Imaging Pearls ❯ Pancreas ❯ Pancreatic Lymphoma
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- • Primary pancreatic lymphoma (PPL) is a rare haematological disease
• PPL presents imaging features in common with pancreatic carcinoma but also some distinctive findings
• The majority of PPLs are large lesions with delayed homogeneous enhancement
• Peri-pancreatic fat stranding and vessel encasement are common in PPL
• Vascular infiltration and pancreatic duct dilatation are rare in PPL
CT imaging of primary pancreatic lymphoma: experience from three referral centres for pancreatic diseases
Enrico Boninsegna et al.
Insights Imaging. 2018 Feb; 9(1): 17–24. - “Vascular encasement was typical, but significant irregularities in the vessels wall were never found. This peculiarity could be helpful in the differential diagnosis with ductal adenocarcinoma, which causes vascular infiltration at early stages, with erosion of vessel walls, stenoses and eventually tumoral thrombosis. The pancreatic parenchyma affected by lymphoma has low attenuation values compared to the healthy pancreas; as opposed to adenocarcinoma, areas of necrosis are rare. After contrast medium administration the attenuation is higher in the portal-venous phase compared to the arterial-pancreatic phase. Enlarged retroperitoneal lymph nodes are frequent and fat stranding is always present. The double duct sign, simultaneous dilation of the common bile and pancreatic ducts, typical in patients with adenocarcinoma of the pancreatic head, was only depicted in 5/14 PPLs.”
CT imaging of primary pancreatic lymphoma: experience from three referral centres for pancreatic diseases
Enrico Boninsegna et al.
Insights Imaging. 2018 Feb; 9(1): 17–24. - “In conclusion, the most suggestive CT signs of primary pancreatic lymphoma are a large hypodense mass lesion with progressive contrast enhancement, absence of necrosis, lack of dilation of the main pancreatic duct and presence of vessel encasement without infiltration.”
CT imaging of primary pancreatic lymphoma: experience from three referral centres for pancreatic diseases
Enrico Boninsegna et al.
Insights Imaging. 2018 Feb; 9(1): 17–24. - Groove Pancreatitis
Soft tissue within pancreaticoduodenal groove with or without delayed enhancement, small cystic lesions along the medial duodenal wall
A Clinical and Radiologic Review of Uncommon Types and Causes of Pancreatitis Shanbhogue
AK et al.
RadioGraphics 2009; 29:1003-1026
- "In pancreatic lymphoma, mild common bile duct dilatation is more common than is main pancreatic duct dilatation. Enlarged lymph nodes, infiltration to retroperitoneal or abdominal organs, and invasive tumor growth with loss of anatomic boundaries are also more commonly seen in lymphoma. Also, vascular invasion, tumor calcification, and necrosis are less common in pancreatic lymphoma than in PDAC. Considering the better prognosis of pancreatic lymphoma and availability of chemotherapy as an effective treatment, the accurate diagnosis of lymphoma and distinguishing it from PDAC is critical.”
Pitfalls in the MDCT of pancreatic cancer: strategies for minimizing errors.
Abdom Radiol 45, 457–478 (2020).
Haj-Mirzaian A, Kawamoto S, Zaheer A, Hruban RH, Fishman EK, Chu LC.
- “Most commonly, lymphoma found in the pancreas is associated with widespread disease. When the bulk of the disease is localized to the pancreas, even in the presence of contiguous nodal involvement or distant spread, it is considered primary pancreatic lymphoma (PPL). PPL is most commonly non-Hodgkin lymphoma, and the B-cell subtype is much more common than the T-cell subtype and has a better prognosis. The most common histologic subtype is diffuse large B-cell lymphoma (DLBCL), followed by follicular lymphoma.”
Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential Maria A. Manning et al. RadioGraphics 2018; 38 (in press) - “PPL accounts for 0.5% of
all pancreatic tumors and 0.6% of extranodal lymphomas. Although PPL can occur in both young and elderly patients (age range, 15–85 years), most patients are middle-aged (median age, 62.5 years), male (55%), and white (75%) . It is more commonly seen in immunocompromised patients, usually associated with organ transplantation and human immunodeficiency virus (HIV)/ acquired immunodeficiency syndrome (AIDS). Although the pancreatic head is the most common location for PPL, obstructive jaundice is infrequently a symptom at presentation. The most common reported symptoms for patients with PPL are abdominal pain and weight loss.”
Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential Maria A. Manning et al. RadioGraphics 2018; 38 (in press)
- “Neoplastic and inflammatory diseases that can closely simulate pancreatic adenocarcinoma include neuroendocrine tumor, metastasis to the pancreas, lymphoma, groove pancreatitis, autoimmune pancreatitis, and focal chronic pancreatitis. Atypical imaging findings that should suggest diagnoses other than adenocarcinoma include the absence of significant duct dilatation, incidental detection, hypervascularity, large size (> 5 cm), IV tumor thrombus, and intralesional ducts or cysts.”
Pancreatic Imaging Mimics: Part 1, Imaging Mimics of Pancreatic Adenocarcinoma Fergus V. Coakley et al. American Journal of Roentgenology. 2012;199: 301-308
- “An enhancing pancreatic tumor without upstream dilatation of the main pancreatic duct should suggest primary pancreatic lymphoma.”
Imaging features of rare pancreatic tumors M. Barrala, S.A. Faraound, E.K. Fishman, A. Dohan, C. Pozzesserea, M.-A. Berthelina,P. Bazeries, M. Barat, C. Hoeffel, P. Soyer Diagnostic and Interventional Imaging (2016) (in press)
- “Primary pancreatic lymphoma is usually a non-Hodgkin lymphoma. Primary pancreatic lymphoma is extremely rare whereas secondary involvement of the pancreas by an abdominal lymphoma is more common. Mostly located in the head, primary pancreatic lymphoma may mimic pancreatic carcinoma. Behrns et al. have reported suggestive criteria for the diagnostic of primary pancreatic lymphoma. They include a tumor predominantly located within the pancreas, with lymph node involvement confined to the peripancreatic region, no superficial or mediastinal lymphadenopathy, hepatic or splenic involvement, and normal white blood cell count.”
Imaging features of rare pancreatic tumors M. Barrala, S.A. Faraound, E.K. Fishman, A. Dohan, C. Pozzesserea, M.-A. Berthelina,P. Bazeries, M. Barat, C. Hoeffel, P. Soyer Diagnostic and Interventional Imaging (2016) (in press)
- “Primary pancreatic lymphomas (PPL) are rare tumours of the pancreas. Symptoms, imaging and tumour markers can mimic pancreatic adenocarcinoma, but they are much more amenable to treatment. Treatment for PPL remains controversial, particularly the role of surgical resection.”
Primary pancreatic lymphoma--pancreatic tumours that are potentially curable without resection, a retrospective review of four cases
Grimison PS et al.
BMC Cancer 2006 May 4;6:117 - “Primary pancreatic lymphoma (PPL) is an extremely rare tumor (<1% incidence) and is often confused with pancreatic adenocarcinoma.”
Pancreaticoduodenectomy for Primary Pancreatic Lymphoma
Rose JF et al.
Gastrointest Cancer Res 2012 Jan-Feb; 5(1):32-34 - “Of all pancreatic malignancies, PPL accounts for only 1%–2%; the diffuse, large, B-cell lymphoma subtype is the most common PPL.This subtype is considered a subtype of non-Hodgkin's lymphoma (NHL), but it is rare for NHL to manifest in this manner. NHL typically manifests in the nodes; extranodal NHL occurs in only 20%–30% of NHL patients. Most cases of extranodal NHL originate in the gastrointestinal tract; only 0.6% of cases of NHL originate in the pancreas.”
Pancreaticoduodenectomy for Primary Pancreatic Lymphoma
Rose JF et al.
Gastrointest Cancer Res 2012 Jan-Feb; 5(1):32-34 - “Diffuse, large, B-cell lymphoma of the pancreas can manifest in two different ways on CT: (1) the pancreas has a similar appearance to pancreatitis, with an infiltrative mass and peripancreatic fat stranding1 or (2) the pancreas seems to have a well-circumscribed tumor. In both CT presentations, peripancreatic lymphadenopathy can be significant, often enough to obscure the borders of the pancreas.”
