Collected series of resected intraductal papillary mucinous tumours (IPMTs) of the pancreas and correlation between the mucin expression profile and the malignant potential

s / Pancreatology 13 (2013) S2–S98 S79 Results: Of 291 PDAC patients 144 (49.4%) had cachexia at presentation (Cþ). Ca 19.9 values and the rate of jaundice (43.7% vs 40%;p1⁄40.5) at onset were similar in Cþ and C-. Cþ patients more frequently reported abdominal pain before diagnosis (56.4% vs 40.8% p1⁄40.004) and had longer diagnostic delay (4.8 vs 2.2 months; p<0.005). Age at diagnosis, and rate of diabetics were similar in Cþ and Cpatients, but Cþwere more frequently male (61.1% vs 47.6%) and overweight (mean BMI 28.6 vs 25.9 p1⁄40.0001). Tumor site, grading and size were similar, but Cþ patients were more frequently metastatic at diagnosis (44.4% vs 32.6%; p1⁄40.004). Survival probability was lower, yet not significantly, in Cþ patients (7 vs 11 months; p1⁄40.84). Conclusion: Weight loss resulting in cachexia is an onset symptom of some 50% PDAC patients, more frequently observed in male and overweight subjects. Cachexia is associated with abdominal pain and longer diagnostic delay, and with a higher rate of metastatic disease at diagnosis, which might have an impact on outcome. PII-89 Abstract id: 165. Disease spectrum of intraductal papillary mucinous neoplasm with an associated invasive carcinoma: Invasive IPMN versus pancreas ductal adenocarcinoma-associated IPMN Jin-Young Jang, Mee Joo Kang, Kyoung Bun Lee, Sun-Whe Kim. Seoul National University Hospital, South Korea Introduction: Current version of WHO classification introduced the concept of ‘intraductal papillary mucinous neoplasm (IPMN) with an associated invasive carcinoma’. However they include large spectrum of malignant disease from minimally invasive to similar to ductal adenocarcinoma. Aims: The authors investigated the clinicopathologic characteristics and prognosis of this disease category according to tumor morphology and percentage of invasive component. Patients & methods: Fifty-nine patients who underwent surgical resection of IPMN with an associated invasive carcinoma at Seoul National University Hospital were subgrouped according to the invasive component of <5% (minimally invasive IPMC; MI-IPMC), 5-50% (invasive IPMC;IPMCI), and 50% (Pancreatic ductal adenocarcinoma-associated IPMN;PDACIPMN). Prognosis was compared with 219 curatively resected conventional PDAC. Results: ElevenMI-IPMN (18.6%), 24 IPMC-I (40.7%) and 24 PDAC-IPMN (40.7%) were identified. With the transition from MI-IPMC to IPMC-I and PDAC-IPMN, percentage of advanced T (P<0.001) or N stage (P1⁄40.001), expression of S100A4 (P1⁄40.004), p53 (P1⁄40.028), and CD24 (P1⁄40.009) increased and SMAD4 expression decreased (P<0.001). The overall 5-year survival rate for MI-IPMC, IPMC-I and PDAC-IPMN were 80.8%, 59.0%, and 29.3%, respectively (P<0.001). PDAC-IPMN had poor prognosis compared with MI-IPMC (P1⁄40.011) or IPMC-I (P1⁄40.026), but had comparable prognosis with conventional PDAC (P1⁄40.138). Conclusion: PDAC-IPMN has different clinicopathological characteristics compared with the IPMC-I. IPMN with an associated invasive carcinoma is composed of a wide spectrum of disease. PII-90 Abstract id: 320. Collected series of resected intraductal papillary mucinous tumours (IPMTs) of the pancreas and correlation between the mucin expression profile and the malignant potential Bal azs Tihanyi , Katalin Borka , L aszl o Neh ez , Bernadett Barkaszi , J ozsef Tim ar , L aszl o Hars anyi , Tibor Tihanyi . 