Small Desmoplastic Round Cell Tumor of the Kidney following Hodgkin Lymphoma: Case Report and Literature Review
Bibliographic record
Abstract
Introduction: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive sarcoma that is most commonly present in males with abdominal and peritoneal invasion. There are potential curative therapeutic options, but survival remains poor. To this day, there have only been 22 reported cases of primary renal DSRCT in the literature and thoracic DSRCT has been rarely reported with very little known about effective therapy. This is the first reported case of renal DSRCT following a primary malignancy, and it is further distinguished by the application of whole-genome sequencing and targeted biological therapy. Case Presentation: The literature on renal DSRCT was reviewed, and we describe a patient with thoracic relapse of DSRCT following a primary renal lesion, initially treated with radical nephrectomy and 14 cycles of chemotherapy (doxorubicin 225 mg/m2, cyclophosphamide 11.4 g/m2, ifosfamide 63 g/m2, etoposide 3.5 g/m2, and vincristine 12 mg/m2). The patient also had a distant history of nodular lymphocyte predominant Hodgkin lymphoma, in remission for 5 years following three cycles of doxorubicin 150 mg/m2, vincristine 8.4 mg/m2, prednisone 840 mg/m2, and cyclophosphamide 2.4 g/m2. Treatment with pazopanib for DSRCT was attempted but discontinued due to significant toxicity. Conclusion: DSRCT remains a highly aggressive malignancy with limited treatment options and poor prognosis. Our case highlights the rarity of primary renal DSRCT and its potential for thoracic relapse, further emphasizing the challenges in disease management.
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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 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.006 | 0.005 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.002 | 0.001 |
| Research integrity | 0.003 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
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".