Davide Facchinelli1, Sokol Sina2, Enrico Boninsegna3, Alex Borin4, Maria C Tisi5, Francesco Piazza6, Greta Scapinello6, Elena Maiolo7, Stefan Hohaus7, Alberto Zamò8, Michele Merli9, Piero M Stefani10, Federica Mellone11, Marco Basso12, Roberto Sartori12, Chiara Rusconi13, Alice Parisi2, Erminia Manfrin2, Mauro Krampera1, Marco Ruggeri5, Carlo Visco1, Cristina Tecchio1. 1. Hematology Unit, Department of Medicine, University of Verona, Verona, Italy. 2. Department of Diagnostics and Public Health, University of Verona, Verona, Italy. 3. Department of Radiology, ASST, Mantova, Italy. 4. Unit of General and Pancreatic Surgery, Department of Surgery and Oncology, Pancreas Institute, University of Verona, Verona, Italy. 5. Hematology Unit, Ospedale San Bortolo, Vicenza, Italy. 6. Hematology Unit, Department of Medicine, University of Padova, Padova, Italy. 7. Fondazione Policlinico Universitario A. Gemelli IRCCS, Istituto di Ematologia, Università Cattolica del Sacro Cuore, Rome, Italy. 8. Department of Oncology and Pathology Unit, AOU Città della Salute e della Scienza, Turin, Italy. 9. Division of Hematology, Ospedale di Circolo e Fondazione Macchi- ASST Sette Laghi, University of Insubria, Varese, Italy. 10. Hematology Unit, Ospedale Cà Foncello, Treviso, Italy. 11. Institute of Medical Statistics, University of Verona, Verona, Italy. 12. Hematology Unit, Ospedale di Castelfranco Veneto, Vicenza, Italy. 13. Division of Hematology, ASST Niguarda Hospital, Milano, Italy.
Abstract
Primary pancreatic lymphoma (PPL) is a rare disease representing 0.1% of malignant lymphomas, which lacks well-defined diagnostic and therapeutic protocols. OBJECTIVES: To describe PPL clinical, diagnostic and histological characteristics, together with therapy and outcome, in a relatively large series of patients. METHODS: The study includes 39 PPL patients, aged ≥15 years, observed from January 2005 to December 2018, in 8 Italian Institutions. RESULTS: The main symptoms were abdominal pain (58%) and jaundice (47%). Lactate dehydrogenase serum levels were elevated in 43% of patients. Histological specimens were mostly obtained by percutaneous (41%) or endoscopic (36%) biopsy, with diffuse large B-cell lymphoma being the most frequent (69%) histological diagnosis. Chemotherapy was administered alone in 65% of patients, with radiotherapy in 17%, or after surgery in 9%. The 2-year overall survival (OS) was 62%, the 2-year progression-free survival (PFS) 44%. Debulking surgery (with or without chemotherapy) was associated with a significant worse OS. Three (9.4%) of 32 high-grade patients experienced a central nervous system (CNS) relapse. CONCLUSIONS: PPL is rare, often high-grade, with symptoms and localization similar to other pancreatic malignancies. Biopsy should be the preferred diagnostic method. High-grade PPL should undergo CNS prophylaxis.
Primary pancreatic lymphoma (PPL) is a rare disease representing 0.1% of malignant lymphomas, which lacks well-defined diagnostic and therapeutic protocols. OBJECTIVES: To describe PPL clinical, diagnostic and histological characteristics, together with therapy and outcome, in a relatively large series of patients. METHODS: The study includes 39 PPL patients, aged ≥15 years, observed from January 2005 to December 2018, in 8 Italian Institutions. RESULTS: The main symptoms were abdominal pain (58%) and jaundice (47%). Lactate dehydrogenase serum levels were elevated in 43% of patients. Histological specimens were mostly obtained by percutaneous (41%) or endoscopic (36%) biopsy, with diffuse large B-cell lymphoma being the most frequent (69%) histological diagnosis. Chemotherapy was administered alone in 65% of patients, with radiotherapy in 17%, or after surgery in 9%. The 2-year overall survival (OS) was 62%, the 2-year progression-free survival (PFS) 44%. Debulking surgery (with or without chemotherapy) was associated with a significant worse OS. Three (9.4%) of 32 high-grade patients experienced a central nervous system (CNS) relapse. CONCLUSIONS: PPL is rare, often high-grade, with symptoms and localization similar to other pancreatic malignancies. Biopsy should be the preferred diagnostic method. High-grade PPL should undergo CNS prophylaxis.