A genetically distinct pediatric subtype of primary CNS large B-cell lymphoma is associated with favorable clinical outcome
Bibliographic record
Abstract
Primary central nervous system (CNS) lymphoma (PCNSL) is an aggressive non-Hodgkin lymphoma with CNS confinement; large B-cell lymphoma (LBCL) accounts for most PCNSL. The median age for PCNS-LBCL is 66 years, with occurrence in younger adults and pediatric cases being uncommon. Although immunodeficient children have an increased risk of developing PCNSL, most cases of pediatric PCNSL in the past few decades have been reported in immunocompetent individuals. Previous studies have reported that the prognosis for PCNS-LBCL is better in pediatric and younger adults than in older adults who have a median survival of ,3 years. 2,3 5] This multi-institutional study was conducted to investigate the molecular characteristics of pediatric and young-adult PCNS-LBCL and define associations with clinical outcomes and pathologic features. The cohort consisted of 12 patients ,40 years of age without known immunodeficiency, including 6 males and 6 females with median age at diagnosis of 17 years (range, 7-38 years). All patients were confirmed to have primary CNS confinement without systemic involvement based on staging positron-emission tomography/computed tomography and bone marrow biopsy. All patients were treated with a combination of dexamethasone, methotrexate, and rituximab, together with various accompanying agents (supplemental Tables Tumors were uniformly composed of dense aggregates of large lymphoid cells with perivascular and parenchymal involvement, were Epstein-Barr virus (EBV) negative, and had diffuse strong immunoreactivity for B-cell markers (CD20 and PAX5). No BCL2 rearrangement and no MYC amplification or rearrangement were present (supplemental Tables No appreciable differences in cytology or growth pattern were identifiable between these pediatric and young-adult patients with PCNS-LBCL compared with their older adult counterparts.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".