Intraductal mucin-hypersecreting neoplasms (IMHN) of the pancreas are a group of rare exocrine tumours characterized by the intraductal growth of a columnar mucinous epithelium that produces large amounts of mucin causing obstruction and dilatation of the main pancreatic duct (MPD) or its side branches leading to recurrent pancreatitis. We report 13 cases of IMHN (8 M, 5 F, mean age 69.0 years, range 41-85), diagnosed between 1990 and 1996 in order to discuss the diagnostic and therapeutic strategies for IMHN. Patients (pts.) presented recurrent pancreatitis (6 cases) or pancreatitis-like symptoms (5 cases) due to temporary complete occlusion of the MPD by viscous mucin; one of them developed diabetes and other two developed exocrine pancreatic insufficiency with weight loss and parenchimal atrophy as late sequela of permanent duct occlusion. Two other patients were admitted for gallstones and the last patient- was admitted for jaundice. At endoscopic retrograde cholangiopancreatography (ERCP) a wide open papilla filled with mucin was observed in 8 pts.; in two cases a patulous minor papilla extruding mucin was observed too. Cannulation of the papilla showed a dilatation of the MPD in 11 pts.; in 8 pts. the dilatation was cystic and in 3 pts. stones were detected in the dilated MPD. In all patients the pancreatogram showed filling defects of various sizes. Endoscopic treatment included pancreatic sphincterotomy (7 cases), insertion of a naso-pancreatic drainage (11 cases), insertion of a pancreatic stent (1 case), and lithotripsy with removal of pancreatic stones with dormia basket and fogarty balloon (1 case). Four patients were operated on: pylorus-preserving pancreatoduodenectomy was performed in 2 cases and distal pancreatic resection in 2 cases. The-postoperative course was uneventful in all cases. Histologically, a well-differentiated adenocarcinoma was diagnosed in one patient whereas no atypia were detected in the other three cases. A follow-up of respectively 4.0 years, 3.0 years, 1,8 years and 6 months disclosed no sign or recurrence or metastases. One 70 year-old patient refused surgery and is still alive and asymptomatic 1 year after endoscopic treatment. Due to age (>75 years) or clinical reasons 9 pts. were not referred for surgical treatment. Three of them died of unrelated causes respectively after 2 months, 3 months and 4 years; no recurrence of symptoms was noted during the follow-up. One patient died after 1 month of septic complications of acute pancreatitis. Two pts. are alive and asymptomatic respectively after 3 and 6 months. One patient is alive but affected with recurrent pancreatitis after 6 months. One patient was still alive and asymptomatic after 5 years but had had recurrent pancreatitis-like symptoms during the first three years. Our conclusions are: a) IMHN are slowly growing neoplasms that cause obstructive pancreatitis probably accounting for a significant number of cases of idiopatic calcified chronic pancreatitis b) ERCP is the most reliable procedure for proper clinical diagnosis of IMHN c) surgical resection should always be performed both to achieve relief from symptoms and to prevent a malignant or premalignant lesion from progressing to invasive carcinoma d) endoscopic treatment provides temporary improvement of symptoms in patients in whom surgical resection is considered inadvisable.
|Publication status||Published - 1997|
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