Neuroblastoma

The Neuroblastoma is a cancer and the second most common malignant growth in children after brain tumors. In Germany around 150 children are affected by neuroblastoma every year, with the survival rate strongly depending on the stage of the tumor.

What is Neuroblastoma?

A neuroblastoma is a solid, malignant tumor that develops from degenerate cells of the sympathetic nervous system. This nervous system controls, among other things, the cardiovascular system or the intestinal activity.

If the immature cells of this nervous system degenerate, a neuroblastoma forms. The degeneration of the immature cells begins prenatally. The tumor occurs most often in the adrenal medulla (approx. 50 percent of all cases) or on both sides of the spine in the so-called borderline. If this trunk is affected, the tumor can develop along the entire spine, i.e. in the abdomen, chest or neck area.

Neuroblastomas can be confined to the site of origin or they can be spread. H. Form metastases. Metastases are not limited to individual locations and can appear in the lungs, kidneys, brain, or lymph nodes. Neuroblastomas have the peculiarity of regressing spontaneously in some cases, which doctors have not been able to find a reason for.

In over 50 percent of all cases, neuroblastomas occur within the first 15 months of life, with boys being more frequently affected than girls. The risk of developing neuroblastoma decreases with age. However, adolescents and adults can rarely be diagnosed with neuroblastoma.

causes

Neuroblastomas are caused by the degenerate cells of the sympathetic nervous system. Since the degeneration often occurs before birth, gene mutations and spontaneous chromosome changes can be the reason for the cell change.

Genetic inheritance has not yet been proven, but there are families in which neuroblastomas occur more frequently (approx. 1 percent of cases). Nutrition during pregnancy, stress and environmental factors have not yet been proven to be the causes of cell degeneration.

Symptoms, ailments and signs

• in the neck area: 15 to 20 percent of the patients have Horner's syndrome
• in the chest area: shortness of breath
• in the abdominal area: abdominal pain, back pain, urinary tract, intestinal problems
• Pain, paleness, fever, and weight loss
• possibly high blood pressure or diarrhea

Diagnosis and course

With neuroblastoma, many patients initially have no symptoms at all. These occur later with advanced tumor growth or metastasis. If symptoms occur, they usually depend on the location of the tumor.

Abdominal pain, fever, diarrhea, sore throat, tiredness and a general feeling of illness can occur. However, since these are all unspecific symptoms, the doctor has to use various aids such as magnetic resonance imaging, X-rays, computed tomography and laboratory tests for diagnosis.

If a tumor is discovered, further examinations can clarify whether individual organs are affected. The final confirmation of the diagnosis is obtained through a tissue examination of the tumor.

To detect or exclude the formation of metastases, an MIBG scintigraphy is carried out in which a radioactive substance is injected into the patient's bloodstream.

Complications

Neuroblastoma leads to a wide variety of complaints and complications. As a rule, the further course of this disease depends very much on its cause and the affected region of the body. For this reason, it is usually not possible to make a general prediction about the further course.

In most cases, those affected suffer from diarrhea and high blood pressure. It can also lead to a fever and weight loss. Not infrequently, a neuroblastoma also leads to pain in the abdomen and back and can have a very negative effect on the patient's digestion. Furthermore, the course of this complaint depends very much on the time of diagnosis, so that it does not always lead to a positive course of the disease.

In the extreme stage, the affected person dies from the symptoms of neuroblastoma. Furthermore, it is not uncommon for the child's parents and relatives to be affected by neuroblastoma and suffer from psychological complaints. There are no particular complications in the treatment of this condition. However, a positive outcome cannot always be guaranteed. The life expectancy of the patient may also be significantly reduced due to the neuroblastoma.

When should you go to the doctor?

If children show abnormalities or changes in their health, special care is required. Since neuroblastoma is a tumor disease, a visit to a doctor is recommended as soon as the first signs of an irregularity appear. The sooner a diagnosis can be made, the better the chances of recovery. Otherwise, in severe cases, the child may die prematurely.

A doctor is required in the event of breathing restrictions, shortness of breath or interruptions in breathing. If the child complains of abdominal pain, general malaise or back pain, a visit to a doctor is advisable. Consultation with a doctor is indicated in the event of digestive tract disorders, a decrease in playful activities or other behavioral problems. Diarrhea or refusal to eat are signs of impaired health. A pale appearance, an increased body temperature, and a change in weight are worrying and must be presented to a doctor. If there are abnormalities in the area of ​​the face or neck, these must be examined. A visual impairment, changes in the retina or a pigmentation disorder are warnings of the organism for an existing disease. A visit to the doctor must be initiated as soon as possible to investigate the cause.

