Clinical Presentation, Evaluation, and Management of Neuroblastoma

Richa Sharma; Jesse Mer; Alex Lion; Terry A. Vik

doi:10.1542/pir.2017-0087

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Richa Sharma, MD*
Jesse Mer, MD^†
Alex Lion, DO MPH^‡
Terry A. Vik, MD^‡

^*Department of Pediatrics;
^†Department of Radiology;
^‡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:

Identify signs and symptoms of neuroblastoma.
Identify patients who require emergency care for a life-threatening presentation.
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 …