Pancreatic lymphoma
Updates to Article Attributes
Pancreatic lymphoma is most commonly a B-cell sub-type of non-Hodgkin lymphoma.
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: ~ 85%
- mass: ~ 60%
- weight loss: ~ 50%
- obstructive jaundice: ~ 40% of cases
- acute pancreatitis: ~ 10%
The classic B-symptoms of non-Hodgkin lymphoma (e.g. fever, chills, night sweats) are present in only 2% of cases 1,2.
Pathology
Aetiology
It 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 <2% of extranodal lymphomas and 0.5% of pancreatic tumours 1,2
- 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
Location
Two morphologic patterns of pancreatic lymphoma are recognised 1:
- focal form: occurs in the pancreatic head in 80% of cases and has a mean size of 8 cm
- diffuse form: infiltrative, leading to glandular enlargement and poor definition, features that can simulate the appearance of acute pancreatitis
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
-</ul><h4>Treatment and prognosis</h4><p>Percutaneous or endoscopic core biopsy should be performed to establish the diagnosis <sup>2</sup>.</p><p>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<sup> 1</sup>.</p><p>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 <sup>2</sup>.</p><h4>Differential diagnosis</h4><p>Imaging differential considerations include</p><ul><li><a href="/articles/acute-pancreatitis">acute pancreatitis</a></li></ul><p>For a diffuse bulkiness to the pancreas consider</p><ul><li><a href="/articles/autoimmune-pancreatitis">autoimmune pancreatitis</a></li></ul>- +</ul><h4>Treatment and prognosis</h4><p>Percutaneous or endoscopic core biopsy should be performed to establish the diagnosis <sup>2</sup>.</p><p>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<sup> 1</sup>.</p><p>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 <sup>2</sup>.</p><h4>Differential diagnosis</h4><p>Imaging differential considerations include</p><ul>
- +<li><a href="/articles/acute-pancreatitis">acute pancreatitis</a></li>
- +<li>
- +<a title=" pancreatic adenocarcinoma" href="/articles/pancreatic-adenocarcinoma"> pancreatic adenocarcinoma</a> <sup>3</sup>
- +</li>
- +</ul><p>For a diffuse bulkiness to the pancreas consider</p><ul><li><a href="/articles/autoimmune-pancreatitis">autoimmune pancreatitis</a></li></ul>
References changed:
- 3. Fukita Y, Asaki T, Adachi S, Yasuda I, Toyomizu M, Katakura Y. Non-Hodgkin Lymphoma Mimicking Pancreatic Adenocarcinoma and Peritoneal Carcinomatosis. JCO. 2013;31(21):e373-6. <a href="https://doi.org/10.1200/jco.2012.45.2904">doi:10.1200/jco.2012.45.2904</a>