• Bret Adams
  • Daphne Haas-KoganEmail author
  • Joseph Panoff
Part of the Practical Guides in Radiation Oncology book series (PGRO)


  • Neuroblastoma is the most common solid non-CNS malignancy of childhood representing 8–10% of all pediatric cancer, and approximately 650 cases are diagnosed per year in the United States.

  • Neuroblastoma is notable for its heterogeneity in behavior, with some cases showing spontaneous regression and others demonstrating dismal outcomes.

  • Patients may present with an abdominal mass with or without obstructive symptoms. Children with neuroblastoma tend to have symptoms at presentation in contrast to children with Wilms tumor. It is not uncommon to see systemic symptoms such as fever, weight loss, sweating, flushing, abdominal pain, failure to thrive, and generalized weakness.

  • Over 50% of children will have metastases at diagnosis involving the bone, liver, lymph nodes, and skin.

  • Bone metastases have the potential to cause pain or refusal to walk, and skin metastases that occur in infants may present with clinical manifestations of blue skin lesions that blanch with pressure due to release of vasoactive catecholamines (referred to as the “blueberry muffin” sign).

  • Paraneoplastic syndromes may cause symptoms that include opsoclonus-myoclonus, truncal ataxia syndrome that is attributed to antibody formation to neurons, and diarrhea from vasoactive intestinal polypeptide secretion.

  • Workup for neuroblastoma includes CT and/or MRI to evaluate the primary lesion. If feasible, it may be of benefit to obtain both studies at initial diagnosis to best evaluate the initial extent of disease and to determine the best imaging modality for subsequent studies.

  • Bilateral bone marrow biopsies and MIBG scan (123I-MIBG) should be obtained to evaluate for metastases. PET/CT and bone scan (with 99mTc-diphosphonate) may be useful for non-MIBG-avid disease. CT of the chest/abdomen and pelvis should be considered to evaluate for metastatic disease, and MRI or CT of the brain may be useful if large metastases are present. Baseline labs including CBC, CMP, and urinary catecholamines (e.g., vanillylmandelic acid and homovanillic acid, elevated in 90% of patients) should be obtained.

  • Pathology is necessary for diagnosis unless urinary catecholamines are elevated since it can provide additional prognostic information. If surgery is performed initially, postsurgical imaging should be obtained to determine the extent of resection.

  • Children under 12 months of age are considered to have more favorable outcomes. Recent studies have indicated that this age cutoff can be extended to 18 months for children with favorable histology.

  • Past Children’s Oncology Group’s (COG) studies stratified neuroblastoma into low-, intermediate-, and high-risk groups based on an amalgamation of these prognostic factors.

  • Radiation therapy is recommended for children with intermediate-risk disease who progress through chemotherapy, for those who have persistent disease following all treatment, and for children with high-risk neuroblastoma.


  1. 1.
    Cohn SL et al (2009) The International Neuroblastoma Risk Group (INRG) classification system: an INRG task force report. J Clin Oncol 27:289–297CrossRefGoogle Scholar
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    Paulino AC, Zach Fowler B (2005) Risk factors for scoliosis in children with neuroblastoma. Int J Radiat Oncol Biol Phys 61(3):865–869CrossRefGoogle Scholar
  3. 3.
    Wolden SL et al (2008) Brain-sparing radiotherapy for neuroblastoma skull metastases. Pediatr Blood Cancer 50(6):1163–1168CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Bret Adams
    • 1
  • Daphne Haas-Kogan
    • 2
    • 3
    • 4
    Email author
  • Joseph Panoff
    • 5
  1. 1.Blue Ridge Cancer CareRoanokeUSA
  2. 2.Department of Radiation OncologyBrigham and Women’s Hospital, Dana-Farber Cancer Institute, Boston Children’s Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Children’s Hospital BostonBostonUSA
  4. 4.Harvard Medical SchoolBostonUSA
  5. 5.21st Century OncologyFort MyersUSA

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