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

被引:26
作者
Facchinelli, Davide [1 ]
Sina, Sokol [2 ]
Boninsegna, Enrico [3 ]
Borin, Alex [4 ]
Tisi, Maria C. [5 ]
Piazza, Francesco [6 ]
Scapinello, Greta [6 ]
Maiolo, Elena [7 ]
Hohaus, Stefan [7 ]
Zamo, Alberto [8 ]
Merli, Michele [9 ]
Stefani, Piero M. [10 ]
Mellone, Federica [11 ]
Basso, Marco [12 ]
Sartori, Roberto [12 ]
Rusconi, Chiara [13 ]
Parisi, Alice [2 ]
Manfrin, Erminia [2 ]
Krampera, Mauro [1 ]
Ruggeri, Marco [5 ]
Visco, Carlo [1 ]
Tecchio, Cristina [1 ]
机构
[1] Univ Verona, Dept Med, Hematol Unit, Verona, Italy
[2] Univ Verona, Dept Diagnost & Publ Hlth, Verona, Italy
[3] ASST, Dept Radiol, Mantua, Italy
[4] Univ Verona, Pancreas Inst, Dept Surg & Oncol, Unit Gen & Pancreat Surg, Verona, Italy
[5] Osped San Bortolo, Hematol Unit, Viale Ferdinando Rodolfi 37, I-36100 Vicenza, Italy
[6] Univ Padua, Dept Med, Hematol Unit, Padua, Italy
[7] Univ Cattolica Sacro Cuore, Ist Ematol, Fdn Policlin Univ A Gemelli IRCCS, Rome, Italy
[8] AOU Citta Salute & Sci, Dept Oncol & Pathol Unit, Turin, Italy
[9] Univ Insubria, Osped Circolo & Fdn Macchi ASST Sette Laghi, Div Hematol, Varese, Italy
[10] Osped Ca Foncello, Hematol Unit, Treviso, Italy
[11] Univ Verona, Inst Med Stat, Verona, Italy
[12] Osped Casteifranco Veneto, Hematol Unit, Vicenza, Italy
[13] ASST Niguarda Hosp, Div Hematol, Milan, Italy
关键词
chemotherapy; lymphoma; pancreas; surgery; HODGKINS-LYMPHOMA; CELL LYMPHOMA;
D O I
10.1111/ejh.13468
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:468 / 475
页数:8
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