Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2020 Oct;105(4):468-475.
doi: 10.1111/ejh.13468. Epub 2020 Jul 2.

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

Davide Facchinelli  1 , Sokol Sina  2 , Enrico Boninsegna  3 , Alex Borin  4 , Maria C Tisi  5 , Francesco Piazza  6 , Greta Scapinello  6 , Elena Maiolo  7 , Stefan Hohaus  7 , Alberto Zamò  8 , Michele Merli  9 , Piero M Stefani  10 , Federica Mellone  11 , Marco Basso  12 , Roberto Sartori  12 , Chiara Rusconi  13 , Alice Parisi  2 , Erminia Manfrin  2 , Mauro Krampera  1 , Marco Ruggeri  5 , Carlo Visco  1 , Cristina Tecchio  1
Affiliations
Multicenter Study

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

Davide Facchinelli et al. Eur J Haematol. 2020 Oct.

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.

Keywords: chemotherapy; lymphoma; pancreas; surgery.

PubMed Disclaimer

References

REFERENCES

    1. Behrns KE, Sarr MG, Strickler JG Pancreatic lymphoma: is it a surgical disease? Pancreas. 1994;9:662-667.
    1. Baylor SM, Berg JW Cross-classification and survival characteristics of 5,000 cases of cancer of the pancreas. J Surg Oncol. 1973;5:335-358.
    1. Freeman C, Berg JW, Cutler SJ Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29:252-260.
    1. Dawson IM, Cornes JS, Morson BC Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg. 1961;49:80-89.
    1. Fléjou JF Classification OMS 2010 des tumeurs digestives: la quatrième édition [WHO Classification of digestive tumors: the fourth edition]. Ann Pathol. 2011;31(5 Suppl):S27-31.

Publication types

LinkOut - more resources