The natural history of hereditary pancreatitis: a national series.

Background And Aims: The prevalence and natural history of hereditary pancreatitis (HP) remain poorly documented. The aims of this study were to assess genetic, epidemiological, clinical and morphological characteristics of HP in an extensive national survey.

Methods: A cohort comprising all HP patients was constituted by contacting all gastroenterologists and paediatricians (response rate 84%) and genetics laboratories (response rate 100%) in France (60,200,000 inhabitants).

Thirteen-month registration of patients with gastroenteropancreatic endocrine tumours in France.

The prevalence, clinical profiles and management of gastroenteropancreatic endocrine tumours (GEP) in France are not known. From August 1, 2001 to September 1, 2002, standardized records on patients with GEP were prospectively completed in 87 participating centres. The total group amounted to 668 patients (median age: 56 years, range: 12-89).

[Pancreatic perivascular epithelioid cell tumor (PEComa)].

Neoplasms with perivascular épithelioid-cell differentiation (PEComas) are rare tumors with a distinctive immunoreactivity for melanocytic markers. They have been described in various organs. We report an intrapancreatic PEComa discovered in a 46-year-old woman during a workup for diarrhea.

Nonsteroidal anti-inflammatory drug use as a risk factor for gastro-oesophageal reflux disease: an observational study.

Background: Although the associations between nonsteroidal anti-inflammatory drugs (NSAIDs) and peptic ulcer disease or dyspepsia are well established, fewer data exist concerning the relationship between NSAIDs and gastro-oesophageal reflux disease (GERD).

Aim: To examine the relationship between NSAIDs and GERD.

Methods: A self-administered questionnaire covering NSAID use and GERD symptoms (heartburn and acid regurgitation) was sent to a representative national sample of 10,000 French adults (> or = 18 years) between 14 October and 21 November 2005.

Endocrine pancreatic tumors in von Hippel-Lindau disease: clinical, histological, and genetic features.

Objectives: Endocrine pancreatic tumors (EPTs) in von Hippel-Lindau (VHL) disease pose difficult management problems. We aimed to assess (1) the accuracy of somatostatin receptor scintigraphy, (2) histological features with focus on malignancy and genotype-phenotype correlations, and (3) prognosis of VHL-EPT.

Methods: Thirty-five patients with EPT-VHL (20 women; median age, 37 years) from 29 families were studied.

Acute pancreatitis in a cohort of 129 patients referred for severe hypertriglyceridemia.

Objectives: The aim of this study was to assess retrospectively the prevalence and the predictive factors of acute pancreatitis (AP) in a population of patients referred in our endocrinology department for evaluation of very high triglyceride (TG) levels.

Methods: One hundred twenty-nine patients (119 with type IV phenotypes and 10 with type V phenotypes according to Fredrickson's classification) were referred to our hospital between 2000 and 2005.

Results: Twenty-six subjects (20.

Cystic dystrophy in gastric heterotopic pancreas complicated by intracystic hemorrhage and fistulisation in the stomach - a pediatric case.

Context: Cystic dystrophy of the digestive wall, a rare but well-known complication of heterotopic pancreas when it is located in the duodenum, has been mainly described in adult series. Cystic dystrophy of the heterotopic pancreas within the gastric wall has been reported in only six adult cases. To our knowledge, no pediatric case has been described.

Sporadic desmoid tumor. An exceptional cause of cystic pancreatic lesion.

Context: Desmoid tumors are rare, benign soft tissue tumors, characterized by the proliferation of fibroblasts in an abundant collagen extra-cellular matrix. Intra-abdominal forms involve the mesentery and retroperitoneum and usually occur associated with familial adenomatous polyposis or Gardner's syndrome. Sporadic cases are more uncommon.

Massive postoperative ascites following pancreatic cysts fenestration in a patient with von Hippel-Lindau disease.

Pancreatic lesions in von Hippel Lindau disease (VHLD) are frequent and mainly consist of cystic lesions, which should not be resected because of their benign evolution. Solid lesions, mostly pancreatic endocrine tumors (PET), are rare and usually occur in combination with cystic lesions. We report a case of a patient with VHLD who underwent PET enucleation in a polycystic pancreas requiring fenestration of multiple adjacent cysts, to ensure complete resection with free resection margins.

Predictors of long-term survival after liver transplantation for metastatic endocrine tumors: an 85-case French multicentric report.

Liver transplantation (LTx) for metastatic endocrine tumors (MET) remains controversial due to the lack of clear selection criteria. From 1989 to 2005, 85 patients underwent LTx for MET. The primary tumor was located in the pancreas or duodenum in 40 cases, digestive tract in 26 and bronchial tree in five.

[Verner-Morrison syndrome revealing a ganglioneuroblastoma in an adult].

Most vasoactive intestinal peptide (VIP)-producing tumours are from epithelial origin. Tumours derived from the sympathetic nervous system can produce VIP as well. We report here the case of a Verner-Morrison syndrome in a 40-year-old woman revealing a metastatic ganglioneuroblastoma.

Two-step surgery for synchronous bilobar liver metastases from digestive endocrine tumors: a safe approach for radical resection.

Objective: We describe the early and distant results of a 2-step surgical strategy that enables complete resection in selected patients with primary digestive endocrine tumors (DET) and synchronous bilobar liver metastases (LM).

Background: Frequent synchronous and bilobar liver involvement limits indications of surgery in LM from DET.

Study Design: From 1996 to 2004, of 41 patients with synchronous bilobar LM from DET, 23 (56%) were selected for 2-step surgery.

Morphologic changes in branch duct intraductal papillary mucinous neoplasms of the pancreas: a midterm follow-up study.

