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Primary pancreatic lymphoma: Clinical presentation, diagnosis, treatment, and outcome

2020; Wiley; Volume: 105; Issue: 4 Linguagem: Inglês

10.1111/ejh.13468

1600-0609

Davide Facchinelli, Sokol Sina, Enrico Boninsegna, Alex Borin, Maria Chiara Tisi, Francesco Piazza, Greta Scapinello, Elena Maiolo, Stefan Hohaus, Alberto Zamò, Michele Merli, Piero Maria Stefani, Federica Mellone, Marco Basso, Roberto Sartori, Chiara Rusconi, Alice Parisi, Erminia Manfrin, Mauro Krampera, Marco Ruggeri, Carlo Visco, Cristina Tecchio,

Gastrointestinal Tumor Research and Treatment

Primary pancreatic lymphoma (PPL) is a rare disease representing 0.1% of malignant lymphomas, which lacks well-defined diagnostic and therapeutic protocols.To describe PPL clinical, diagnostic and histological characteristics, together with therapy and outcome, in a relatively large series of patients.The study includes 39 PPL patients, aged ≥15 years, observed from January 2005 to December 2018, in 8 Italian Institutions.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.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.