1st. Dept. of Surgery, Semmelweis University, Budapest, Hungary 2 2nd. Dept. of Pathology, Semmelweis University, Budapest, Hungary Introduction: IPMTs have attracted attention as a new pathological entity. Behind the different appearance of the different subtypes, there is different behaviour. Aims: The aim of our study was to present the different mucin expression and its correlation with the malignant potential. Patients & methods: The resected specimens were examined twice by different pathologists and when either different or uncertain diagnoses resulted, was the sample excluded. 41 patients were proved to have IPMT between the period of 2002-2012. Samples were immunhistochemically labelled for MUC1, MUC2 and MUC5AC. The patients were grouped according to type of the tumour, i.e. main duct(MD), branch duct(BD) or mixed-type (MX) IPMTs. We also created subgroups based on the mucin expression profile, like intestinal (I – MUC1-, MUC2þ, MUC5AC ), pancreatobiliary (PB – MUC1þ, MUC2-, MUC5AC ) and gastric-type (G – MUC1-, MUC2-, MUC5ACþ). Results: 13 MD-, 10 BDand 18 MXtype IPMTs were found. In total only 17,1% were found to be benign. Themalignancy rate in theMD, BD and MX groups was 61,5%, 60%, 72,2%, respectively. Based on the mucin expression profile 17 I-, 11 Gand 13 PBtype IPMTs were found. All patients in the PBgroup while 64,7% and 27,3% of the Iand G-groups were malignant, respectively. Conclusion: The lack of MUC1 expression may be related to a less invasive characteristics of IPMTs, while the MUC5AC expression alone can be related to benign potential. In contrast, MUC2þ and MUC5ACþ or MUC1þ and MUC5ACþ expression can increase the rate of success of diagnosing invasive IPMTs. PII-91 Abstract id: 249. The carboxyl-ester lipase (CEL) gene A risk factor for pancreatic cancer? Monica Dalva , Karianne Fjeld , Bente Berg Johansson , Solrun Steine , Dag Hoem , P al Rasmus Njolstad , Anders Molven . 1 KG Jebsen Center for Diabetes Research, Department of Clinical Science, University of Bergen, Norway 2 The Gade institute, Department of Clinical Medicine, University of Bergen, Norway Department of Surgery, Haukeland University Hospital, Bergen, Norway Introduction: The CEL gene is known to be highly polymorphic. We have previously described a rare syndrome of exocrine and endocrine pancreatic dysfunction, caused by single-base mutations in the CEL gene. In addition, we have identified copy number variants (CNVs) of the CELlocus and variable number of tandem repeat (VNTR)-length polymorphisms in CEL exon 11. Aims: The aim of this study was to examine if the CEL CNV alleles predispose to pancreatic cancer. We also investigated the association between pancreatic cancer and the CEL VNTR-lenght. Patients & methods: We examined patients with pancreas cancer in a Norwegian cohort (n1⁄4250), and Norwegian blood donors (n1⁄4190-233) were used as healthy controls. For screening of CEL CNVs, we used a duplex PCR assay. Genotyping of the CEL VNTR was performed using multiplex PCR and DNA fragment analysis. Results:We have identified three CEL CNVs, twowith gene duplication and one with a deletion. The carrier frequencies of the duplicated alleles were 4.6% and 2.9%, respectively, among the pancreas cancer patients, and 2.6% and 3.2% in the controls. The deleted allele was not detected in pancreas cancer, compared to 0.5% in the controls. When analyzing the CEL VNTR-lengths, we found alleles harboring from 11 to 23 repeats, with 16 repeats being the most frequent VNTR length. Alleles with more than 17 repeats were more frequent in patients with pancreatic cancer. Conclusion:We observed no statistical significant association between the CEL CNV alleles and pancreatic cancer. However, there is a tendency of a positive association between pancreatic cancer and increased CEL VNTRlength.