Primary pancreatic lymphoma: Clinical presentation, diagnosis, treatment, and outcome. (2nd July 2020)

• Record Type: Journal Article
• Authors: Facchinelli, Davide; Sina, Sokol; Boninsegna, Enrico; Borin, Alex; Tisi, Maria C.; Piazza, Francesco; Scapinello, Greta; Maiolo, Elena; Hohaus, Stefan; Zamò, Alberto; Merli, Michele; Stefani, Piero M.; Mellone, Federica; Basso, Marco; Sartori, Roberto; Rusconi, Chiara; Parisi, Alice; Manfrin, Erminia; Krampera, Mauro; Ruggeri, Marco; Visco, Carlo; Tecchio, Cristina
• Is Part Of: European journal of haematology. Volume 105:Number 4(2020)
• Page Start: 468
• Page End: 475
• Publication Date: 2020-07-02
• Subjects: chemotherapy -- lymphoma -- pancreas -- surgery; Hematology -- Periodicals; Blood -- Diseases -- Periodicals; Blood -- Periodicals; 616.15005
• DOI: 10.1111/ejh.13468
• Languages: English
• ISSNs: 0902-4441

Abstract

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.