The Clinicopathologic Spectrum of Rosai–Dorfman–Destombes Disease in Adults: An Analysis of 16 Cases
Bibliographic record
Abstract
Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic proliferation with protean clinical manifestations, resulting from the accumulation of activated histiocytes within nodal and extra-nodal tissues. The diagnosis can be missed, particularly when biopsies are obtained from extra-nodal sites, where histological features may be less specific than those observed in nodal specimens. Here, we examined a case series of 16 adults diagnosed with RDD from Vancouver, Canada between 2015 and 2025. Cases were classified as diagnostically challenging if the time to a definitive pathologic diagnosis exceeded 60 days from the initial biopsy for RDD-related clinical symptoms and > 1 pathologic sampling was required to establish a diagnosis. Eight of 16 patients (50%) were classified as having diagnostically challenging cases. The median number of independent pathological samples required to establish a diagnosis of RDD was 2.0 (interquartile range: 2.3), with five patients needing three or more independent specimens. The median timeto final diagnosis was 12 days for non-diagnostically challenging cases (IQR: 15 days) and 240 days for diagnostically challenging cases (IQR: 138 days). Notably, diagnostically challenging cases tended to have specimen sampling from atypical anatomical sites (62.5% vs. 37.5%), utilized suboptimal pathological sampling techniques (87.5% vs. 25.0%; e.g., cytology), and delayed or absent consultation with hematolymphoid-specialized pathologists (25.0% vs. 87.5%). Together, this study underscores the extreme clinical heterogeneity of extra-nodal RDD, emphasizes the critical role of expert hematolymphoid pathology expertise in its diagnosis, and suggests practical strategies-such as liberal use of immunohistochemistry on atypical histiocyte-rich infiltrates-to avoid premature exclusion of RDD from the differential diagnosis.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".