Lymphoplasmacytic Sclerosing Pancreatitis: Inflammatory Mimic of Pancreatic Carcinoma
Abstracts
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Introduction: Lymphoplasmacytic sclerosing pancreatitis (LP) is a rare form of pancreatitis that is often associated with autoimmune disease. Lymphoplasmacytic infiltration and associated fibrosis within the pancreas can result in a mass lesion clinically resembling adenocarcinoma. Little is known regarding its presentation and clinical course. Methods: Patients undergoing pancreatic resection for benign disease were identified from a prospective pancreatic database. Pathologic review of these cases was performed to identify and classify patients with LP. Patients were divided into two groups: (1) classic LP, patients having all four of the characteristic histologic features of LP, including lymphoplasmacytic infiltration of the pancreas, interstitial fibrosis, periductal inflammation, and periphlebitis, and (2) intermediate LP, patients with at least two of these histologic findings. Patient demographics, pathologic and clinical features, and outcome were analyzed. Results: From 1985 to 2001, 1287 pancreatic resections were performed, of which 159 had benign disease at pathologic evaluation. Of these, 31 had pathologic features consistent with LP. All 31 patients had a presumed preoperative diagnosis of pancreatic carcinoma based on clinical presentation and/or radiologic findings. Most patients presented with jaundice (21) or abdominal pain (7). The median age was 62 (range, 17-87) with a male: female ratio of 2:1. Six patients had a preoperative history of autoimmune disease. Eight (26%) patients developed recurrence of symptoms after resection, 7 with jaundice and 1 with recurrent pancreatitis (median time to recurrence, 11 months, median f/u 38 months). All patients with recurrent jaundice appeared to have biliary strictures at the time of direct cholangiography; no patient had evidence of malignancy. Patients with classic and intermediate LP had a similar clinical presentation and outcome. (Table) Conclusion: Lymphoplasmacytic sclerosing pancreatitis is a rare cause of pancreatitis that is difficult to differentiate clinically from carcinoma. Patients with classic and intermediate LP appear to have similar clinical behavior. A significant percentage of patients have a progressive course after resection, thus close follow-up is mandated in all patients. |
