Primary pancreatic lymphoma – pancreatic tumours that are potentially curable without resection, a retrospective review of four cases

Institute of Oncology, Prince of Wales Hospital, Sydney, Australia.
BMC Cancer (Impact Factor: 3.36). 02/2006; 6(1):117. DOI: 10.1186/1471-2407-6-117
Source: PubMed


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.
Four cases of primary pancreatic lymphoma were identified at Prince of Wales Hospital, Sydney, Australia. A literature review of cases of PPL reported between 1985 and 2005 was conducted, and outcomes were contrasted.
All four patients presented with upper abdominal symptoms associated with weight loss. One case was diagnosed without surgery. No patients underwent pancreatectomy. All patients were treated with chemotherapy and radiotherapy, and two of four patients received rituximab. One patient died at 32 months. Three patients are disease free at 15, 25 and 64 months, one after successful retreatment. Literature review identified a further 103 patients in 11 case series. Outcomes in our series and other series of chemotherapy and radiotherapy compared favourably to surgical series.
Biopsy of all pancreatic masses is essential, to exclude potentially curable conditions such as PPL, and can be performed without laparotomy. Combined multimodality treatment, utilising chemotherapy and radiotherapy, without surgical resection is advocated but a cooperative prospective study would lead to further improvement in treatment outcomes.

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Available from: Melvin T Chin, Oct 14, 2014
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    • "Primary pancreatic lymphoma (PPL) is extremely rare, comprising 2.2% of non-Hodgkin's lymphomas (NHLs) and 4.9% of all pancreatic malignancies [1]. Clinical manifestations and imaging findings of PPL are similar to adenocarcinoma, but PPL must be distinguished from it because unlike pancreatic adenocarcinoma, PPL is potentially treatable [2, 3, 4]. Hepatitis C virus (HCV) infection has been associated with the development of B cell NHL (including diffuse large B cell lymphoma, marginal zone lymphoma and extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue) [5, 6, 7, 8, 9]. "
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    ABSTRACT: We report a rare case of pancreas tumor (lymphoma) in a patient with a history of chronic hepatitis C virus (HCV) infection without treatment, with a high viral load (20,199,805 IU/ml). He presented with abdominal pain, jaundice, weight loss and sweating. Computed tomography showed a hypodense mass located in the head of the pancreas, and immunohistochemistry of a specimen obtained by endoscopic ultrasound-guided fine needle aspiration revealed non-Hodgkin's lymphoma of the pancreas, B cell type. An association of HCV infection with pancreatic lymphoma has only been reported rarely in the literature and its clinical significance is uncertain.
    Case Reports in Gastroenterology 06/2012; 6(2):387-93. DOI:10.1159/000339693
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    • "Baylor and Berg reported the incidence of PPL as <0.5% in their pathology series of 5,000 pancreatic tumors [1]. A review of the literature between 1951 and 1999 included 122 patients [2], a more recent review compiled 11 series published between 1985 and 2005 and included 103 patients; 4 other patients were reported from the reviewers’ institution [3]. In comparison with most of the other pancreatic malignancies, PPL has much more favorable survival after aggressive treatment [4, 5]. "
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    ABSTRACT: Primary pancreatic lymphoma (PPL) is a rare tumor that usually presents with the clinical picture of advanced adenocarcinoma but has a much better prognosis. A 38-year-old man was referred after percutaneous transhepatic external biliary drainage for obstructive jaundice. Abdominal magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography had revealed a 5-cm pancreatic head mass that caused biliary tract dilation. Computed tomography angiography showed that the mass encased the celiac trunk as well as the common hepatic and splenic arteries. MRI also revealed a metastatic lesion at the third lumbar vertebra. Serum carcinoembryonic antigen and carbohydrate antigen 19-9 levels were within normal range. The initial diagnosis was inoperable pancreatic adenocarcinoma; however, Tru-Cut pancreatic biopsy showed a large B cell lymphoma. After 6 sessions of chemotherapy and 21 sessions of radiotherapy, both the pancreatic mass and the vertebral metastasis had disappeared. However, he had persistent distal common bile duct stricture that could not be negotiated by either the endoscopic or percutaneous route. A Roux-en-Y hepaticojejunostomy was performed. The patient stayed alive without recurrence for 52 months after the initial diagnosis and 45 months after completion of oncologic treatment. In conclusion, a large pancreatic mass with grossly involved peripancreatic lymph nodes, without ascites, liver or splenic metastasis, should alert the clinician to the possibility of PPL. Cure is possible by chemoradiotherapy even in the presence of vertebral metastasis. Persistent stricture in the distal common bile duct may require a biliodigestive anastomosis.
    Case Reports in Gastroenterology 09/2011; 5(3):642-7. DOI:10.1159/000334725
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    • "PPL is a very rare neoplasm that may be confused with adenocarcinoma, the most common neoplasm of the pancreas. An extensive review of the international literature has revealed a total of 162 adult patients with biopsy-proven NHL primarily involving the pancreas (Table 1) [2,3,10-17]. The clinical manifestation of PPL is non-specific and differentiation from pancreatic adenocarcinoma on a clinical basis is difficult. "
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    ABSTRACT: Primary pancreatic lymphoma is a very rare but manageable malignant tumour which may be clinically confused as a pancreatic carcinoma. This case report demonstrates the value of surgical resection in the management of pancreatic lymphoma. We report a case of a 65-year-old man who was admitted with obstructive jaundice, vague upper abdominal pain and weight loss. Ultrasonography and computed tomography showed a mass at the head of the pancreas which was compressing the bile duct. The patient underwent pancreaticoduodenectomy. Histopathologic and immunohistochemical assessment of the pancreatic lesion established the diagnosis of a diffuse, extranodal, high-grade B-cell non-Hodgkin's lymphoma. Several doses of chemotherapy were administered postoperatively and at present the disease remains in remission. The favourable outcome for this patient and a thorough review of the literature underline the important role that operative resection may have in the management of at least the early stage of primary pancreatic lymphoma.
    Journal of Medical Case Reports 02/2008; 2:167. DOI:10.1186/1752-1947-2-167
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