Neuroblastoma in Adults: A Scoping Review of Presentations, Genetics and Therapies

Abstract

Purpose: As a scoping review, evaluate the literature on the presentations, genetics, and therapies for neuroblastoma in adult patients. Methods: We searched four databases for studies reporting adults with neuroblastoma. Cohort studies, case series, and case reports were synthesized qualitatively. Progression-free and overall survival were compared amongst cohort studies. Results: Of 2287 unique records, 136 studies published in 141 articles were included. A total of 679 patients were included. On review of individual patient-level data, the adrenal gland and retroperitoneum were the most common primary site (47.3%). MYCN was rarely amplified: seven studies reported zero patients with MYCN amplified, two studies with a single patient, and one study with 3/7 patients. Adult patients appear to show a high frequency of somatic mutations, specifically ALK (42%) and ATRX (58%). Registry data of included studies showed 5-year overall survival to be 36.3% in adults aged ≥20 years. Conclusion: Of nearly 700 cases of adult neuroblastoma published in the literature, the most common primary site is the adrenals or retroperitoneum. Relative to pediatric cases, adult cases demonstrate a considerable rate of somatic mutations such as ALK and ATRX. Registry data showed 5-year survival of 36%. Future studies evaluating targeted therapies in larger samples are needed.

Competing Interest Statement

The authors have declared no competing interest.

Funding Statement

This study did not receive any funding

Author Declarations

I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained.

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The details of the IRB/oversight body that provided approval or exemption for the research described are given below:

Only publicly available human data from published studies were used for this review. All published studies were cited in the manuscript of the study.

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Yes

I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance).

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I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable.

Yes

Data Availability

All data produced in the present study are available upon reasonable request to the authors

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