PEDIATRICS CLINICAL RESEARCH
Bibliographic record
Abstract
INTRODUCTION: Primitive neuroectodermal tumor (PNET) is the most common primary malignant childhood brain tumor and is usually seen as an intraparenchymal lesion, typically arising in the cerebellum (as a medulloblastoma).Peripheral PNET (PPNET), a member of the Ewing's sarcoma (ES) family of tumors, is a tumor of putative parasympathetic neural histogenesis, typically developing in the soft tissue and skeletal structures of the trunk and extremities.We report here four cases of atypical primary ES/PPNET in very young children involving pericranial structures, two with significant intracranial involvement.METHODS: Following IRB approval, we compiled the clinical, imaging, and pathology records from four children with aggressive pericranial ES/PPNET.RESULTS: Ages at diagnosis were 1 day (female), 5 days (male), 12 months (male), and 13 months (female).Two patients had orbitofacial tumors with significant bony erosion but no intracranial extension, one patient had a temporal bone lesion with significant middle cranial fossa extension, and one patient had an intracranial temporal lobe-based tumor without extracranial extension.Immunohistochemical evidence of the EWS/FLI-1 chimeric protooncogene, typical of PPNET, was identified in one patient; genetic analysis in two patients revealed EWSR1 gene rearrangement in one patient and a novel t(20;22)(q11.2;q12)translocation in the other.All tumors were relatively large (for body size), measuring 3-8 cm in maximal dimension.Treatment included tumor resection and sarcoma-style combination chemotherapy.Two patients are alive and continue therapy, whereas two have died of sepsis and progressive disease at 3 and 6 months after diagnosis, respectively.CONCLUSIONS: Pericranial primary ES/PPNET is a rare tumor, heretofore typically described only in pre-school and school-aged children.Our study suggests this entity can occur in very young children, in whom it has a poor prognosis.It appears to be more infiltrative and to violate the usual tissue compartments.Further awareness and reporting of this rare tumor will aid understanding of histogenesis, prognosis, and optimal therapy.
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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 | 0.001 | 0.004 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.001 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.002 | 0.002 |
| Insufficient payload (model declined to judge) | 0.490 | 0.317 |
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".