Neonatal Neuroblastoma

摘要

Neuroblastoma is the most common solid, extracranial malignant neoplasm of childhood and the most common malignant tumor in infants less than 1 year of age, with an overall incidence of 1 per 100,000 children in the United States.1 The Children?s Oncology Group (COG) Statistics and Data Center estimates that neonatal neuroblastoma, defined as neuroblastoma diagnosed prenatally or within 28 days after birth, represents 5% of all neuroblastoma cases.2 Neuroblastoma displays a very heterogeneous clinical phenotype, with some tumors spontaneously regressing or maturing (with or without adjuvant therapy), whereas others exhibit an aggressive clinical phenotype and respond poorly to adjuvant therapy. Overall, the 5-year survival rate for children with neuroblastoma is approximately 75%, but those younger than 18 months at presentation, including neonates, have a 5-year survival rate greater than 85%.3,4 Therefore, the prevailing trend for treating neonates with localized neuroblastoma is to minimize or avoid therapeutic interventions altogether, including surgery and chemotherapy, except for a few specific indications. Most of the morbidity and mortality associated with neonatal neuroblastoma occurs in a subset of patients with disease that has disseminated in a specific pattern, primarily to the liver and not to the bone. This often bulky disease can result in respiratory compromise and abdominal compartment syndrome.