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Pancreatic lymphoma

Pancreatic lymphoma is most commonly a B-cell sub-type of non-Hodgkin lymphoma and is classified as either primary or secondary.

  • primary pancreatic lymphoma is a rare extranodal manifestation of any histopathologic subtype of B-cell non-Hodgkin's lymphoma, representing less than 2% of extranodal lymphomas and 0.5% of pancreatic tumors1-2.
  • secondary lymphoma : found in 30% of non-Hodgkin lymphoma patients with widespread disease - it is the dominant form and is the result of direct extension from peripancreatic lymphadenopathy1.

Epidemiology

Typically seen in middle-aged patients: mean of 55 years; range, 35 - 75 years and in immunocompromised patients.

Clinical presentation

Presentation is often nonspecific. reported symptoms include 1

  • abdominal pain:  ~ 83%
  • a mass : ~ 58%
  • weight loss : ~ 50%1
  • obstructive jaundice : ~ 37 - 42% of cases
  • acute pancreatitis : 12 % 

The classic symptoms of non-Hodgkin lymphoma (eg, fever, chills, night sweats) are present in only 2% of cases1-2.

Pathology

Location / patterns

Two morphologic patterns of pancreatic lymphoma are recognized:

  • the focal form occurs in the pancreatic head in 80% of cases and has a mean size of 8 cm1.
  • the diffuse form is infiltrative, leading to glandular enlargement and poor definition, features that can simulate the appearance of acute pancreatitis1.

Radiographic features

CT
  • minimal enhancement.
  • peripancreatic lymphnode enlargement.
  • it typically has uniform low attenuation.
  • diffuse enlargement (diffuse form) may simulate acute pancreatitis.
  • encasement of the peripancreativ vessels may occur. Vascular invasion is less common in lymphoma than in adenocarcinoma 1-2.
MRI
  • focal form : low signal intensity on T1-weighted images and intermediate signal intensity on T2-weighted images (slightly higher signal intensity than the pancreas but lower signal intensity than fluid), and shows faint contrast enhancement 1.
  • diffuse form: low signal intensity on T1- and T2-weighted MR images and shows homogeneous contrast enhancement, although small foci of reduced or absent enhancement are sometimes seen1.

Treatment and prognosis

Percutaneous or endoscopic core biopsy should be performed to establish the diagnosis2.

Pancreatic lymphoma carries a better prognosis than adenocarcinoma because first-line treatment with chemotherapy is generally effective in producing long-term disease regression or remission. Surgery is not required in most cases1.

Using complex treatment approaches, cure rates of up to 30% are reported for patients with primary pancreatic lymphoma. This prognosis is much better than the dismal 5% 5-year survival rate in patients with pancreatic adenocarcinoma 2.

Differential diagnosis

Conderations include

See also

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