Orbital Rhabdomyosarcoma in a Pediatric Patient With Costello Syndrome
Bibliographic record
Abstract
Costello Syndrome (CS) results from a germline mutation in the HRAS gene. Rhabdomyosarcoma in CS patients has arisen from various sites but not the orbit. The authors present the first case report of orbital rhabdomyosarcoma in a patient with CS. An 18-month-old male known for CS presented with progressively worsening proptosis and periorbital edema. On presentation, he had proptosis with inferolateral globe displacement, chemosis and injection. An urgent computed tomography scan revealed a left orbital mass extending from the posterior aspect of the globe to the orbital apex, and pathological examination confirmed embryonal rhabdomyosarcoma. Treatment with chemotherapy consisted of vincristine, dactinomycin, and cyclophosphamide, and received 8 cycles of photon beam radiation. Radiological surveillance up to 12 months showed no signs of recurrence or metastatic lesions. Patients with CS are at a high risk of developing rhabdomyosarcoma and clinicians should be vigilant in the context of any clinical orbital signs of disease.
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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 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 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".