Neuroblastomas are tumors of small children (usually under 5 years of age) that arise from the so-called autonomic nerve nodes, which are located as "border cords" to the right and left of and also centrally in front of the spinal column. Their task is to control the unconscious body and organ functions (hence the term "autonomic nervous system"). Another typical organ in which these tumors occur is the adrenal glands, which are also part of the autonomic nervous system. The cause of tumor formation from these nerve cells is unknown, and familial clustering is very rare. Environmental factors or harmful substances do not appear to play a role.

Neuroblastomas are noticeable as a palpable tumor, for example in the flank, neck or pelvis, or through general symptoms, ultrasound examinations or X-ray examinations; some produce hormone-like substances that trigger symptoms.

There are many different subtypes of neuroblastoma, which vary in frequency depending on age and require individually tailored treatment. Histological examination often reveals abnormalities such as a greatly increased chromosome count in the nucleus of neuroblastoma cells. The duplication of a gene on chromosome 2, the so-called N-MYC gene, an enlargement of chromosome 17 and a defect on chromosome 1, for example, are known for neuroblastomas that grow particularly aggressively.

The principle of all treatments is based on a combination of chemotherapy and surgical partial or complete removal of the tumor. Radiotherapy and immunotherapy are also used. The primary care of children with neuroblastoma is provided by colleagues in [LINK] pediatric oncology, who develop an individual treatment concept for each patient based on the protocols of the pediatric cancer studies (www.kinderkrebstudien.de).

In Germany, all malignant diseases that occur in children are treated according to binding protocols specified by the LINK Society for Pediatric Oncology and Hematology (GPOH) LINK (www.kinderkrebstudien.de). For us, this means that all oncology patients are always treated jointly with the [LINK] Department of Pediatric Oncology and Hematology at the MHH.

If a neuroblastoma is suspected, imaging examinations are carried out first (in babies and small children often under general anesthesia with the involvement of our LINK pediatric anesthesiology department). In addition, the blood, urine and bone marrow are examined for general (e.g. anemia) and specific signs of the tumor disease (certain hormones, e.g. "catecholamines"). If the tumor is small and can be completely removed immediately, a tumor biopsy, i.e. the removal of a small, representative piece of tissue, is usually performed by surgery. If the tumor is located in the body cavities, this is usually done here at the MHH Children's Surgery Department using minimally invasive surgery, as there is virtually no risk of tumor cell spread in neuroblastoma. The advantages of minimally invasive biopsy, apart from the reduced pain for the patient, are that the body has to exert less healing force due to the lack of an abdominal or thoracic incision and the patient's immune system can focus more on the tumor than on healing the abdominal incision. Recovery after the biopsy is also faster, so that your child can start the usually necessary preliminary drug therapy (so-called neoadjuvant chemotherapy) earlier.

Once all the information on the spread of the tumor and the histological examination of the tumor tissue (in which risk markers such as N-MYC can be detected) is available, the tumor is formally classified into a tumor stage or risk class (INSS, International Neuroblastoma Staging System). Treatment is then carried out in accordance with the aforementioned treatment protocols of the LINK Society for Pediatric Oncology and Hematology (GPOH).

The MHH has particular expertise in so-called high-dose chemotherapy with subsequent autologous stem cell transplantation (so-called stem cell rescue), whereby the child's own stem cells, which have previously been removed and purified of tumor cells, are reintroduced after chemotherapy. Modern facilities are available for this, which guarantee special protection for the children.
For drug therapy, a so-called port catheter (a permanent access to the venous blood circulation under the skin) or another central venous catheter (e.g. "Broviak catheter") is often implanted, whereby special systems are available for babies.

For surgical therapy, anatomically uncomplicated localizations on the back wall of the chest and in the area of the adrenal glands can be removed using the minimally invasive technique of abdominal or thoracic endoscopy. However, extensive neuroblastomas can grow together with vital structures such as the intestinal or lung root, the large blood vessels and the spinal cord and then require an open surgical procedure.

After the operation, your child will be cared for in our pediatric intensive care unit before being transferred back to the normal ward. Further treatment is then based on the individually tailored treatment plan and is carried out together with our LINK pediatric oncology partners.

 

Results:

The goal of every treatment for children with neuroblastoma is complete remission. This is an important step on the way to a complete cure and is achieved when your child no longer shows any signs of the disease using the available methods (bone marrow puncture, examination, imaging). However, it is not possible to know in the early stages whether any "invisible" tumor cells remain, which means that follow-up care is necessary for many years.

The data collected in therapy control studies on many children provide indicative figures. These indicate that, depending on the stage, an average of 75-85% of all children can be permanently cured.

 

 

Even after the operation, further chemotherapy often follows before the implanted port system can be removed again. Follow-up care is also carried out in accordance with the study protocols and usually by colleagues from LINK Pediatric Oncology.