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

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.

journal_name

Eur J Haematol

authors

Facchinelli D,Sina S,Boninsegna E,Borin A,Tisi MC,Piazza F,Scapinello G,Maiolo E,Hohaus S,Zamò A,Merli M,Stefani PM,Mellone F,Basso M,Sartori R,Rusconi C,Parisi A,Manfrin E,Krampera M,Ruggeri M,Visco C,Tecchio C

doi

10.1111/ejh.13468

subject

Has Abstract

pub_date

2020-10-01 00:00:00

pages

468-475

issue

4

eissn

0902-4441

issn

1600-0609

journal_volume

105

pub_type

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