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.

Take home messages 679 cases of adult neuroblastoma have been published in the literature.

Compared to pediatrics, adult cases have more somatic mutations (eg: ALK, ATRX).

Registry data showed that adult neuroblastoma has a 5-year survival of 36%.

The authors have declared no competing interest.

This study did not receive any funding

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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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