Neuroblastoma

Ricarda Schwarz studied medicine in Würzburg, where she also completed her doctorate. After a wide range of tasks in practical medical training (PJ) in Flensburg, Hamburg and New Zealand, she is now working in neuroradiology and radiology at the Tübingen University Hospital.

More about the experts All content is checked by medical journalists.

Neuroblastoma is the second most common childhood cancer. It is formed from nerve cells and is usually located on the spine or in the adrenal gland. Often there are no complaints or they are unspecific. A neuroblastoma can be operated on, irradiated, and treated with chemotherapy drugs. Even after complete removal, it returns in some children. Here you can read everything you need to know about neuroblastoma.

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. D43C71D33

Neuroblastoma: description

Neuroblastoma is the second most common cancer in children in Germany. It is a tumor of the so-called sympathetic nervous system, which controls involuntary tasks in the body. For example, it controls the cardiovascular function, the bladder and bowel functions and the energy metabolism. The sympathetic nervous system ensures that our body functions optimally in stressful situations. Since it occurs in many parts of the body, a neuroblastoma can also form in very different places:

Neuroblastoma is located in the adrenal medulla in about half of the cases. In addition, it often occurs in the nerve plexuses on the right and left of the spine, the borderline - most often at the level of the abdomen, but sometimes also at the level of the neck, chest or pelvis.

In the brain, however, the neuroblastoma only occurs when cells separate from the original tumor, reach the brain and form daughter tumors (metastases) there. But that rarely happens. Also, settlements in the lungs are not very common. Typically, neuroblastoma metastases are found in bone marrow, bones, lymph nodes, liver or skin. About half of all neuroblastomas have already spread at the time of diagnosis.

Depending on the severity, neuroblastoma diseases are divided into four disease stages. It takes into account how large the tumor is, whether it has spread and whether lymph nodes are involved. It is also assessed to what extent the tumor can be surgically removed. Both the therapy and the prognosis of the disease depend on this classification.

Neuroblastoma: incidence

The neuroblastoma is formed from immature (embryonic) cells. Therefore, the disease occurs mainly in early childhood. Every year around one in 100,000 children develops neuroblastoma. About 40 percent of them fall ill in the first year of life. 90 percent of the children affected are younger than six years old. As you age, the likelihood of developing neuroblastoma decreases. Nevertheless, older children, adolescents and adults can also get it. Overall, boys are affected somewhat more often than girls.

Neuroblastoma: symptoms

Neuroblastoma doesn't always cause symptoms. The tumor is therefore often only discovered by chance, for example as part of a routine examination by the pediatrician. If neuroblastoma causes symptoms, the cancer is in many cases well advanced. Symptoms then arise, for example, when the tumor constricts surrounding structures or when organ functions are impaired by metastases. Because neuroblastoma can grow in many places in the body, the signs of the disease also vary.

A tumor in the chest can cause shortness of breath. In the abdomen, a neuroblastoma can impede the flow of urine and, in extreme cases, damage the kidneys (hydronephrosis). If, on the other hand, the neuroblastoma grows in the trunk along the spine, it can displace the nerve roots and cause paralysis. A tumor in the neck area can lead to what is known as Horner's syndrome. One eyelid hangs down, the eyeball sinks back and the pupil narrows. Almost every fifth child with neuroblastoma develops Horner syndrome.

Patients with an already metastatic neuroblastoma often suffer from general symptoms such as pain, impaired general condition, fever or paleness. Metastases in the eyeball sometimes cause a bruise to form on the eye (monocle or eyeglass hematoma). In less than two percent of those affected, a neuroblastoma becomes noticeable through what is known as an opsomyoclonus syndrome. The patients involuntarily twitch their eyes, arms and legs.

Lethargy or increased sweating can also be traced back to a neuroblastoma. If the tumor affects lymph nodes, you can sometimes feel small lumps or swellings in the abdomen or neck. Toddlers often have a bloated stomach, constipation, diarrhea, nausea, or vomiting. Sometimes the tumor also increases blood pressure.

Neuroblastoma: causes and risk factors

A neuroblastoma occurs when cells in the sympathetic nervous system that are still immature undergo malignant changes. It may happen before the birth. However, so far there is no evidence that certain events during pregnancy increase the risk of neuroblastoma. Although this tumor is not a hereditary disease, experience reports exist for neuroblastoma according to which the disease occurs in families in very rare cases.

Neuroblastoma: examinations and diagnosis

If a neuroblastoma is suspected, the doctor first inquires about the symptoms (type, severity and time of occurrence) as well as other health problems (taking the medical history = anamnesis).

