Objective: The authors compared the clinicopathologic features of the intraductal papillary and mucinous cystic neoplasms of the pancreas and clarified the similarities as well as the differences between these two tumors. In addition, they reviewed 104 cases of the intraductal papillary neoplasm in the English literature to provide a global view of the condition.
Summary background data: Controversy about the term and clinicopathologic entity still exist regarding intraductal papillary neoplasm of the pancreas. Currently, with only a few cases of this rare tumor in each report, there continues to be inadequate knowledge available regarding the tumor and methods by which to distinguish it from the mucinous cystic neoplasm.
Methods: Multiple demographic and clinicopathologic parameters were compared between intraductal papillary and mucinous cystic neoplasms identified from 1985 to 1994 in the Medical Center, Veterans General Hospital--Taipei.
Results: There were four intraductal papillary adenocarcinomas and 10 mucinous cystic neoplasms (8 cystadenocarcinoma and 2 cystadenoma). The sex, age, size, tumor location, and pathologic findings were quite different between these two groups. Clinical presentation of intraductal papillary adenocarcinomas were similar to those of periampullary tumors. The most common presentations of mucinous cystic neoplasm were epigastric pain and abdominal mass. All four intraductal papillary adenocarcinoma showed mucin secretion from a patulous orifice of the ampulla of Vater and filling defects in the dilated main pancreatic duct by endoscopic retrograde cholangiopancreatography (ERCP). Accurate preoperative diagnosis was not easy regarding either group. Serum carbohydrate antigen 19-9 (CA 19-9) was more useful for diagnosis in both groups.
Conclusions: The intraductal papillary neoplasm is a unique clinical entity but not a variant of mucinous cystic neoplasm in terms of sex, age, size, tumor location, or pathologic picture. The pathognomonic findings of ERCP should lead to diagnosis. Very aggressive surgical procedures should be attempted for these two mucin-producing neoplasms with low-grade malignancy.