Orbital Synovial Sarcoma: Novel Case Report and Review of the Literature
Bibliographic record
Abstract
PURPOSE: To describe the clinical, histologic, and molecular features of a rare case of orbital synovial sarcoma and to provide a comprehensive literature review. METHODS: The authors present a case report and up-to-date literature review of orbital synovial sarcoma. Variables analyzed included patient demographics, clinical presentations, imaging findings, histopathologic features, molecular diagnostics, treatment, and outcomes. RESULTS: A 25-year-old female presented with a left orbital mass causing swelling, tearing, and mild vision impairment. Imaging identified a mass in the inferior orbit, which was subsequently confirmed by pathology as a monophasic spindle cell synovial sarcoma. Notably, initial fluorescence in situ hybridization testing for the SS18 rearrangement was negative; however, next-generation sequencing later identified an SS18-synovial sarcoma X breakpoint protein 2 fusion. The patient underwent complete surgical resection, followed by proton beam radiotherapy and chemotherapy. A PubMed and Medline search revealed 10 prior cases of orbital synovial sarcoma. Most patients were female (90.9%) and presented with painless swelling, lacrimation, and headaches. All tumors were localized at diagnosis. Surgical resection was the primary treatment in 81% of cases, with adjuvant radiation or chemotherapy each administered in 36% of cases. No recurrence or metastasis was observed on follow-up (average 11.6 months). CONCLUSIONS: Orbital synovial sarcoma is rare and typically localized at diagnosis. This is the first reported orbital synovial sarcoma with an SS18-synovial sarcoma X breakpoint protein 2 fusion identified by next-generation sequencing after negative fluorescence in situ hybridization. The case underscores the diagnostic value of molecular profiling when standard testing is inconclusive.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.006 | 0.005 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.003 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 0.002 |
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".