Neuroblastoma is the third most common malignant cancer in children. In many cases, the abnormal cell growth begins even before birth. The exact causes are still unknown.
Here you will find more information and specialists in the treatment of neuroblastoma.
Every year, more than 100 children are diagnosed with neuroblastoma. It most commonly affects newborns and infants. Only 10 percent of all patients are older than six years. In rare cases, however, neuroblastoma can also occur in adults.
In neuroblastoma, the tumor develops from degenerated cells of the nervous system. For this reason, neuroblastomas can develop anywhere where tissue of the sympathetic nervous system is present. The most common site is the adrenal medulla. About 70 percent of all neuroblastomas are found in the abdomen. Only 20 percent of tumors manifest in the neck or chest area.
Neuroblastoma is a cancer of the nervous system that primarily affects young children up to the age of six @ Dr_Microbe /AdobeStock
Neuroblastoma develops from embryonic—and thus immature—cells of the nervous system. In many cases, the degeneration of these cells begins even before birth. The exact causes remain unknown.
Although the disease is considered unlikely to be hereditary, there are families in which the tumor occurs more frequently. It is presumed that genetic or chromosomal changes underlie the development of the tumor.
It is not yet clear whether external factors (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 few or no symptoms for a long time.
Symptoms do not appear until the tumor has grown quite large. They vary depending on the tumor’s location. Large neuroblastomas may be palpable.
In affected children, they manifest as swelling in the neck or abdomen. Tumors in the abdomen or near the adrenal glands can compress other organs. For example, narrowing of the ureters can lead to urinary retention or even complete urinary obstruction. In such cases, there is a risk of acute renal failure.
Tumors in the neck region can cause what is known as Horner’s triad. Damage to a part of the autonomic nervous system results in a characteristic three-part set of symptoms:
In advanced stages of the disease in the neck or head region, bruising may form around the eyes. This is also referred to as a “spectacle” or “monocle” hematoma.
Extensive skin hemorrhages may also occur in the area of the eyelids (blepharochymosis). In rare cases, affected children suffer from opsomyoclonus-ataxia syndrome. This is characterized by brief and irregular eye movements in various directions, as well as brief twitching of the legs and arms.
In advanced neuroblastoma, patients feel tired, listless, and weak. A moderate fever with no apparent cause, combined with increased sweating, is also a symptom of neuroblastoma.
Depending on the tumor’s location, the following symptoms may indicate this cancer:
In young children, neuroblastoma grows very rapidly and metastasizes to the bone marrow, bones, liver, and lymph nodes. In cases of neuroblastoma that occur in early infancy, doctors may also observe spontaneous regression of the tumor in some instances.
When planning treatment, doctors primarily consider the stage of the disease in children.
For neuroblastoma, staging is based on:
In tumor stages 1 and 2, doctors can often remove the tumor through surgery alone. In advanced stages of the disease, chemotherapy with various cytostatic drugs is usually administered before surgery.
If any malignant tissue remains after surgery and/or chemotherapy, radiation therapy may be administered. Treatment methods such as MIBG therapy or high-dose chemotherapy are used as adjuncts or alternatives.
In MIBG therapy, patients receive radioactively labeled methyliodobenzylguanidine (MIBG). This substance is designed to destroy the tumor tissue from within. As part of high-dose chemotherapy, the doctor administers high doses of cytostatic drugs to the patient, which inhibit cell growth.
The goal 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 performed following high-dose chemotherapy.
In this procedure, the patient receives their own stem cells, which doctors collect from the bone marrow or blood prior to chemotherapy.
Depending on the severity of the neuroblastoma, treatment with retinoic acid—a synthetic form of vitamin A—may be used. 13-cis-retinoic acid is believed to reduce cell division in neuroblastomas.
Retinoic acid can also be used as part of maintenance therapy to prevent relapses. In recent years, antibodies have been increasingly used after completion of therapy for neuroblastoma with metastases. These are intended to recognize any remaining neuroblastoma cells and stimulate the body’s own immune system to eliminate them.
