Neuroblastoma in Adults: A Scoping Review of Presentations, Genetics and Therapies
Bibliographic record
Abstract
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. 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%.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.005 | 0.023 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.003 | 0.002 |
| Bibliometrics | 0.016 | 0.016 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.003 | 0.003 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".