Lymphoplasmacytic sclerosing pancreatocholangitis successfully treated by pancreatoduodenectomy

A 68-year-old man complaining of jaundice was admitted to our hospital in October 1996. Radiological imaging studies, including dynamic computed tomography, endoscopic retrograde cholangiography, and angiography, were highly suggestive of pancreatic head cancer, and laparotomy was performed on October 25, 1996. On gross examination, the pancreas appeared firm, as in chronic pancreatitis, with a mass lesion in the pancreatic head measuring 35 x 35 x 25 mm. A pylorus-preserving pancreatoduodenectomy was carried out. Histological findings were characterized by uniform fibrosis with diffuse lymphoplasmacytic infiltration and lymph follicles in the thickened wall of the bile duct and in and around the pancreas, with acinar atrophy. The histological diagnosis was lymphoplasmacytic sclerosing pancreatocholangitis. Approximately 5 years postoperatively, the patient was alive and well without adjunctive corticosteroid therapy. This positive result suggests that pancreatoduodenectomy may be effective for lymphoplasmacytic sclerosing pancreatocholangitis that is localized in the pancreatic head.