Primary Pancreatic Lymphoma: Clinical Presentation, Diagnosis, Treatment and Outcome
| Autor: | Cristina Tecchio, Alice Parisi, Piero Maria Stefani, Marco Ruggeri, Carlo Visco, Federica Mellone, Enrico Boninsegna, Davide Facchinelli, Francesco Piazza, Maria Chiara Tisi, Elena Maiolo, Alberto Zamò, Erminia Manfrin, Stefan Hohaus, Sokol Sina, Roberto Sartori, Michele Merli, Alex Borin, Greta Scapinello, Chiara Rusconi, Marco Basso, Mauro Krampera |
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| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Male
Abdominal pain medicine.medical_specialty Biopsy medicine.medical_treatment lymphoma chemotherapy Gastroenterology surgery 03 medical and health sciences 0302 clinical medicine Internal medicine Humans Medicine pancreas Neoplasm Staging Chemotherapy medicine.diagnostic_test business.industry Disease Management Hematology General Medicine Jaundice medicine.disease Debulking Lymphoma Pancreatic Neoplasms Patient Outcome Assessment Pancreatic Lymphoma Italy 030220 oncology & carcinogenesis Female Disease Susceptibility Neoplasm Grading Symptom Assessment medicine.symptom business 030215 immunology Rare disease |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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