Tu1238 Clinical Characteristics of Familial Intraductal Papillary Mucinous Neoplasm of the Pancreas

Abstract

Background & Aim: It is reported that 4~10% of patients with pancreatic cancer (PC) have a familial predisposition to their disease, however family history of intraductal papillary mucinous neoplasm (IPMN) is not well known. The aim of this study is to determine the clinical characteristics of IPMN with family history of pancreatic cancer, or familial IPMN. Methods: We analyzed 448 cases of IPMN for their demographic data and image findings [size of IPMN, maximum width main pancreatic duct (MPD), number of IPMN lesions, macroscopic-type (MPD-type or branch-type)] depicted by contrast enhanced CT and other modalities, and these findings were compared between familial IPMN (F-IPMN) and sporadic IPMN (S-IPMN). Family history of PC was analyzed in the first degree relatives of the patients. Results: Of 448 IPMN cases, 35 cases (8%) were F-IPMN and 413 cases (92%) SIPMN. Between two groups, no difference was seen in demographic data [F-IPMN vs. SIPMN; age (years): 70 vs. 70, gender (M/F): 20/15 vs. 257/156, smoking: 57% vs. 59%, drinking: 34% vs. 42%, diabetes: 34% vs. 20%]. In image findings, sizes (mm) of the tumor (36 vs. 32) and main pancreatic duct (MPD)(7 vs. 6) were slightly larger in familial than sporadic cases (P = 0.35 and 0.18), however cases with multiple IPMNs (34% vs. 17%, odds ratio: 2.5, P = 0.01) and those with MPD-type (43% vs. 20%, odds ratio: 3.3, P = 0.0007) were more frequently recognized in F-IPMN than S-IPMN. Conclusions: IPMN is recognized in 8% of the family of cases with PC, so that kindred of IPMN is the candidate for PC surveillance. For the clinical decisions of IPMN, we must bare in mind that the familial cases tends to develop multiple lesions and MPD-type, frequently associated with malignant lesion.