Objective: To compare the presentation and short-term results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis (LPSP) and pancreatic adenocarcinoma (PA) and to provide long-term follow-up on patients undergoing resection for LPSP. Summary Background Data: LPSP is a rare form of chronic pancreatitis characterized by a mixed inflammatory infiltrate centered around pancreatic ducts and ductules, combined with obliterative phlebitis. Its presentation may mimic that of PA. Methods Among 1,648 patients undergoing pancreaticoduodenectomy at the authors' institution from January 1992 to May 2002, 37 with LPSP were identified. The demographics, clinical features, and short- and long-term outcomes of these patients were analyzed. Where applicable, comparisons were made to a consecutive group of 45 patients who underwent pancreaticoduodenectomy for PA from July 2001 to December 2001. Results: Twenty-four percent (9/37) of the LPSP patients and none of the PA patients had a history of either atopic or autoimmune disease. The LPSP patients had a similar clinical presentation to the PA patients, but the LPSP patients were less likely to have a discrete pancreatic mass on CT and more likely to have CT findings suggesting a diffusely enlarged pancreas. The LPSP patients were also less likely to have a discrete pancreatic mass at operation, more likely to have a diffusely firm or hard gland, and more likely to have a difficult portal vein/superior mesenteric vein dissection when compared to the PA patients. Transfusion requirement, operative time, postoperative length of stay, and overall complication rate were similar between groups; however, the LPSP patients had a greater operative blood loss. The median length of follow-up for LPSP patients was 33 months. No patients had recurrent jaundice. One patient with LPSP had clinically evident recurrent pancreatitis. Among LPSP patients available for current telephone interview, 68% subjectively rated their quality of life as better, 18% reported no change, and 14% reported diminished quality of life compared to before surgery. Conclusion: LPSP mimics PA in clinical presentation, though CT findings of a diffusely enlarged pancreas without a discrete mass may suggest a diagnosis of LPSP. Nevertheless, differentiation from pancreatic neoplasia remains difficult. Patients undergoing pancreaticoduodenectomy for LPSP have durable relief of symptoms and a subjectively improved quality of life.
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