings very good results. However, it should not be forgotten that the key to curing neuroblastoma is the correct diagnosis and treatment as soon as possible.

The prognosis for this neoplasm also depends on the age of the child. Infants up to a year are curable in almost ninety percent. In children from the age of two years, the chances of recovery beyond the advancement of the disease depend on a number of other factors. Regardless of the prognostic factors, about 60% of them are now healed. sick children.

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Side Effects of Neuroblastoma Treatment

Neuroblastoma can cause long-term side effects. Most side effects depend exactly on the type of treatment your child has, the location of the tumor, and the child’s age during treatment. Children who have received 3 or more treatments (surgery, radiotherapy, chemotherapy, etc.) are more likely to experience serious long-term side effects from treatment, so it is very important to monitor your child’s condition after recovery for these symptoms.

Side effects of treating neuroblastoma can include:

1. hearing loss;
2. bone and muscle problems such as scoliosis (curvature of the spine);
3. thyroid problems;
4. problems with growth and development;
5. fertility problems;
6. neurological problems;
7. other cancers, including leukemia;
8. emotional or psychological problems.

In very rare cases and for unknown reasons, in some children with neuroblastoma, the body’s immune system attacks the child’s normal nervous tissue. This can lead to problems such as learning disabilities, muscle development delays, language problems, and behavioral problems. This is called myoclonus-opsoclonus syndrome.

Children with lumps in the neck or chest and eye problems or muscle spasms may need further treatment with corticosteroids, intravenous immunoglobulin (IVIG), or other medications.

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Neuroblastoma and the risk of disease recurrence

In the case of neuroblastoma, it is possible for the cancer to recur, unfortunately it is currently unclear what can be done to reduce the risk of this happening. However, it can help to adopt healthy behaviors, such as not smoking, eating well, exercising regularly, and maintaining a healthy weight. These types of behavior can have a positive effect on your baby’s health, beyond the risk of developing neuroblastoma, but also other cancers.

Neuroblastoma and emotional and social problems in sick children

The diagnosis of cancer in a child is a crisis for the whole family. Younger children may not remember much of their experiences after treatment ends, but getting through them and helping them cope with the situation can be a huge challenge. Older children may have a hard time staying away from school, friends, and the activities they enjoy, in addition to dealing with the stresses of treatment. In

Most pediatric cancer centers have special support programs that help children with cancer during treatment and for many years afterwards. It should be added that in such cases not only the sick children themselves suffer, but also their parents and other family members, especially siblings, can also be affected, both emotionally and otherwise.

Some common family problems include stress over treatment funding, traveling to and close to an oncology center, family members taking time off from work, possible job loss, and the need to study at home. Social workers and other specialists in cancer centers can help families solve these problems.

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