PRIMARY PANCREATIC LYMPHOMA: CLINICAL PRESENTATION, DIAGNOSIS, TREATMENT AND OUTCOME IN A MULTICENTRIC ITALIAN EXPERIENCE
Bibliographic record
Abstract
Introduction: Primary Pancreatic Lymphoma (PPL) is a very rare disease, representing only 0.1% of lymphomas and 0.2% of pancreatic tumors. PPL presentation is variable and may overlap the onset of other neoplastic or inflammatory pancreatic diseases, often resulting in diagnostic difficulties. Due to the limited number of PPL study series, standardized diagnostic and therapeutic protocols are currently lacking. Methods: Diagnostic criteria for PPL were established according to the “Fourth WHO Classification of digestive tumors”. Clinical and treatment data of patients diagnosed with PPL between 2005 and 2018 were retrieved from medical record databases of 7 Italian Hematology Units. Overall survival (OS) and progression free survival (PFS) were calculated according the Kaplan Meier test and compared by the log-rank test. Results: Overall 37 patients were affected by PPL. Clinical manifestations at diagnosis are summarized in the table. Most patients (83%) had a high grade B cell histotype. Fifty nine % of patients were first line treated with chemotherapy, 21% with chemo + radiotherapy, 10% with surgery + chemotherapy, 3% with surgery and 7% did not received any treatment. With a median follow up of 37.5 months the 2 year OS is 59% while the 2 year PFS is 45%. Provided the relatively low number of cases, patients with low grade lymphomas and a good risk Revised International Prognostic Index (R-IPI) had better median OS (not reached vs 26 months and not reached vs 8 months, p=0.31 and 0.001) and PFS (not reached vs 108 months and 108 vs 8 months, p=0.095 and 0.01) as compared to the others. There were no differences in OS and PFS beetween chemotherapy or chemo-radiotherapy treatments (p=0.8 and 0.33) while patients who underwent debulking surgery (alone or + chemotherapy) had a significant worse OS as compared to those treated with chemotherapy alone (median not reached vs 6 months, p=0.033). Importantly 3/28 (10.7%) patients with high grade PPL experienced a central nervous system (CNS) relapse of disease. Conclusions: PPL represents a rare and difficult-to-recognize disease with symptoms and pancreatic gland localization superimposable to those of pancreatic ductal adenocarcinoma. Most PPL are high grade non Hodgkin lymphoma. According to our study biopsy should be the preferred diagnostic method since debulking surgery was associated with a worse OS. The high CNS relapse rate suggests that patients with high grade PPL should receive CNS directed prophylaxis. Keywords: extranodal lymphomas.
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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 | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.000 |
| 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".