Primary pancreatic lymphoma: Clinical presentation, diagnosis, treatment, and outcome.
Eur J Haematol
; 105(4): 468-475, 2020 Oct.
Article
em En
| MEDLINE
| ID: mdl-32542880
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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Pancreáticas
/
Linfoma
Tipo de estudo:
Clinical_trials
/
Diagnostic_studies
/
Etiology_studies
/
Guideline
Limite:
Female
/
Humans
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Male
País/Região como assunto:
Europa
Idioma:
En
Revista:
Eur J Haematol
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Itália