Multicentric papillary-cystic neoplasm of the pancreas

Two distinct papillary-cystic neoplasms were found in the pancreas of a young black woman. She presented to the hospital in her first trimester of pregnancy with the chief complaint of sharp right upper quadrant abdominal pain that radiated to the right shoulder. This was associated with jaundice, vomiting, and pruritus. On examination, a large, nontender, midepigastric abdominal mass was palpated. Serum liver enzyme levels were moderately to markedly elevated. An abdominal computed tomographic scan revealed a 9-cm solid and cystic mass located within the head of the pancreas with associated marked bile duct dilatation. A total pancreatectomy was performed. Gross examination of the specimen revealed two separate well-circumscribed tumors of unequal size. The larger one was found within the head of the pancreas and contained multiple hemorrhagic, cystic cavities. The smaller one, located within the tail, consisted primarily of solid tissue. Microscopic examination of both lesions revealed papillary-cystic neoplasms. To our knowledge, this is the first report of two synchronous papillary-cystic tumors of the pancreas and the first reported demonstration of the potential of this tumor for multicentricity.