{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T03:28:49Z","timestamp":1790393329453,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,7,2]],"date-time":"2020-07-02T00:00:00Z","timestamp":1593648000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:label\/><jats:p>Primary pancreatic lymphoma (PPL) is a rare disease representing 0.1% of malignant lymphomas, which lacks well\u2010defined diagnostic and therapeutic protocols.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To describe PPL clinical, diagnostic and histological characteristics, together with therapy and outcome, in a relatively large series of patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The study includes 39 PPL patients, aged \u226515\u00a0years, observed from January 2005 to December 2018, in 8 Italian Institutions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>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\u2010cell 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\u2010year overall survival (OS) was 62%, the 2\u2010year progression\u2010free survival (PFS) 44%. Debulking surgery (with or without chemotherapy) was associated with a significant worse OS. Three (9.4%) of 32 high\u2010grade patients experienced a central nervous system (CNS) relapse.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>PPL is rare, often high\u2010grade, with symptoms and localization similar to other pancreatic malignancies. Biopsy should be the preferred diagnostic method. High\u2010grade PPL should undergo CNS prophylaxis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.13468","type":"journal-article","created":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T09:49:17Z","timestamp":1592300957000},"page":"468-475","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Primary pancreatic lymphoma: Clinical presentation, diagnosis, treatment, and outcome"],"prefix":"10.1111","volume":"105","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7581-5824","authenticated-orcid":false,"given":"Davide","family":"Facchinelli","sequence":"first","affiliation":[{"name":"Hematology Unit Department of Medicine University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sokol","family":"Sina","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Public Health University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrico","family":"Boninsegna","sequence":"additional","affiliation":[{"name":"Department of Radiology ASST Mantova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex","family":"Borin","sequence":"additional","affiliation":[{"name":"Unit of General and Pancreatic Surgery Department of Surgery and Oncology Pancreas Institute University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria C.","family":"Tisi","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale San Bortolo Vicenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Piazza","sequence":"additional","affiliation":[{"name":"Hematology Unit Department of Medicine University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Greta","family":"Scapinello","sequence":"additional","affiliation":[{"name":"Hematology Unit Department of Medicine University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Maiolo","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario A. Gemelli IRCCS Istituto di Ematologia Universit\u00e0 Cattolica del Sacro Cuore Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Hohaus","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario A. Gemelli IRCCS Istituto di Ematologia Universit\u00e0 Cattolica del Sacro Cuore Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Zam\u00f2","sequence":"additional","affiliation":[{"name":"Department of Oncology and Pathology Unit AOU Citt\u00e0 della Salute e della Scienza Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0905-5927","authenticated-orcid":false,"given":"Michele","family":"Merli","sequence":"additional","affiliation":[{"name":"Division of Hematology Ospedale di Circolo e Fondazione Macchi\u2010 ASST Sette Laghi University of Insubria Varese Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piero M.","family":"Stefani","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale C\u00e0 Foncello Treviso Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Mellone","sequence":"additional","affiliation":[{"name":"Institute of Medical Statistics University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Basso","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale di Castelfranco Veneto Vicenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Sartori","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale di Castelfranco Veneto Vicenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Rusconi","sequence":"additional","affiliation":[{"name":"Division of Hematology ASST Niguarda Hospital Milano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice","family":"Parisi","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Public Health University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erminia","family":"Manfrin","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Public Health University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mauro","family":"Krampera","sequence":"additional","affiliation":[{"name":"Hematology Unit Department of Medicine University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1557-0957","authenticated-orcid":false,"given":"Marco","family":"Ruggeri","sequence":"additional","affiliation":[{"name":"Hematology Unit Ospedale San Bortolo Vicenza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Visco","sequence":"additional","affiliation":[{"name":"Hematology Unit Department of Medicine University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Tecchio","sequence":"additional","affiliation":[{"name":"Hematology Unit Department of Medicine University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,7,2]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006676-199409000-00019"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/jso.2930050410"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197201)29:1<252::AID-CNCR2820290138>3.0.CO;2-#"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/bjs.18004921319"},{"issue":"5","key":"e_1_2_7_6_1","first-page":"S27","article-title":"Classification OMS 2010 des tumeurs digestives: la quatri\u00e8me \u00e9dition [WHO Classification of digestive tumors: the fourth edition]","volume":"31","author":"Fl\u00e9jou JF","year":"2011","journal-title":"Ann Pathol"},{"key":"e_1_2_7_7_1","first-page":"335","article-title":"AIDS\u2010related non\u2010Hodgkin's lymphoma of the pancreas","volume":"92","author":"Jones WF","year":"1997","journal-title":"Am J Gastroenterol"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.21037\/jgo.2017.06.03"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1148\/rg.2018170201"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/COC.0b013e3182354bbb"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-014-4176-6"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gie.2015.09.028"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s005350200143"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12029-014-9657-0"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.3346\/jkms.2010.25.4.536"},{"issue":"1","key":"e_1_2_7_16_1","first-page":"ECR09","article-title":"Primary pancreatic lymphoma. A report of five cases","volume":"90","author":"Arcari A","year":"2005","journal-title":"Haematologica"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-01-643569"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2013.54.8800"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics9010018"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1080\/17474124.2018.1489230"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13244-017-0585-y"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.14136"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2013.51.5866"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1186\/1752-1947-2-167"},{"issue":"5","key":"e_1_2_7_25_1","first-page":"mdw175","article-title":"Extranodal diffuse large B\u2010cell lymphoma (DLBCL) and primary mediastinal B\u2010cell lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow\u2010up","volume":"27","author":"Vitolo U","year":"2016","journal-title":"Ann Oncol."},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11864-018-0555-8"}],"container-title":["European Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fejh.13468","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.13468","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ejh.13468","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ejh.13468","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T18:33:01Z","timestamp":1693938781000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ejh.13468"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,7,2]]},"references-count":25,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2020,10]]}},"alternative-id":["10.1111\/ejh.13468"],"URL":"https:\/\/doi.org\/10.1111\/ejh.13468","archive":["Portico"],"relation":{},"ISSN":["0902-4441","1600-0609"],"issn-type":[{"value":"0902-4441","type":"print"},{"value":"1600-0609","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,7,2]]},"assertion":[{"value":"2020-03-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-06-09","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-07-02","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}