PNR-28PRIMARY DISSEMINATED LEPTOMENINGEAL NEUROECTODERMAL TUMORS IN CHILDREN: A CASE SERIES AND REPORT OF A LONG TERM SURVIVOR
Bibliographic record
Abstract
Central nervous system primitive neuroectodermal tumours (CNS-PNETs) are a heterogeneous group of poorly differentiated neuroepithelial tumours. Disseminated leptomeningeal CNS- PNET without a clear primary lesion is extremely rare, and nearly uniformly fatal in published cases. We report a series of 5 children with primary leptomeningeal CNS-PNET, including one long-term survivor. METHODS: Clinical data was collected retrospectively, and pathology was reviewed to confirm diagnosis. RESULTS: From 2001 to 2015, five children ages 1 - 12 years (median 4 years) were confirmed to have primary leptomeningeal CNS-PNET. Three children, who were treated with surgery and chemotherapy alone, progressed and died at 1, 3, and 5 years respectively. One child diagnosed at 4 years of age was treated with craniospinal radiation of 3600cGy and multi-agent chemotherapy including etoposide, vincristine, cyclophosphamide, and cisplatin. Nine years later, a persistent spinal lesion was felt to be slowly increasing in size, and was therefore treated with 3 cycles of temozolomide and etoposide. She remains alive and disease-free, 13 years from diagnosis. One child is currently still receiving therapy, but demonstrated a complete response following 5 cycles of HeadStart II regimen. CONCLUSION: We present a single-institutional series of primary leptomeningeal CNS-PNET. Generally, these children have poor outcomes; however, we describe a long-term survivor treated with craniospinal radiation and chemotherapy. The variation in outcomes of these cases suggests there may be differences in underlying biology. Further molecular subtyping may capture these differences and potentially influence treatment.
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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 | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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".