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

Intraductal papillary-mucinous tumor of the pancreas is currently considered to be a tumor which is an entity of its own, different from classic pancreatic ductal carcinoma. It is basically characterized by slow 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 clarification of its natural history, clinical presentation, the most useful diagnostic tests, methods of detection of stromal invasion and handling of treatment.

Of 297 pancreatectomies undertaken at the "12 de Octubre" hospital between May 1985 and January 1998, only 1 case of Intraductal papillary-mucinous tumor was found. We have revised 127 cases published in 10 series over the last 10 years. We also contribute a review of our own case.

These tumors, which are very infrequent, produce non-specific symptoms, with long latency periods from the first symptom up to stromal invasion. Endoscopic retrograde cholangiopancreatography showed alterations in 100% of cases where this was undertaken. Tumor-related mortality was zero amongst 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.

We confirm the low frequency and difficulty of diagnosis, the sensitivity of endoscopic retrograde cholangiopancreatography, the difficulty of early detection of stromal invasion, and the high survival rate in cases where resection is done before this occurs. Early diagnosis and treatment is therefore of utmost importance.