Clinical Features and Outcome of Primary Pancreatic Lymphoma

Abstract

Introduction Primary pancreatic lymphoma (PPL) is a rare tumor that is often misdiagnosed. Clinicopathologic features, optimal therapy, and outcomes are not well defined. We reviewed our institutional experience with PPL. Methods Search of our institutional database identified that between 1987–2012, 21,760 patients with lymphoma and 11,286 patients with a primary pancreatic tumor were evaluated. There were 44 patients with pathologically confirmed PPL. Clinical data were obtained by chart review and survival distributions were estimated using the Kaplan–Meier method and compared using the log-rank test. Results At baseline, LDH was elevated in 55 % of the patients, CA 19-9 in 25 %, and CEA in 20 %. Imaging characteristics included large, unresectable tumors (67 %), and lymphadenopathy inferior to the renal vein (50 %). Twenty-three patients underwent surgery for resection (5), diagnosis (13), or palliation (5). Chemotherapy alone achieved a 75 % complete response rate. Eight patients experienced relapse, 88 % of which occurred at distant sites. Median overall survival was 6.1 years and 10-year disease-specific survival (DSS) was 69 %. Patients with a low risk International Prognostic Index (IPI) and those with a follicular histologic subtype demonstrated 5-year DSS of 100 %. Conclusions Chemotherapy for PPL results in a high complete response rate and long DSS, which is similar to nodal non-Hodgkin’s lymphoma (NHL). A favorable outcome is expected for IPI low risk patients and follicular histologic subtype. Systemic therapy should generally be the initial therapy when the diagnosis is known. Prolonged follow up is recommended to detect relapses. Surgery alone should be reserved for non-curative intent (i.e. diagnostic or palliative).

Pancreatic T/histiocyte-rich large B-cell lymphoma: A case report and review of literature

“…PPL can also present as follicular lymphoma, small lymphocytic lymphoma, Burkitt’s lymphoma, and T cell lymphoma[17-19]. T/HRBCL is a nodal disease, but extralnodal sites, such as spleen, liver and bone marrow can be involved[10-12,20,21].…”

A Case of Primary Pancreatic non-Hodgkin B-cell Lymphoma Mimicking Autoimmune Pancreatitis

“…Gastrointestinal involvement by pancreatic lymphoma is reported in the literature as a possible but infrequent presentation of the disease [6]. To our knowledge, the only recent case was reported by Dupre et al in 2011 as a fatal upper gastrointestinal hemorrhage due to erosion of the duodenal wall by a pancreatic lymphoma [8].…”

“…Primary pancreatic lymphoma accounts for less than 1% of all malignant lymphomas and for 0.2% of all pancreatic malignancies [1,6]. It frequently represents a diagnostic challenge as it shares clinical and radiological findings with other pancreatic disorders such as other neoplasms and AIP, an inflammatory disease of the pancreas that usually presents with painless obstructive jaundice, pancreatic mass/enlargement and response to steroids administration.…”

Rare Pancreatic Tumors

“…14 Diagnostic confirmation through percutaneous/endoscopic US-guided biopsy or surgical biopsy is important, because pancreatic lymphoma needs to be treated by chemotherapy, whereas pancreatic adenocarcinoma treatment warrants surgery with or without chemoradiation. 17 Although PPL can arise anywhere in the pancreas, the incidence s higher in the head of pancreas due to abundance of lymphoid tissue in the head. PPL is usually large at presentation.…”