Neuroblastoma (ICD-10-GM C74.-: Malignant neoplasm of the adrenal gland) is a malignant neoplasm (malignant neoplasm) of the autonomic nervous system.
Neuroblastoma is the second most common malignant neoplasm in children behind acute lymphoblastic leukemia (ALL).
Sex ratio: girls and boys are affected with approximately equal frequency.
Frequency peak: the disease occurs in childhood. In 90% of cases, the tumor occurs in the first six years of life, with 40% of children developing the disease in the first year of life.
The incidence (frequency of new cases) is approximately 1 case per 7,000 children per year (in Germany).
Course and prognosis: The neoplasia (neoplasm) can occur anywhere where sympathetic nerve tissue is located. It occurs mainly in the adrenal medulla or at the cervical, thoracic and abdominal border cord (along the spine). Approximately 70% of tumors are located outside the abdomen in the retroperitoneal space (anatomical structures that lie behind the peritoneum and are not enclosed by the peritoneum) and approximately 20% are located between the lung lobes in the mediastinum (mediastinal space, is a vertical tissue space in the chest cavity). Neuroblastoma usually metastasizes (forms daughter tumors) rapidly. At diagnosis, metastases are already present in 50% of those with the disease (typically in bone marrow, bone, regional and distant lymph nodes, liver, or skin). In infants, spontaneous regression to cure is possible.
Neuroblastoma can be recurrent (recurring), so follow-up for up to 10 years is recommended.
The 5-year survival rate for all patients is 79% and the 15-year survival rate is 75%.Depending on individual risks such as stage of neuroblastoma, age, molecular genetic alterations, a distinction can be made between low-risk patients and high-risk patients. The survival rates of these two groups of patients are as follows: The survival rate of low-risk patients is >95% and the survival rate of high-risk patients is between 30% and 50% (at 5 years).