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American Academy of Pediatrics
Article

Clinical Presentation, Evaluation, and Management of Neuroblastoma

Richa Sharma, Jesse Mer, Alex Lion and Terry A. Vik
Pediatrics in Review April 2018, 39 (4) 194-203; DOI: https://doi.org/10.1542/pir.2017-0087
Richa Sharma
*Department of Pediatrics;
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Jesse Mer
†Department of Radiology;
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Alex Lion
‡Department of Pediatrics, Division of Pediatric Hematology-Oncology, Indiana University School of Medicine, Indianapolis, IN
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Terry A. Vik
‡Department of Pediatrics, Division of Pediatric Hematology-Oncology, Indiana University School of Medicine, Indianapolis, IN
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  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
  • AUTHOR DISCLOSURE

    Drs Sharma, Mer, Lion, and Vik have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

  • 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
  • Education Gap

    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.

    Objectives

    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.

    Introduction

    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.

    Epidemiology

    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 …

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    Pediatrics in Review: 39 (4)
    Pediatrics in Review
    Vol. 39, Issue 4
    1 Apr 2018
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    Clinical Presentation, Evaluation, and Management of Neuroblastoma
    Richa Sharma, Jesse Mer, Alex Lion, Terry A. Vik
    Pediatrics in Review Apr 2018, 39 (4) 194-203; DOI: 10.1542/pir.2017-0087

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    Clinical Presentation, Evaluation, and Management of Neuroblastoma
    Richa Sharma, Jesse Mer, Alex Lion, Terry A. Vik
    Pediatrics in Review Apr 2018, 39 (4) 194-203; DOI: 10.1542/pir.2017-0087
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