Pancreaticoduodenectomy for Primary Pancreatic Lymphoma
Rose JF et al.
Gastrointest Cancer Res 2012 Jan-Feb; 5(1):32-34 - “A tissue biopsy is necessary for a definitive diagnosis. Initially the biopsy can be performed by fine-needle aspiration (FNA). Although FNA is the most common initial biopsy method, it frequently results in both false-negative and false-positive results and often does not provide enough information to classify the lymphoma. More tissue is needed for flow cytometric analysis, which can make the diagnosis more accurately.”
Pancreaticoduodenectomy for Primary Pancreatic Lymphoma
Rose JF et al.
Gastrointest Cancer Res 2012 Jan-Feb; 5(1):32-34 - “Non-Hodgkin lymphoma predominantly involving the pancreas is a rare tumor and accounts for less than 0.7% of all pancreatic malignancies and 1% of extranodal lymphomas. Diagnosis of primary pancreatic lymphoma can be difficult because it may mimic carcinoma.”
Primary pancreatic lymphoma: diagnostic and therapeutic dilemma
Battula N et al
Pancreas. 2006 Aug;33(2):192-4.
- Pancreatic Lymphoma: Facts
- May simulate a pancreatic adenocarcinoma but usually more infiltrating, and does not obstruct the pancreatic duct usually
- More common to be secondary involvement by lymphoma than primary involvement
- When primary the pancreas may be the only organ of involvement like this case. When secondary will involve multiple organs and organ systems - Pancreatic Lymphoma: Facts
- Secondary Lymphoma may have;
- Large homogeneous mass
- Peripancreatic adenopathy
- Peripancreatic vessels splayed or stretched but not occluded
- Pancreatic duct dilatation is rare
- Pancreatic Lymphoma: Facts
- May simulate a pancreatic adenocarcinoma but usually more infiltrating, and does not obstruct the pancreatic duct usually
- More common to be secondary involvement by lymphoma than primary involvement
- When primary the pancreas may be the only organ of involvement like this case. When secondary will involve multiple organs and organ systems - Pancreatic Lymphoma: Facts
- Secondary Lymphoma may have;
- Large homogeneous mass
- Peripancreatic adenopathy
- Peripancreatic vessels splayed or stretched but not occluded
- Pancreatic duct dilatation is rare - Pancreatic Involvement by Lymphoma: Findings
- Pancreas is involved in about 30% of cases of NHL
- Patterns include a circumscribed mass, diffuse infiltration with glandular enlargement
- Pancreatic duct dilatation is rare
- Pancreatic Lymphoma
- Primary pancreatic lymphoma is a rare extranodal manifestation of any histopathologic subtype of B-cell non-Hodgkin's lymphoma that predominantly involves the pancreas
- Instead of being primary in the gland, pancreatic lymphoma can also represent direct extension from adjacent peripancreatic lymphadenopathy.
- Merkle AJR 2000; 174:671-675.
- Pancreatic Lymphoma
- Diagnostic criteria of primary pancreatic lymphoma include a pancreatic mass that predominates with gross involvement of only the peripancreatic lymph nodes
- no hepatic or splenic involvement
- no palpable superficial lymphadenopathy
- no enlargement of the mediastinal lymph nodes
- normal leukocyte count
- Pancreatic Lymphoma
- Pancreas is involved secondarily in more than 30% of patients with non-Hodgkin's lymphoma but < 2% of extranodal non-Hodgkin's lymphomas arise in the pancreas
- The incidence increases to 5% in HIV patients because the gastrointestinal tract is the most commonly affected extranodal site in AIDS-related non-Hodgkin's lymphoma
- Pancreatic Lymphoma
- CT Findings
- Focal mass
- Can be easily misinterpreted as adenocarcinoma, especially in patients with ductal dilatation (usually <5mm)
- If enlarged lymph nodes are encountered below the level of the renal veins, virtual exclusion of adenocarcinoma is possible
- Diffuse infiltrating mass replacing most of the pancreatic gland
- May mimic the imaging findings of acute pancreatitis with gland enlargement and irregular infiltration of the peripancreatic fat