1. Richa Sharma, MD* 2. Jesse Mer, MD† 3. Alex Lion, DO MPH‡ 4. Terry A. Vik, MD‡ 1. *Department of Pediatrics; 2. †Department of Radiology; 3. ‡Department of Pediatrics, Division of Pediatric Hematology-Oncology, Indiana University School of Medicine, Indianapolis, IN * Abbreviations: CBC: : complete blood cell CT: : computed tomography HVA: : homovanillic acid IHC: : immunohistochemical IL-2: : interleukin-2 mIBG I-123: : metaiodobenzylguanidine MRI: : magnetic resonance imaging OMS: : opsoclonus myoclonus syndrome VMA: : vanillylmandelic acid WBC: : white blood cell Pediatricians play a pivotal role in the diagnosis of neuroblastoma and as such should be aware of the elusive signs and symptoms to provide clinical surveillance, appropriate referral, and medical support as part of the patient’s multidisciplinary team. After completing this article, readers should be able to: 1. Identify signs and symptoms of neuroblastoma. 2. Identify patients who require emergency care for a life-threatening presentation. 3. Discuss the basics of clinical presentation, diagnostics, and management of neuroblastoma. Pediatric cancers occur in 171 per 1 million children in the United States each year and are the leading cause of disease-associated death in children. (1) Neuroblastoma is not only the most common cancer in infancy but also the most prevalent solid tumor outside the cranium, (2) and it sometimes requires the most aggressive treatment plan in pediatric oncology. Therefore, pediatricians should be familiar with clinical presentations that should prompt appropriate and timely referral. In this review, we present neuroblastoma, which exemplifies several principles of pediatric oncology, including its multidisciplinary treatment approach. The annual incidence of neuroblastoma is approximately 700 cases in North America. (1) In a review of national cancer registries from 2001 through 2009, a diagnosis of neuroblastoma or ganglioneuroblastoma was found in approximately 6% of the cases. (1) Neuroblastoma is more common in the white population (9.7 per 1 million) than in the African American population (6.8 per 1 million) (2) and more common in males (8.5 per 1 million) than in females (7.6 per 1 million). (3) Although the median age at diagnosis of neuroblastoma is 18 months, (4) there is a wide age range, from in utero diagnosis to …
