NEUROBLASTOMA
Overview
Neuroblastoma is the most common intra-abdominal solid tumor in childhood accounting for 8-10% of all pediatric cancers 85% of patients present before 6 years of age Neuroblastoma is well known to undergo spontaneous regression and maturation Amplified N-myc (more than 10 copies)- occurs in approximately 30% of neuroblastoma and is associated with advanced disease at diagnosis and poor outcome Outcomes in low and most intermediate Risk
neuroblastomas are excellent; however the treatment of High Risk Neuroblastoma remains extremely challenging
Signs and Symptoms
Abdominal mass, fever, weight loss, weakness, bone pain, high blood pressure
Screening and Diagnosis
The International Criteria for diagnosis of neuroblastoma, states that diagnosis must be based on one of the following:
- An unequivocal pathologic diagnosis made from tumor tissue by light microscopy (with or without immunohistochemistry, or increased levels of serum catecholamines or urinary catecholamine metabolites [VMA or HVA] ).
- The combination of bone marrow aspirate or trephine biopsy containing unequivocal tumor cells (e.g., syncytia or immunocytologicallypositive clumps of cells) and increased levels of serum catecholamines or urinary catecholamine metabolites
Treatment
Low risk
Surgery +/- Low dose chemotherapy, Asymptomatic: Observation Symptomatic: Low dose chemotherapy / Radiation
Intermediate risk
Surgery + chemotherapy +/- Radiation
High risk
Induction: Multiagent chemotherapy Local Treatment : surgery + Radiation Consolidation with Myeloablative chemotherapy and autologous stem cell transplant Differentiation therapy with 13 cis retinoic acid +/- Immunotherapy