Risk factors for intraductal papillary mucinous neoplasm (IPMN) of the pancreas: a multicentre case-control study.

OBJECTIVES: To investigate environmental, personal, and hereditary risk factors associated with the occurrence of intraductal papillary mucinous neoplasms of the pancreas (IPMNs). METHODS: Multicentre case-control study. Risk factors were identified from a questionnaire collecting data on family and medical history, and environmental factors. Cases were prevalent IPMNs seen at the participating units within an 18-month timeframe. Matched controls were enrolled alongside patients seen at outpatient clinics. RESULTS: Three-hundred and ninety patients with IPMN and 390 matched controls (166 males, mean age 65 in each group) were enrolled. Of the IPMNs, 310 had branch-duct involvement and 80 main-duct involvement. The only cancer with a 1st degree family history significantly higher in IPMN was pancreatic ductal adenocarcinoma (PDAC) (5.4% vs. 1.5%). Previous history of diabetes (13.6% vs. 7.5%), chronic pancreatitis (CP) (3.1% vs. 0.3%), peptic ulcer (7.2% vs. 4.3%), and insulin use (4.9% vs. 1.1%) were all more frequent with IPMNs. Logistic regression multivariate analysis revealed that history of diabetes (odds ratio (OR): 1.79, confidence interval (CI) 95%: 1.08-2.98), CP (OR: 10.10, CI 95%: 1.30-78.32), and family histories of PDAC (OR: 2.94, CI 95%: 1.17-7.39) were all independent risk factors. However, when analysis was restricted to diabetics who had taken insulin, risk of IPMN became stronger (OR: 6.03, CI 95%: 1.74-20.84). The association with all these risk factors seemed stronger for the subgroup with main duct involvement. CONCLUSIONS: A previous history of diabetes, especially with insulin use, CP, and family history of PDAC are all relevant risk factors for the development of IPMN. These results suggest an overlap between certain risk factors for PDAC and IPMN.

Endodermal sinus tumor of the omentum: case report.

We report a case of primary endodermal sinus tumor of the omentum in a 46-year-old woman. The patient had been referred to our hospital for pain in the right abdomen. CT scan revealed a right-sided abdominal mass. At laparotomy, a large omental tumor extending to the bowel, uterus, ovaries and peritoneum was found, and it was totally resected. The tumor displayed the typical histological patterns observed in endodermal sinus tumor, and the immunohistochemical findings confirmed the diagnosis. Serum AFP level was 21.550 ng/ml prior to laparotomy, and decreased to 13.845 ng/mL after surgery, whereas the level of serum beta human chorionic gonadotropin was normal. Post-surgery, the patient received combination chemotherapy consisting of cisplatin, etoposide and bleomycin every 3 weeks for a total of 6 cycles. At the end of the treatment, all the findings, including AFP level, were normal.

Diagnostic value of tumor M2-pyruvate kinase in neuroendocrine tumors. A comparative study with chromogranin A.

OBJECTIVE: M2-pyruvate kinase isoenzyme (M2-PK) is expressed by undifferentiated or proliferating tissue. The aim of the study was to establish the diagnostic role of tumor M2-PK (TM2-PK) in patients with neuroendocrine tumors and to compare its diagnostic value with that of chromogranin A (CgA) which is well-established. MATERIALS AND METHODS: Forty-nine patients with proven neuroendocrine tumors were divided as follows: subgroup A (17 patients in whom the tumor was surgically removed), subgroup B (9 patients with the neoplasm without metastases) and subgroup C (23 patients with tumor and distant metastases). Twenty-four healthy subjects were also studied as controls. Plasma TM2-PK and CgA were measured using ELISA techniques. RESULTS: TM2-PK concentrations (cut-off value 5.6 U/mL) were abnormally high in 25.0% of the healthy subjects, in 41.2% of the patients of subgroup A, in 66.7% of those of subgroup B and in 78.3% of those of subgroup C. CgA concentrations (cut-off value 5.0 U/L) were normal in all healthy subjects, whereas they were abnormally high in 76.5% of the patients of subgroup A, in 88.9% of those of subgroup B and in 95.7% of those of subgroup C. CONCLUSION: Since TM2-PK has a sensitivity similar to that of CgA while its specificity is significantly lower (p = 0.031), it seems of limited value in detecting neuroendocrine tumors.

Italian consensus guidelines for the diagnostic work-up and follow-up of cystic pancreatic neoplasms.

This report contains clinically oriented guidelines for the diagnostic work-up and follow-up of cystic pancreatic neoplasms in patients fit for treatment. The statements were elaborated by working groups of experts by searching and analysing the literature, and then underwent a consensus process using a modified Delphi procedure. The statements report recommendations regarding the most appropriate use and timing of various imaging techniques and of endoscopic ultrasound, the role of circulating and intracystic markers and the pathologic evaluation for the diagnosis and follow-up of cystic pancreatic neoplasms.

