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Record W1969676656 · doi:10.2350/13-02-1300-cr.1

Nested Stromal Epithelial Tumor of the Liver in Beckwith-Wiedemann Syndrome

2013· article· en· W1969676656 on OpenAlexaff
Janet I. Malowany, Neil Merritt, Nancy Chan, Bo‐Yee Ngan

Bibliographic record

VenuePediatric and Developmental Pathology · 2013
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenetic Syndromes and Imprinting
Canadian institutionsCanada Research ChairsHospital for Sick ChildrenLondon Health Sciences CentreUniversity of Toronto
Fundersnot available
KeywordsHepatoblastomaPathologyMedicineBeckwith–Wiedemann syndromeWilms' tumorCD117Stromal tumorAbdominal massMalignancyLiver tumorStromal cellHepatocellular carcinomaInternal medicineBiology

Abstract

fetched live from OpenAlex

ABSTRACT Beckwith-Wiedemann syndrome (BWS) is an imprinting disorder characterized by overgrowth, congenital malformation, and tumor predisposition. Children with BWS have a higher incidence of tumors, commonly intra-abdominal tumors such as Wilms tumor, hepatoblastoma, and adrenal cortical carcinoma. Here, we describe the first case of a rare hepatic malignancy of nested stromal epithelial tumor (NSET) of the liver in a child with BWS. A 22-month old girl with BWS had a new incidental liver mass. Her alpha-fetoprotein levels were normal. She underwent a liver segmentectomy. Histopathologic features combined with immunohistochemistry results (positivity for pankeratin [AE1/3], CD56, CK19, CD117, CD99 [weak membranous pattern], β-catenin, and WT1-COOH [focal]), were diagnostic of NSET of the liver. This is the first case of NSET of the liver associated with BWS. Its occurrence at such an early age is consistent with the tumor predisposition of BWS.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.001
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Case report · Consensus signal: Case report
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.001
Threshold uncertainty score0.004

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.000
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0000.001
Research integrity0.0010.000
Insufficient payload (model declined to judge)0.0010.000

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.

Opus teacher head0.006
GPT teacher head0.188
Teacher spread0.182 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designCase report
Domainnot available
GenreEmpirical

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".

Quick stats

Citations27
Published2013
Admission routes1
Has abstractyes

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