More than 100 children are diagnosed with neuroblastoma every year. Newborns and infants are most frequently affected. Only ten percent of all patients are older than six years. In individual cases, however, neuroblastoma can also occur in adults.
In neuroblastoma, the tumor develops from degenerated cells of the nervous system. Therefore, neuroblastomas can develop anywhere where the tissue of the sympathetic nervous system is found. The most common localization is the adrenal medulla. Around 70 percent of all neuroblastomas are found in the abdominal cavity. Only 20 percent of tumors manifest themselves in the neck or chest area.
Neuroblastoma is a cancer of the nervous system that mainly affects small children up to the age of six @ Dr_Microbe /AdobeStock
Neuroblastoma develops from embryonic and therefore immature cells of the nervous system. In many cases, the degeneration of the cells begins even before birth. The exact causes are still unknown.
Although the disease is unlikely to be inherited, there are families in which the tumor occurs more frequently. The tumor is probably caused by a change in genes or chromosomes.
It is not yet clear whether external influences (alcohol, nicotine during pregnancy, environmental toxins) play a role in the development of the disease.
Doctors usually discover neuroblastoma during a routine examination because it causes little or no symptoms for a long time.
Symptoms only appear when the tumor is already quite large. They vary depending on the location of the tumor. Large neuroblastomas can be palpable.
In affected children, they appear as swellings on the neck or abdomen. Tumors in the abdomen or in the area of the adrenal glands can compress other organs. A narrowing of the ureters can lead to urinary retention or even complete urinary retention. In such a case, there is a risk of acute renal insufficiency.
The so-called Horner's triad can occur with tumors in the neck area. Nerve damage to part of the involuntary nervous system results in a characteristic three-part symptom complex:
- pupil constriction (miosis)
- drooping upper eyelid (ptosis)
- An eyeball that sinks into the eye socket (enophthalmos)
With advanced disease in the neck or head area, bruising can form around the eyes. This is also referred to as a spectacle or monocular hematoma.
There may also be extensive bleeding of the skin around the eyelids (eyelid ecchymosis). In rare cases, affected children suffer from opsomyoclonus ataxia syndrome. This is characterized by short and irregular eye movements in different directions as well as short twitches of the legs and arms.
Patients with advanced neuroblastoma feel tired, listless and weak. Moderate fever without an identifiable cause in combination with increased sweating are also symptoms of neuroblastoma.
Depending on the location of the tumor, the following symptoms may indicate this cancer:
- Swelling of the lymph nodes
- Diarrhea, abdominal pain and constipation
- loss of appetite
- Nausea and vomiting
- Bone pain
In young children, neuroblastoma grows very quickly and metastasizes to the bone marrow, bones, liver and lymph nodes. In the case of neuroblastomas that occur in early infancy, doctors can also observe a spontaneous regression of the tumor in some cases.
When planning treatment, doctors primarily consider the stage of the child's disease.
In the case of neuroblastoma, the classification is based on
- The size of the tumor
- The involvement of lymph nodes
- The presence and extent of metastasis
In tumor stages 1 and 2, doctors can often remove the tumor by surgery alone. In advanced stages of the disease, chemotherapy with various cytostatic drugs is usually given before surgery.
If degenerated tissue is still found after surgery and/or chemotherapy, radiotherapy can be carried out. In addition or as an alternative, treatment methods such as MIBG therapy or high-dose chemotherapy are used.
In MIBG therapy, patients are given radioactively labeled methyl iodobenzylguanidine (MIBG). This substance is intended to destroy the tumor tissue from the inside. As part of high-dose chemotherapy, the doctor administers high doses of cytostatic drugs to the patient to inhibit cell growth.
The aim is to eliminate all malignant cells in the body. However, the therapy damages the bone marrow so severely that an autologous stem cell transplant must be carried out following the high-dose chemotherapy.
This involves the patient receiving the body's own stem cells, which doctors remove from the bone marrow or blood before chemotherapy.
Depending on the severity of the neuroblastoma, the patient may be treated with retinoic acid, a synthetic vitamin A acid. The 13-cis-retinoic acid is said to reduce cell division in neuroblastomas.
Retinoic acid can also be used to prevent relapses as part of maintenance therapy. For some years now, antibodies have increasingly been used after therapy for neuroblastoma with metastasis has been completed. These are intended to recognize remaining neuroblastoma cells and stimulate the body's own immune system to eliminate them.