Key Highlights
- Neuroblastoma accounts for approximately 6-10% of all childhood cancers
- The annual incidence of neuroblastoma is about 10.2 cases per million children under 15 years old globally
- Neuroblastoma predominantly affects children under the age of 5, with about 80% of cases diagnosed by age 5
- The median age at diagnosis for neuroblastoma is approximately 17 months
- Neuroblastoma accounts for around 7% of all pediatric cancers
- In children, neuroblastoma has a survival rate of about 75% for low-risk cases, but less than 50% for high-risk cases
- MYCN gene amplification occurs in approximately 20-25% of neuroblastoma cases and is associated with poor prognosis
- Approximately 40-50% of neuroblastoma cases show metastasis at diagnosis, often to bones, bone marrow, liver, and skin
- The overall five-year survival rate for neuroblastoma varies significantly depending on risk stratification, with about 80-90% in low-risk and less than 50% in high-risk groups
- Neuroblastoma accounts for approximately 15% of pediatric cancer fatalities
- The median age at diagnosis for high-risk neuroblastoma is roughly 2 years old
- Neuroblastoma can spontaneously regress in some infants, especially around 12 months of age, in about 1-2% of cases
- Approximately 60% of neuroblastoma tumors originate in the adrenal glands
Neuroblastoma, a rare yet formidable pediatric cancer accounting for 6-10% of childhood cancers, primarily strikes children under five, with groundbreaking advances in targeted therapies and immunotherapy offering new hope amid its complex diagnosis and variable prognosis.
Demographic and Risk Factors
- Neuroblastoma is more prevalent in children of Caucasian ethnicity compared to African-American or Asian populations
Demographic and Risk Factors Interpretation
Epidemiology and Incidence
- Neuroblastoma accounts for approximately 6-10% of all childhood cancers
- The annual incidence of neuroblastoma is about 10.2 cases per million children under 15 years old globally
- Neuroblastoma predominantly affects children under the age of 5, with about 80% of cases diagnosed by age 5
- The median age at diagnosis for neuroblastoma is approximately 17 months
- Neuroblastoma accounts for around 7% of all pediatric cancers
- Approximately 40-50% of neuroblastoma cases show metastasis at diagnosis, often to bones, bone marrow, liver, and skin
- Neuroblastoma accounts for approximately 15% of pediatric cancer fatalities
- The median age at diagnosis for high-risk neuroblastoma is roughly 2 years old
- Neuroblastoma can spontaneously regress in some infants, especially around 12 months of age, in about 1-2% of cases
- Approximately 60% of neuroblastoma tumors originate in the adrenal glands
- Neuroblastoma symptoms vary widely but commonly include abdominal pain, a palpable mass, and signs of metastasis like bone pain or skin lesions
- Neuroblastoma has a higher incidence in boys than girls, with a ratio of approximately 1.2:1
- Only about 30% of neuroblastoma cases present with localized disease at diagnosis, the rest are metastatic or advanced
- The incidence of neuroblastoma is higher in infants and decreases with age, with the few cases in adolescents and adults often associated with poorer prognosis
- Neuroblastoma is diagnosed through a combination of imaging, biopsy, and检测 catecholamine metabolites in urine, with no single definitive test
- Approximately 10-15% of neuroblastoma patients have congenital neuroblastoma detected prenatally via ultrasound
Epidemiology and Incidence Interpretation
Genetics and Molecular Markers
- MYCN gene amplification occurs in approximately 20-25% of neuroblastoma cases and is associated with poor prognosis
- Amplification of the ALK gene is present in approximately 10-15% of neuroblastoma cases, associated with poorer outcomes
- Neuroblastoma tumors often produce catecholamines, with elevated levels of homovanillic acid (HVA) and vanillylmandelic acid (VMA) used as diagnostic markers
- Genetic factors such as chromosome 1p deletion and 11q deletion are associated with poorer prognosis in neuroblastoma
- The gene PHOX2B is involved in neuroblastoma development, especially in cases associated with neurocristopathies like Hirschsprung disease
Genetics and Molecular Markers Interpretation
Prognosis and Survival Rates
- In children, neuroblastoma has a survival rate of about 75% for low-risk cases, but less than 50% for high-risk cases
- The overall five-year survival rate for neuroblastoma varies significantly depending on risk stratification, with about 80-90% in low-risk and less than 50% in high-risk groups
- The International Neuroblastoma Risk Group (INRG) classification divides neuroblastoma into low, intermediate, and high-risk groups, which heavily influence prognosis
- Standard risk neuroblastoma has a five-year survival rate exceeding 90%, while high-risk can be as low as 40-50%
- Neuroblastoma often metastasizes early, which is a key factor in its prognosis and treatment approach
- Approximately 15-20% of neuroblastoma cases have spontaneous differentiation into benign ganglioneuroma, especially in infants, leading to better outcomes
- Relapse occurs in approximately 40-50% of children with high-risk neuroblastoma, often leading to poor prognosis
- Children's neuroblastoma survival rates have improved significantly over the past 30 years due to advances in treatment, from about 20% to over 70% for some risk groups
- In recent years, immunotherapy using anti-GD2 monoclonal antibodies has increased survival rates in high-risk groups by approximately 20-30%
- The expression of TrkA neurotrophin receptor in neuroblastoma is associated with favorable prognosis, especially in infants
Prognosis and Survival Rates Interpretation
Treatment Options and Therapies
- The main treatment options for neuroblastoma include surgery, chemotherapy, radiotherapy, and immunotherapy
- Newer targeted therapies, including ALK inhibitors and anti-GD2 antibodies, are improving outcomes for high-risk neuroblastoma patients
- The drug dinutuximab, an anti-GD2 antibody, is approved for treatment of high-risk neuroblastoma in children, significantly improving survival in clinical trials
- The median duration of treatment for high-risk neuroblastoma may extend over 12-18 months, involving multiple modalities and phases
- High-risk neuroblastoma patients often undergo stem cell transplant following high-dose chemotherapy to improve outcomes
Treatment Options and Therapies Interpretation
Sources & References
- Reference 1CANCERResearch Publication(2024)Visit source
- Reference 2NCBIResearch Publication(2024)Visit source
- Reference 3CANCERResearch Publication(2024)Visit source
- Reference 4CANCERJOURNALResearch Publication(2024)Visit source
- Reference 5MEDLINEPLUSResearch Publication(2024)Visit source
- Reference 6PUBMEDResearch Publication(2024)Visit source
- Reference 7SOCResearch Publication(2024)Visit source