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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

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

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  • Medientyp: E-Artikel
  • Titel: Primary pancreatic lymphoma: Clinical presentation, diagnosis, treatment, and outcome
  • Beteiligte: 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
  • Erschienen: Wiley, 2020
  • Erschienen in: European Journal of Haematology
  • Sprache: Englisch
  • DOI: 10.1111/ejh.13468
  • ISSN: 0902-4441; 1600-0609
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <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‐defined 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 ≥15 years, 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‐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.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>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.</jats:p></jats:sec>