Neuroblastoma in Children - Medical Experts

In medicine, the term “nerve cancer” refers to all tumor diseases that affect the central nervous system or the autonomic nervous system.

There are various types of nerve tumors, such as glioblastoma, astrocytoma, ependymoma, neurocytoma, and neuroblastoma.

Neuroblastoma is a malignant tumor of the sympathetic nervous system. It is the third most common type of cancer in children and occurs in 7 to 8 percent of children. Brain tumors, another type of nervous system cancer, are the second most common type of cancer in children (right after leukemia).

The sympathetic nervous system is part of the autonomic nervous system. @ bilderzwerg / AdobeStock

The exact causes are not yet known. Heredity is generally ruled out for most cancers of the nervous system. Nevertheless, there are families in which neuroblastomas occur more frequently. Genetic changes, known as mutations, may play a role in their development.

Brain tumors are more likely to develop after radiation therapy to the skull during childhood, for example, as part of leukemia treatment. Some cancers frequently occur in connection with congenital disorders such as neurofibromatosis type 1 or tuberous sclerosis.

Since nerve cancer can occur in the adrenal medulla, the chest, head, or neck, the symptoms vary widely:

• Urinary retention: A tumor in the abdomen can put pressure on the abdominal organs and thus cause urinary retention.
• Bruising around the eyes: In the case of tumors in the head region.
• Shortness of breath: In cases of tumors in the lung area.
• Headaches, swollen lymph nodes, and high blood pressure
• Severe headaches or back pain
• Dizziness, nausea, and vomiting: In cases of tumors of the central nervous system, particularly the brain. Nausea and vomiting occur regardless of food intake; the urge to vomit is most common in the morning and when lying down.
• Difficulty concentrating
• Personality changes
• Pressure in the head: Due to increasing pressure inside the skull.
• Hydrocephalus and enlarged head circumference: If the drainage of cerebrospinal fluid (CSF) is impaired, hydrocephalus may develop. In babies and toddlers with an open fontanelle, the head circumference increases due to the pressure. In medicine, this is referred to as macrocephaly.

In hydrocephalus, excess cerebrospinal fluid (CSF) accumulates in the brain. @ Pepermpron / AdobeStock

Other nonspecific signs of cancer of the nervous system include:

• Fatigue
• Weakness and rapid exhaustion
• Persistent fever with no other apparent cause
• (Nighttime) sweating
• Loss of appetite
• Weight loss

If a tumor of the nervous system is suspected, the doctor will perform various tests. These include imaging techniques such as computed tomography (CT) or magnetic resonance imaging (MRI).

A tissue sample (biopsy) can further confirm the diagnosis. In the case of neuroblastoma, a bone marrow aspiration is also essential. This is the only way for the doctor to determine whether the tumor has already spread to the bone marrow.

The treatment of nervous system cancers in children depends on the type of tumor, its location, and its size. The presence of metastases also plays a role. If possible, the treating doctor surgically removes the malignant solid tumor. 

• Surgery: In the earlier stages of the disease, the focus of treatment is on surgically removing the tumor.

• Chemotherapy: If the tumor is too large, has metastasized, or is difficult to access, chemotherapy is used.

• Combination therapy: Depending on the tumor, doctors may prefer combination therapy. This consists of surgery, chemotherapy, and radiation therapy. In many cases, surgery is performed first, followed by concurrent chemotherapy and radiation therapy.

• Palliative care: If removal of the tumor is not possible because too much healthy brain tissue would be lost in the process, patients receive palliative care to alleviate pain and symptoms.

The course of the disease and the prognosis depend on the type, location, and size of the tumor, as well as the child’s overall health:

• Neuroblastomas

Localized and confined neuroblastomas have a 5-year survival rate of over 90 percent. However, if metastases are present, only 20 percent of children are still alive 5 years after diagnosis. Unlike other tumors, however, neuroblastoma has a high spontaneous remission rate of up to 80 percent.

• Brain Tumors

The figures also vary for brain tumors. Children with a so-called low-grade glioma have very good prognoses. Low-grade gliomas grow relatively slowly and cause fewer symptoms. In the case of high-grade gliomas, however, there are patients for whom there is no cure. They die from their disease. High-grade gliomas are aggressive and spread rapidly throughout the body.

• Long-Term Effects

After surviving cancer, symptoms may persist: for example, damage from surgery is possible. Some children are paralyzed or have other movement disorders. Speech disorders may also remain. Follow-up care is essential in many cases. Regular check-ups are also conducted to detect recurrences early.

Neuropediatrics, also known as pediatric neurology, is a medical specialty that deals with brain tumors in children. A doctor who specializes in these conditions is called a pediatric neurologist or neuropediatrician.

After completing medical school and residency training in pediatrics, a prospective neuropediatrician undergoes three years of specialized training.

Another medical specialty that deals with nervous system cancers in children is pediatric oncology. After completing medical school, a prospective pediatric oncologist must complete residency training to become a specialist in hematology and oncology. This training lasts 72 months. Subsequently, further advanced training in the field of pediatric oncology is required.

To treat neurological cancers in children quickly and effectively, doctors from various specialties work together. Close collaboration with pediatricians is therefore essential.

Neurological conditions are often complex and require comprehensive care. To prevent or counteract long-term complications, collaboration with occupational therapists, speech-language pathologists, or physical therapists can be beneficial.