Background & Aims: Because there is a low risk of malignancy for intraductal papillary and mucinous neoplasms of the pancreas (IPMNs) confined to branch ducts (BD), patient follow-up evaluation without surgery is possible. The aim of this study was to assess time-related morphologic changes and risk of progress to malignancy in patients with BD IPMN. A prospective design was used in an academic tertiary referral center.

[Cholangiocarcinoma: epidemiology and global management].

Scope: Cholangiocarcinoma, or biliary tract tumors, are rare tumors for which survival is short, as diagnosis is often made at an advanced stage. Indeed, diagnosis remains difficult, since symptoms are often unspecific and appear at latest stages. This article presents an update of recent data and therapeutic options.

Risk of pancreatic adenocarcinoma in patients with hereditary pancreatitis: a national exhaustive series.

Background And Aims: An increased risk of pancreatic adenocarcinoma (PA) in patients with hereditary pancreatitis (HP) was previously demonstrated in two multinational studies. The PA frequency in this setting is however unknown due to lack of exhaustive case collection. The aims of this study were to evaluate the standardized incidence ratio (SIR) of PA in an exhaustive national series of patients with HP and to search for risk factors.

Gastroenteropancreatic neuroendocrine tumours.

Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects.

[Severe thrombopenia as single sign of hemophagocytosis in a patient with cirrhosis and lethal infection of ascitis fluid by Escherichia coli].

Hemophagocytosis is defined by a systemic macrophages activation, phagocyting the blood elements. This syndrome can be primary or secondary to multiple causes such as neoplasia or infections. We report here the first case of a hemophagocytosis caused by an Escherichia coli infection of ascitis fluid.

Fatal bile duct necrosis: a rare complication of transcatheter arterial chemoembolization in a patient with endocrine hepatic metastasis.

We report the first case of fatal bile duct necrosis following transcatheter arterial chemoembolization (TACE) in a 58-year-old woman. The patient underwent two TACEs to treat hepatic metastases from an ileal endocrine tumor. Persistent cholestasis occurred after the second procedure, leading to the diagnosis of bile duct necrosis confirmed by liver biopsy.

[PET and digestive cancers].

In digestive oncology, the most frequent indication for FDG PET, in our experience and as reported in the literature, is the localisation of recurrent colorectal cancer. This molecular imaging method has also been shown to be clinically useful in various other settings, especially for preoperative staging, for colorectal, esophageal, gastric, pancreatic, hepatic and biliary cancers. We also report on current PET practice in two particular cancers: hepatocellular carcinoma, for which other tracers, including fluoromethylcholine-(18F), are being currently evaluated, and gastrointestinal endocrine tumours, which are included in the recent French marketing authorisation of fluoroDOPA-(18F) and which are also potential targets for radiolabelled somatostatin analogues for PET imaging.

Diagnosis and management of pancreatic fistulae resulting in pancreatic ascites or pleural effusions in the era of helical CT and magnetic resonance imaging.

Objectives: Diagnosis of internal pancreatic fistulae (IPF) resulting in ascites or pleural effusions may be facilitated by multislice helical CT-scan and MR-pancreatography [MRP]). Conservative treatment with parenteral nutrition and somatostatin analogues (+/- pancreatic stenting) yields varying results. We aimed to evaluate the usefulness of helical CT and MRP in the diagnosis of IPF.

Malignant intraductal papillary mucinous neoplasm of the pancreas: in situ versus invasive carcinoma surgical resectability.

Purpose: To retrospectively evaluate computed tomographic (CT) findings in patients with in situ and invasive malignant intraductal papillary mucinous neoplasms (IPMNs) of the pancreas and to evaluate the accuracy for surgical resectability, with surgery and pathologic analysis as the reference standards.

Materials And Methods: Institutional review board approval was obtained, and the informed consent requirement was waived. Forty-six patients with malignant IPMN proved at pathologic examination of the surgically resected specimen (n = 44) or laparotomy (n = 2) underwent surgery after multidetector CT was performed.

Effects of esomeprazole on acid output in patients with Zollinger-Ellison syndrome or idiopathic gastric acid hypersecretion.

Objectives: To evaluate the efficacy and safety of oral esomeprazole in the control of gastric acid hypersecretion in patients with hypersecretory states.

Methods: In this 12-month, open-label, multicenter study, acid output (AO) was evaluated at baseline, day 10, and months 3, 6, and 12. The starting dose of esomeprazole was 40 mg or 80 mg twice daily.

Co-inheritance of a novel deletion of the entire SPINK1 gene with a CFTR missense mutation (L997F) in a family with chronic pancreatitis.

Quantitative fluorescent multiplex PCR (QFM-PCR) was established in order to make possible the rapid and efficient mutational analysis of the pancreatic secretory trypsin inhibitor (SPINK1) gene. Using QFM-PCR, a novel heterozygous deletion encompassing the entire SPINK1 gene was identified in one of nine newly recruited French Caucasian families with chronic pancreatitis. The breakpoints were fully characterized and the approximately 30 kb deletion was termed c.

[Suspected malignant cancer of the pancreas associated with pseudo-invasive duodenal hamartomas in a patient with Peutz-Jeghers syndrome].

A 29-year-old man with a previously known Peutz-Jeghers syndrome (PJS) was admitted for epigastric pain, emesis and weight loss due to both intestinal intussusception causing bowel obstruction and obstructive pancreatitis. The patient had cholestasis with an enlarged common bile duct on imaging. Because duodenal and/or pancreatic cancer was suspected due to weight loss, the pancreatic and bile duct obstruction, and the increased risk of small intestine and pancreatic adenocarcinoma in patients with PSJ, a pancreatoduodenectomy was performed.