Neuroblastoma (NB) is the most common solid extracranial malignancy of childhood with an incidence of 1 per 100,000 in the United States compromising approximately 10% of childhood cancer. Neuroblastoma arises from neural crest cells which are located throughout the sympathetic nervous system and therefore malignancy can be found anywhere sympathetic cells are located, with about 50% arising from the adrenal medulla. Beyond the wide variability in location, neuroblastoma is a heterogenous disease with a wide spectrum of presentations from rapidly progressive disease to tumors with spontaneous regression. Due to the broad range of disease progression, the clinical management of neuroblastoma poses unique challenges. The advances over the past thirty years have led to an optimization of treatment with improvement in 5-year overall survival from 46% in the 1980’s to 71% in the early 2000s1. Despite these leaps in survival for all comers, patients with high-risk neuroblastoma continue to have dismal outcomes with long term survival less than 50%. Given these unique characteristics and the high burden of disease, neuroblastoma research has focused on finding factors to help improve survival in the most at-risk groups. In recent years both the Children's Oncology Group (COG) and International Society of Paediatric Oncology (SIOP) have worked to define the role and extent of surgery in local recurrence and survival. The work presented in this edition of Seminars of Pediatric Surgery will delineate and expand on key components of surgical resection with a focus on high-risk disease.
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