Intraductal papillary-mucinous tumors: An entity which is infrequent and difficult to diagnose

BACKGROUND/AIMS: Intraductal papillary-mucinous tumor of the pancreas is cu rrently considered to be a tumor which is an entity of its own, different f rom classic pancreatic ductal carcinoma. It is basically characterized by s low growth and low malignancy potential, as: well: as by the production of mucin. The aim of this study is to contribute to world literature some clar ification of its natural history, clinical presentation, the most useful di agnostic tests, methods of detection of stromal invasion and handling of tr eatment, METHODOLOGY: Of 297 pancreatectomies undertaken at the "12 de Octubre" hosp ital between May 1985 and January 1998, only 1 case of Intraductal papillar y-mucinous tumor was found. We have revised 127 cases published in 10 serie s over the last 10 years. We also contribute a review of our own case. RESULTS: These tumors, which are very infrequent, produce non-specific symp toms, with long latency periods from the first symptom up to stromal 1 inva sion. Endoscopic retrograde cholangiopancreatography showed alterations in 100% of cases where this was undertaken. Tumor-related mortality was zero a mongst patients with non-invasive tumor who underwent surgery. None of the cases published presented upper gastrointestinal hemorrhage. This indicated the correct surgery and led us to our diagnosis. CONCLUSIONS:We confirm the low frequency and difficulty of diagnosis, the s ensitivity of endoscopic retrograde cholangiopancreatography, the difficult y of early detection of stromal invasion, and the high survival rate in cas es where resection is done before this occurs. Early diagnosis and treatmen t is therefore of utmost importance.