Physical exam and ultrasound

He then does a physical exam. An ultrasound examination (sonography) can also be helpful: The doctor can use it to check whether there is a mass (due to tumor growth) in the patient's abdomen or neck area.

Laboratory tests

Laboratory tests are also important: in blood and urine samples, tumor markers are searched for - substances that can be present in high concentrations in cancer. Tumor markers in neuroblastoma are the breakdown products of certain hormones (catecholamines) and the enzyme NSE (neuron-specific enolase). In addition, blood values ​​are examined, which may indicate an increased cell metabolism and thus a malignant tumor.

MRI, CT & Co.

If these initial examinations result in the diagnosis of neuroblastoma, the patient is usually referred to a center for cancer diseases (oncology). This is where further examinations are carried out to determine the exact size and location of the tumor and to detect any metastases. For example, magnetic resonance imaging (magnetic resonance imaging, MRT), computed tomography (CT), bone marrow and tissue examinations and so-called scintigraphy can help.

Scintigraphy

Scintigraphy is a nuclear medicine examination in which a radioactive substance is injected into the bloodstream through a vein and then accumulates in certain organs. These can then be represented graphically, because the radiation emanating from the radioactive substance can be recorded and made visible with a special camera.

The radioactively marked substance MIBG (methyliodobenzylguanidine) is used to mark and detect neuroblastoma cells. Almost all tumors accumulate this substance (MIBG-positive neuroblastomas), only a few do not (MIBG-negative neuroblastomas).

Bone marrow examination

Cancer cells in the bone marrow are easily overlooked in scintigraphy when their numbers are low. Therefore, some patients will have a bone marrow test done. Under local anesthesia, the doctor removed a small amount of bone marrow from the bone with a long hollow needle. Often this examination is carried out in several places. The tissue obtained is examined for cancer cells under a microscope.

Other tests

For later therapy it is also sometimes necessary to check the heart, hearing and kidney function.

Neuroblastoma: treatment

A neuroblastoma can be operated on, irradiated or treated with special cancer drugs (chemotherapeutic agents). These three methods are often combined with one another. In addition, MIBG therapy, stem cell transplantation, and medication containing retinoic acid are used in some patients. Which treatment is recommended in each individual case depends primarily on the stage of the disease and the age of the patient.

Neuroblastoma: surgery

During an operation, the surgeon tries to remove the neuroblastoma as completely as possible without damaging other structures. If the tumor is very large or in an unfavorable position, it is sometimes shrunk with chemotherapy before the operation (neoadjuvant chemotherapy). Even if the tumor cannot be completely removed, the operation is often followed by chemotherapy or radiation (adjuvant therapy).

Neuroblastoma: chemotherapy

If a neuroblastoma cannot be completely removed or if there are already metastases, chemotherapy is usually carried out. To ensure that as many malignant cells as possible are recorded, various chemotherapeutic agents are combined. They are given through a vein at regular intervals.

Neuroblastoma: stem cell transplant

If a neuroblastoma affects the bone marrow, a stem cell transplant may be necessary. To do this, healthy stem cells are first removed from the patient's bone marrow and stored safely. This is followed by high-dose chemotherapy designed to destroy all malignant cells. The remaining healthy cells in the bone marrow also perish. So that blood can be formed again in the bone marrow, the patient receives the stem cells taken before the chemotherapy back via an infusion. They are supposed to settle in the bone marrow again and make blood cells. Patients are very susceptible to pathogens throughout the treatment. Therefore, special protective measures must be observed.

Neuroblastoma: MIBG Therapy

The radioactively labeled substance MIBG used in scintigraphy (see above) can also be used to treat neuroblastoma patients. The radioactive dose chosen is so high that the neuroblastoma cells that attach the MIBG are destroyed. MIBG therapy can be used, for example, if there are still malignant tumor cells in the body after chemotherapy.

Neuroblastoma: disease course and prognosis

The course of the disease in neuroblastoma depends primarily on the age of the patient and the stage of the disease. Young patients and those in the early stages of the disease therefore have a better prognosis. Around 75 percent of neuroblastoma patients survive the next 15 years. In low-risk patients, the survival rate is over 95 percent. Five years after diagnosis, however, only 30 to 40 percent of high-risk patients are still alive.

Even if the tumor has been completely removed, in some patients it returns after a certain time (relapse). Most recurrences occur in the first few years after therapy. That is why regular check-ups should be carried out, especially in the first ten years: A recurring neuroblastoma can be detected early on by means of physical examinations, laboratory tests and imaging procedures.

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