Primary Pancreatic lymphoma: a rare clinical entity

Area of Science:

• Oncology
• Hematology
• Gastroenterology

Background:

• Primary pancreatic lymphoma (PPL) is an extremely rare malignancy, accounting for less than 0.5% of pancreatic cancers and 1% of extranodal lymphomas.
• Its rarity leads to poorly defined clinicopathological features, diagnostic challenges, and suboptimal treatment strategies.
• Non-specific clinical presentations often lead to diagnostic pitfalls, delaying accurate diagnosis and appropriate management.

Observation:

• A 52-year-old male presented with epigastric pain and obstructive jaundice, found to have a pancreatic head mass.
• Imaging revealed a mass suspicious for malignancy involving the common bile duct, leading to biliary dilation.
• Biopsies confirmed high-grade B-cell pancreatic lymphoma.

Findings:

• The patient received multiple chemotherapy regimens including R-CHOP, DA-EPOCH-R, and RICE, with limited response.
• Despite aggressive treatment, the patient experienced disease progression and succumbed to the illness 5 months after initial presentation.
• This case highlights the diagnostic challenges and aggressive nature of PPL.

Implications:

• Distinguishing PPL from pancreatic adenocarcinoma preoperatively is critical due to mutually exclusive management and prognostic differences.
• Accurate differentiation can prevent unnecessary surgical resection for lymphoma patients.
• Further research into optimal therapeutic strategies for PPL is warranted, potentially including novel agents like CAR T-cell therapy.