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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 tumours1-2.
  • 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 lymphadenopathy 1.

Epidemiology

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

Clinical presentation

Presentation is often non-specific. 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 (e.g. 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 cm 1
  • the diffuse form is infiltrative, leading to glandular enlargement and poor definition, features that can simulate the appearance of acute pancreatitis 1

Radiographic features

CT
  • minimal enhancement
  • peripancreatic lymph node enlargement
  • it typically has uniform low attenuation
  • diffuse enlargement (diffuse form) may simulate acute pancreatitis
  • encasement of the peripancreatic 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 seen 1

Treatment and prognosis

Percutaneous or endoscopic core biopsy should be performed to establish the diagnosis 2.

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 cases 1.

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

Imaging differential considerations include

For a diffuse bulkiness to the pancreas consider

See also

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