Neuroblastoma Frequently Asked Questions

When does neuroblastoma usually appear and what is its incidence in children?

Neuroblastoma is the most common solid tumor in childhood; 9 to 10 cases per million children are diagnosed each year.

Its incidence is approximately double that of leukemia, and it is more frequent under the age of one year, becoming rarer after the third year of life.

Neuroblastoma: symptoms and diagnosis

The symptoms depend on the age at which it is diagnosed, the type of tumor, its location and whether or not it has metastases at diagnosis. It can range from a chance finding in an imaging test performed for another reason to the presence of a large abdominal mass that grows very rapidly in a few days. As the most frequent localization is abdominal, digestive symptoms (loss of appetite, vomiting, increase in abdominal perimeter, presence of palpable mass, diarrhea…) are usually the first to present.

The involvement of children is also variable. In those in whom the diagnosis is casual, the involvement is nil. But in aggressive tumors, of large size and with metastases, the general condition can be greatly affected. It also depends on the age at diagnosis, since newborns are not the same as older patients.

Risk factors for neuroblastoma

Although much research has been done on the risk factors that can lead to the development of neuroblastoma, very few have been discovered to date. The presence of other neuroblastomas in the family is very rare. The association with certain syndromes such as multiple endocrine neoplasia, neurofibromatosis, central hypoventilation syndrome (Ondine) or Hirschsprung’s disease is more frequent, although rare.

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Is it possible to prevent neuroblastoma?

It can probably be diagnosed early in those syndromes with a risk of developing it. But its prevention is complex and early detection programs by measuring certain metabolites in the urine of newborns have been discontinued because of their low yield.

Neuroblastoma: diagnosis and treatment

Diagnosis is always clinical, imaging and biopsy. It requires both sophisticated imaging tests and sampling of the tumor or its metastases, as both are necessary for the classification of the tumor into different risk groups and its subsequent accommodation to certain lines of treatment.

Treatment is variable depending on this risk. It is standardized by supranational pediatric oncological associations, following several different lines that can range from doing nothing (conservative or expectant treatment) to include chemotherapy, radiotherapy, surgical treatment, immunotherapy and bone marrow transplantation.

There are few centers throughout Spain familiar with all of these therapies, so patients at higher risk should probably be treated in more experienced Pediatric Oncology Services.