Neuroblastoma childhood cancer: cause, symptoms, treatment

A neuroblastoma is a malignant growth that arises from nerve tissue, often in the adrenal gland. In children under the age of one year, neuroblastomas are the most common malignant tumors. What are the causes and symptoms? How is neuroblastoma treated, what is immunotherapy and what is the prognosis for neuroblastoma? Villa Joep raises money for research specifically aimed at neuroblastoma childhood cancer. However, the foundation does much more, such as maintaining an overview of blogs and links to foundations that have been created by parents who are currently in the treatment process and write about their experiences.

Article content

  • Neuroblastoma
  • Where can neuroblastomas appear?
  • Causes of neuroblastoma
  • Symptoms of neuroblastoma
  • Complications of neuroblastoma
  • Diagnosis of neuroblastoma
  • Neuroblastoma treatment and immunotherapy
  • What does immunotherapy entail?
  • Neuroblastoma prognosis
  • Villa Joep
  • Neuroblastoma blog
  • Research

Neuroblastoma

Neuroblastomas are uncommon, but they are the most common malignant tumors (cancer) in children under the age of one year . They occur slightly more frequently in boys. The tumors often originate from nerve tissue that has been ‘stuck’ in the embryonic stage. In the Netherlands, approximately 25 to 30 children are affected by neuroblastoma each year. The majority of the children are younger than six years old.

Where can neuroblastomas appear?

Neuroblastomas can occur in the abdomen, the pelvic cavity and the abdominal cavity and sometimes the tumor is located in the chest cavity or neck. From there they can spread. A third of all cases are found in the adrenal glands.

Causes of neuroblastoma

The cause is unknown, but it is suspected that there is a hereditary component. There may be a gene that is abnormal. A person’s lifestyle is not an important factor.

Symptoms of neuroblastoma

The symptoms you see with neuroblastoma depend on where the tumor is located. The following symptoms may be present at birth, but they may also develop slowly over childhood:

  • With a neuroblastoma in the abdomen: swelling in the abdomen, sometimes abdominal pain and a feeling of nausea;
  • With a neuroblastoma in the nerve canal of the spine: symptoms of paralysis may occur, the child may have difficulty walking or have problems urinating or defecating;
  • With a neuroblastoma in the neck: swelling, sometimes the eyelid droops slightly;
  • The tumor produces adrenaline-like substances, which can lead to hypertension (high blood pressure), diarrhea, excessive sweating;
  • A tired feeling;
  • Blueish bumps on the skin, they do not hurt;
  • Sometimes ‘dancing eyes’: rapid, restless movements of both eyes.

Complications of neuroblastoma

If a neuroblastoma spreads in the body, the following complaints may arise:

  • A painful feeling in the bones;
  • In affected lymph nodes, swelling may occur in the armpit or neck;
  • Anemia can occur if it spreads to the bone marrow.

Diagnosis of neuroblastoma

If a neuroblastoma is suspected, a urine test will be performed. It is then checked whether there are any substances that indicate a tumor. The diagnosis can be confirmed by microscopic examination of tissue (by means of a biopsy). If there is a neuroblastoma, it will be checked whether or not there are metastases. This can be done by means of MRI or a radionuclide scan. Some bone marrow may also be removed to examine whether the cancer has spread to the bones.

Neuroblastoma treatment and immunotherapy

If possible, the tumor is removed by surgery. Chemotherapy and radiation may be necessary. Because the tumors often return in an aggressive form after initial treatment, immunotherapy has been developed. There are only a few hospitals in the world where this is possible, including the Children’s Hospital of Philadelphia (CHOP). Treatment takes approximately six months. The child’s medical costs are usually reimbursed by the health insurer, but travel costs and family accommodation costs are not. So it is a costly and far-reaching thing. Many families organize actions to raise the money. This treatment has also taken place in the Princess Máxima Center in Utrecht since 2016.

What does immunotherapy entail?

During treatment, various medications are administered to ensure that the tumor no longer grows and spreads. Furthermore, cancer cells are found and destroyed. The body’s defenses are increased in bone marrow and blood. White blood cells are stimulated to destroy the cancer cells. The drugs also attempt to change the neuroblastoma cells that remain into normal cells, so that they divide at a slower pace. The treatment increases the chance of survival by more than twenty percent.

Neuroblastoma prognosis

  • If there are no metastases, approximately nine out of ten children are doing well five years after the operation.
  • However, if there is metastasis, the prospects are not good. The cure rate for the most aggressive type of neuroblastoma is shockingly low (thirty percent).
  • In babies under one year of age, neuroblastomas sometimes disappear on their own.
  • Immunotherapy increases the chance of survival by more than twenty percent. Approximately five to ten children from the Netherlands undergo the treatment every year.

Villa Joep

Joep is the namesake for Villa Joep. In November 2002, a neuroblastoma was discovered in his abdomen. A series of chemotherapy treatments was immediately started. Joep seemed to be doing better, but in September 2003 metastases were discovered again. Joep died in October 2003, aged four and a half. His family has promised Joep to continue the fight against neuroblastoma and they have set up Villa Joep for this purpose. The foundation raises money for research specifically aimed at neuroblastoma childhood cancer. But the foundation does much more.

Neuroblastoma blog

Especially for affected families who are currently in the treatment process, Villa Joep maintains an overview of blogs and links to foundations that have been created by parents who are also currently in the treatment process and write about their experiences.

Research

  • August 23 , 2013 , oncogeneticist Prof. Dr. Rogier Versteeg, leader of the AMC neuroblastoma research (sponsored by Villa Joep) received an ERC Advanced Grant. This is a prestigious European scientific fair. This grant of no less than 2.5 million euros has been awarded by the European Research Council (ERC) on the basis of the researcher’s CV and current and future research line.
  • In November 2015 it was announced that a new gene defect had been found in neuroblastoma. With this discovery, seventy percent of aggressive neuroblastomas have been identified as having a genetic abnormality. The research is published (online) in Nature Genetics (journal). It has been discovered that many growths show an abnormality in chromosome 5, near the so-called TERT gene. There is often a break in the chromosome near the TERT gene and another piece of chromosome is attached to it. This makes the TERT gene overactive in the cancer cells and causes cell growth to be stimulated too much.