Intraductal papillary mucinous tumours of the pancreas. Clinical and therapeutic issues in 32 patients

• Published in: Gut Vol. 39; no. 3; pp. 457 - 464
• Main Authors: Azar, C, Van de Stadt, J, Rickaert, F, Devière, M, Baize, M, Klöppel, G, Gelin, M, Cremer, M
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Society of Gastroenterology 01.09.1996; BMJ; BMJ Publishing Group LTD
• Subjects: Acute Disease; Adult; Aged; Aged, 80 and over; Biological and medical sciences; Cholangiopancreatography, Endoscopic Retrograde; Cystadenoma, Mucinous - complications; Cystadenoma, Mucinous - diagnosis; Cystadenoma, Mucinous - surgery; Female; Follow-Up Studies; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical sciences; Middle Aged; Pancreatic Neoplasms - complications; Pancreatic Neoplasms - diagnosis; Pancreatic Neoplasms - surgery; Pancreatitis - etiology; Survival Rate; Treatment Outcome; Tumors; Adenocarcinoma; Human; Pancreatography; Radiodiagnosis; Acute; Pancreatitis; Radiologic investigation; Malignant tumor; Pancreatic duct; Differential diagnostic; Result; Papillary cystadenoma; Symptomatology; Treatment; Digestive diseases; Evolution; Benign neoplasm; Endoscopy; Pancreas; Pancreatic disease
• ISSN: 0017-5749; 1468-3288; 1458-3288
• DOI: 10.1136/gut.39.3.457

BACKGROUND/AIM: The clinical presentation, pancreatographic findings, and outcome of patients with intraductal papillary mucinous tumours have not been reported in a large patient series in the English literature. This study reviewed 32 patients diagnosed between 1980 and 1994, with special attention to these features. PATIENTS/METHOD: Data on 24 operated and eight non-operated patients were abstracted from inpatient, outpatient, and procedure records. RESULTS: Acute pancreatitis was the most common presentation seen in 56% of patients. Relapses occurred during an average of 43 months before diagnosis. A patulous papilla was observed in 55% of the cases. Endoscopic pancreatography showed communicating cysts, a diffusely dilated main pancreatic duct, and amorphous defects in 42, 71, and 97% respectively. An invasive carcinoma was found in nine of 24 (37.5%) of operated patients: six of the patients (66%) died or developed metastases within three years after surgery. No mortality was related to the tumour in absence of invasive carcinoma. Benign recurrence on the remaining pancreas was unusual and occurred late after surgery. CONCLUSIONS: Intraductal papillary mucinous tumours must be considered in the differential diagnosis of relapsing pancreatitis. Despite slow growing, these tumours have an obvious malignant potential and a very poor prognosis when invasive carcinoma has developed. Early recognition and resection are the cornerstones of treatment.