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

Abstract

ABSTRACTPurposeAs a scoping review, evaluate the literature on the presentations, genetics, and therapies for neuroblastoma in adult patients.MethodsWe 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.ResultsOf 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%).MYCNwas 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, specificallyALK(42%) andATRX(58%). Registry data of included studies showed 5- year overall survival to be 36.3% in adults aged ≥20 years.ConclusionOf 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 asALKandATRX. Registry data showed 5-year survival of 36%. Future studies evaluating targeted therapies in larger samples are needed.Take home messages679 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%.