This OMEDLIVE presentation is Part Two of a two-part series surrounding Pediatric Neuroblastoma. During the conversation, Dr. Jason Shohet and Jennifer Saggio, MSN, CRNP, CPON focus on the following areas:

  • Overview of pathology and standard therapy for high-risk NB
  • Required imaging and evaluations for the revised INRGSS system
  • Overall treatment scheme, including recent proposed changes
  • Discussion of therapeutic options for relapsed disease occurring at several stages of therapy
  • Updates on late effects of treatment
  • Psychosocial aspects of therapy and relapse
  • Multidisciplinary approaches to clinical challenges

Accounting for around 9% of pediatric cancer and almost 13% of all pediatric cancer deaths, neuroblastomas are the most common malignant solid tumor of childhood (excluding brain tumors). Despite treatment efforts, more than half of children with metastatic disease will relapse. Authors point out relapsed disease has a very poor survival outcome (<10%), with outcomes dependent upon stage, timing, and treatment options.

Until very recently, the International Neuroblastoma Staging System (INSS) is used to stage pediatric neuroblastomas, however a newly adopted classification/staging system has come into use which is less complicated and uses radiographic imaging rather than surgical resection to determine risk factors, pathology, and treatment. The International Neuroblastoma Risk Group Staging System (INRGSS) uses only the results of imaging tests taken before surgery, and not surgical results, to describe the pediatric neuroblastomas into four stages (L1, L2, M, and MS) rather than the six stages identified with INSS.

Treatment for high-risk pediatric neuroblastoma patients is broken into three categories or stages, each of which is discussed in further detail in Part One of the series:

  1. Induction: six cycles of dose intensive chemotherapy, plus surgical resection of primary
  2. Consolidation: High dose chemo with autologous stem cell transplant (ASCT), plus radiation to primary site
  3. Maintenance: treatment with dinutuximab, plus IL-2/GM-CSF and retinoic acid

As explained by Dr. Shohet, the plan of care for treating relapsed neuroblastoma is dependent upon the timing of relapse, the extent of relapse, condition and age of patient, and family and patient choices. He goes on to say it is important to set realistic expectations and understanding with regards to the risks and benefits of treatment (recall relapsed disease has a very poor survival outcome).

A large portion of the presentation is dedicated to examining four case studies. Categories for these patients are Early Relapse, Post-Transplant Relapse, Older Adolescent, and a 5 year old female diagnosed with Stage 4, high-risk neuroblastoma with MYCN amplification and unfavorable histology. Jennifer goes through each case in detail, discussing treatment options, risks, and additional considerations as they pertain to relapsed patients.

For a more information and in-depth explanation, please watch “Cases, Challenges, and Controversies on High-Risk Pediatric Neuroblastomas” presented by OMEDLIVE.

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