Pancreatic lymphoma
Updates to Article Attributes
Pancreatic lymphoma is most commonly a B-cell sub-type of non-Hodgkin lymphoma and is classified as either primary or secondary:.
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primary pancreatic lymphomais 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 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 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 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 cases1-2,2.
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/patterns
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
-<p><strong>Pancreatic lymphoma</strong> is most commonly a B-cell sub-type of non-Hodgkin <a href="/articles/lymphoma">lymphoma</a> and is classified as either primary or secondary:</p><ul>-<li>-<strong>primary pancreatic lymphoma</strong> 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 <sup>1-2</sup>-</li>-<li>-<strong>secondary lymphoma:</strong> 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<sup> 1</sup>-</li>-</ul><h4>Epidemiology</h4><p>Typically seen in middle-aged patients: mean of 55 years; range, 35-75 years and in immunocompromised patients.</p><h4>Clinical presentation</h4><p>Presentation is often non-specific. reported symptoms include <sup>1</sup>:</p><ul>- +<p><strong>Pancreatic lymphoma</strong> is most commonly a B-cell sub-type of non-Hodgkin <a href="/articles/lymphoma">lymphoma</a>.</p><h4>Epidemiology</h4><p>Typically seen in middle-aged patients: mean of 55 years; range, 35-75 years and in immunocompromised patients.</p><h4>Clinical presentation</h4><p>Presentation is often non-specific. reported symptoms include <sup>1</sup>:</p><ul>
-</ul><p>The classic B-symptoms of non-Hodgkin lymphoma (e.g. fever, chills, night sweats) are present in only 2% of cases<sup>1-2</sup>.</p><h4>Pathology</h4><h5>Location/patterns</h5><p>Two morphologic patterns of pancreatic lymphoma are recognised <sup>1</sup>:</p><ul>- +</ul><p>The classic B-symptoms of non-Hodgkin lymphoma (e.g. fever, chills, night sweats) are present in only 2% of cases <sup>1,2</sup>.</p><h4>Pathology</h4><h5>Aetiology</h5><p>It is classified as either primary or secondary:</p><ul>
- +<li>
- +<strong>primary pancreatic lymphoma</strong> 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 <sup>1,2</sup>
- +</li>
- +<li>
- +<strong>secondary lymphoma:</strong> 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<sup> 1</sup>
- +</li>
- +</ul><h5>Location</h5><p>Two morphologic patterns of pancreatic lymphoma are recognised <sup>1</sup>:</p><ul>
Systems changed:
- Haematology
- Oncology