Synchronous association of two neuroendocrine gastroenteropancreatic tumors, an adenocarcinoma of the cecum, and a Meckel's diverticulum: a case report.

Neuroendocrine gastroenteropancreatic tumors constitute a heterogeneous group of neoplasms, with the primary tumors being located in the gastric mucosa, pancreas, and small and large intestine. The development of a second primary malignancy in patients with these tumors is a well-described phenomenon, and the reported incidence ranges from 12% to 46%. The most common site of associated noncarcinoid malignancies is the gastrointestinal tract, which involves from 30% to 60% of the tumors. We report a case of concurrent colon carcinoma and two neuroendocrine tumors of the duodenum.

Italian consensus guidelines for chronic pancreatitis.

This paper gives practical guidelines for diagnosis and treatment of chronic pancreatitis. Statements have been elaborated by working teams of experts, by searching for and analysing the literature, and submitted to a consensus process by using a Delphi modified procedure. The statements report recommendations on clinical and nutritional approach, assessment of pancreatic function, treatment of exocrine pancreatic failure and of secondary diabetes, treatment of pain and prevention of painful relapses. Moreover, the role of endoscopy in approaching pancreatic pain, pancreatic stones, duct narrowing and dilation, and complications was considered. Recommendations for most appropriate use of various imaging techniques and of ultrasound endoscopy are reported. Finally, a group of recommendations are addressed to the surgical treatment, with definition of right indications, timing, most appropriate procedures and techniques in different clinical conditions and targets, and clinical and functional outcomes following surgery.

Tumor M2-pyruvate kinase, a new metabolic marker for pancreatic cancer.

An isoenzyme of pyruvate kinase (Tu M2-PK) is overexpressed by tumor cells and can be measured in blood by a specific immunoenzymatic assay. Our objective was to investigate the diagnostic value of Tu M2-PK in comparison with that of CA 19-9 in pancreatic cancer. We studied 265 subjects: 60 with histologically confirmed pancreatic cancer, 43 with benign pancreatic diseases (acute and chronic pancreatitis), 5 with benign cystic neoplasms of the pancreas, 9 with neuroendocrine tumors, 77 with other abdominal malignancies, 47 with benign digestive diseases, and 24 healthy controls. Levels of plasma Tu M2-PK and serum CA 19-9 were determined by commercially available specific immunoassays. The diagnostic sensitivity and specificity of Tu M2-PK for pancreatic cancer were 85 and 41%, respectively, while those of CA 19-9 were 75 and 81%. The combination of the two tests significantly increased sensitivity (97%) but lowered specificity (38%). In discriminating between pancreatic cancer and acute or chronic pancreatitis, Tu M2-PK turned out to be less accurate than CA 19-9. In patients without pancreatic tumor, cholestasis appeared not to affect the values of Tu M2-PK, while CA 19-9 was found to be significantly higher. Tu M2-PK was also abnormally high in the majority of patients with other digestive malignancies or neuroendocrine tumors. The results demonstrate that Tu M2-PK has a satisfactory sensitivity but a poor specificity in the diagnosis of pancreatic cancer. Used together with CA 19-9, the sensitivity increases considerably.

Effect of secretin on serum pancreatic enzymes and on the Wirsung duct in chronic nonpathological pancreatic hyperenzymemia.

AIM: Chronic nonpathological pancreatic hyperenzymemia (CNPH) is a new syndrome characterized by an increase in serum pancreatic enzymes in the absence of pancreatic disease. The aim of this study was to increase our understanding of this condition by determining the serum pancreatic enzyme response as well as changes in the caliber of the Wirsung duct after secretin stimulation. METHODS: Twenty subjects with CNPH and 9 healthy subjects without CNPH were studied. Blood samples were drawn 5 and 0 min before and 5, 10, 15, 30, 45, and 60 min after intravenous injection of secretin (1 U/kg). Amylase, pancreatic isoamylase, and lipase concentrations were determined. The caliber of the Wirsung duct was measured by ultrasonography. RESULTS: The injection of secretin caused a marked and statistically significant (p < 0.05) increase in serum pancreatic enzymes in the subjects with CNPH that persisted for the duration of the study. The increase over the basal value was in the range of 1.2- to 1.6-fold for amylase, 1.4- to 2.1-fold for pancreatic isoamylase, and 2.6- to 4.2-fold for lipase. In the control subjects the increase was mild, but statistically significant (p < 0.05), ranging from 1.1- to 1.2-fold for amylase, 1.2- to 1.4-fold for pancreatic isoamylase, and 1.5- to 2.2-fold for lipase. The injection of secretin caused a slight increase in the diameter of the Wirsung duct in both groups of subjects, but this was statistically significant only during the first 30 min of the study. CONCLUSIONS: The serum pancreatic enzyme response to secretin was more marked in CNPH than in controls. The Wirsung duct showed no alterations after secretin injection that would help to explain the hyperenzymemia.