If the symptoms persist for several days or if they increase in intensity, a doctor must be consulted immediately. A rescue service must be called in the event of an acute health condition.

Treatment and therapy

Treatment planning should begin as soon as possible after the diagnosis of neuroblastoma. The stage of the tumor, the age of the patient and possible metastasis must be taken into account. A distinction is made between the following stages:

1. The tumor is limited to the site of origin and is completely operable.

2. The tumor is operable, but a residual tumor can still be detected.

3. The tumor is inoperable, but lymph nodes can already be affected.

4. There are metastases in the brain, liver, bone marrow or other organs.

5. Criteria according to 1. and 2., but the patient is under 18 months and there are few or no metastases. Here the tumor can regress spontaneously.

Treatment for neuroblastoma usually consists of a combination of chemotherapy and surgery. Tumors can often only be surgically removed after a reduction by radiation, in other cases the neuroblastoma is first removed and then irradiation of any malignant residual tissue that may still be present is started. Other therapeutic methods can also be used to treat a neuroblastoma.

These include MIBG therapy, stem cell transplants or treatment with retinoic acid. Each individual case requires individual treatment, which is primarily determined by the stage of the tumor. In stage 1, it may be advisable not to use radiation therapy after the surgical removal, but to wait first.

If the tumor has already spread and metastases in other organs, high-risk therapy is usually used, which consists of surgery, radiation and chemotherapy. It can last up to 2 years.

Outlook & forecast

Basically, the course of a neuroblastoma is to be viewed as individual. In neuroblastoma, in addition to the stage of development, the age of the patient is decisive for the course of the disease and prognosis. The prognosis is better in young patients or those in the early stages of the disease. About 75 percent of people diagnosed with neuroblastoma survive the next fifteen years. In low-risk patients, the survival rate is even higher - it is even over 95 percent.

The prognosis for high-risk patients is much worse. Here the rate is only 30 to 40 percent after five years. Even if the tumor has been completely removed, some patients will relapse (relapse) after a certain period of time. The majority of recurrences occur in the first few years following therapy.

For this reason, patients should definitely undergo regular check-ups, especially in a period of ten years after the end of therapy. In addition to physical examinations, this also includes laboratory examinations and examinations using imaging methods. In this way, a possibly recurring neuroblastoma can be identified at an early stage.

So far, neuroblastomas cannot be specifically prevented. Since this disease occurs mainly in children and adolescents, the parents have a special responsibility here. This includes creating a good, stable and harmonious environment for those affected.

prevention

According to current scientific knowledge, one cannot prevent neuroblastoma. Neuroblastomas are mostly discovered by chance, which is why doctors have been trying to find a reliable early detection method for years. In addition to marker tests, neuroblastoma screening was therefore developed in Germany and Canada. However, it cannot yet be said whether this really improves early detection.

Aftercare

After the intensive care treatment of the tumor, rehabilitation and aftercare of the patient begin. As part of tumor follow-up, regular check-ups and patient discussions are carried out. In detail, aftercare includes a thorough physical examination, laboratory tests and imaging tests such as ultrasound and X-rays. If necessary, other imaging methods can be used, for example to examine certain areas of the body or to detect side effects and interactions of drugs.

The close-knit monitoring enables any recurrences to be recognized at an early stage. Possible concomitant diseases or consequences of the therapy are also identified and treated as part of the follow-up care. In addition, aftercare includes therapeutic care for the patient and their relatives.

Affected children, in particular, must be fully cared for in the clinic. As part of the follow-up care, the people involved are given the necessary contact points and measures in order to process the disease optimally. The attending physician accompanies both the examinations and the care of the patient.

Follow-up can last for several years. If there are no complications, the intervals between the examinations are gradually increased. In the event of complications such as relapses or concomitant diseases, therapy must be restarted. Follow-up care is usually canceled in the event of major complications.

You can do that yourself

As children and adolescents are increasingly affected by this disease, parents have a special responsibility. It is extremely important to create a good, harmonious and stable environment for those affected. The children should be seen and included as full family members.

It is important, however, that the prescribed treatments are taken regularly. Supportive homeopathic preparations or other natural products can also be taken in consultation with the doctor to alleviate the course of the disease or to relieve the body. A lifestyle that is almost "normal" will make everyday life easier for patients. This includes meeting friends, going to school and doing lots of things that bring joy. Of course, the attending physician must endorse this, taking into account the stage of the disease.

Otherwise, care should be taken to eat healthy, high-quality food, adequate rest and sleep, and appropriate exercise in the fresh air in order to do good to the body. Many of those affected find it pleasant to go to a self-help group. In this way they can exchange ideas with other people affected and encourage each other. Those who want to take advantage of psychological support will also benefit from it. This helps sick people to accept the disease and to live with it.