Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) - A Helicobacter-opposite point.

Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is a rare familial gastric cancer syndrome with an autosomal dominant pattern of inheritance. It is characterised by fundic gland polyposis of the gastric body and is associated with a significant risk of gastric adenocarcinoma. Unlike sporadic gastric cancer, Helicobacter pylori is usually absent in patients with GAPPS. This opposite-point finding has so far not been fully clarified. Prophylactic total gastrectomy is indicated in all cases of GAPPS with fundic gland polyposis and the presence of any dysplasia. If no dysplasia is found at histology, prophylactic gastrectomy is suggested at between 30 and 35 years of age, or at five years earlier than the age at which the youngest family member developed gastric cancer. Different phenotypes of GAPPS demand an individual approach to particular family members.

Pancreatic Cysts: Diagnostic Role of EUS-Guided Microforceps Biopsy and Confocal Laser Endomicroscopy.

Frequent use of high-quality cross-sectional imaging has led to a significant rise in diagnosis of pancreatic cystic lesions (PCLs). Despite the fact that enormous effort has been put into the research of PCLs within the last two decades and multiple guidelines have been developed, our clinical decision-making especially in regard to mucinous lesions remains limited. Currently, clinical assessment, cross-sectional imaging and EUS with fluid analysis (if appropriate) belong to the standard care in patients with PCLs. For differentiation of mucinous from nonmucinous cysts, the sensitivity of cytological investigation and CEA in the cyst fluid is 42% and 52-79%, respectively. Due to the limited accuracy, further diagnostic tools are warranted. Two EUS-guided approaches have been introduced recently. Through-the-(19-gauge EUS) needle Moray microforceps have been developed, and several studies have acknowledged their contribution to the correct diagnosis as they help to overcome limited cellularity of the EUS-guided cyst fluid aspiration and traditional cytology. Confocal laser endomicroscopy offers real-time images and seems to be a promising method for the diagnosis and differential diagnosis of pancreatic PCLs. Example images of the needle-based confocal laser endomicroscopy criteria for the diagnosis of PCLs have been suggested recently. Before both, Moray microforceps and confocal laser endomicroscopy can be widely accepted, further studies are necessary to determine the real diagnostic yield and the clinical efficacy.

Investigation of 23 Bile Acids in Liver Bile in Benign and Malignant Biliary Stenosis: A Pilot Study.

Differential diagnosis between benign and malignant biliary stenosis can be difficult in clinical practice. Histology of biopsy specimens is often indeterminate. Laboratory markers (serum bilirubin > 75 µmol/L, carbohydrate antigen 19-9 > 400 U/mL) and the length of stenosis (>15 mm) can be helpful but are not specific enough. The aim of this study was to investigate bile acids in liver bile of patients with benign and malignant biliary stenosis and controls without stenosis. A total of 73 patients entered the study: 7 subjects with benign biliary stenosis (6 men, 1 woman; 68 ± 13 years old), 21 with malignant biliary stenosis (15 men, 6 women; 72 ± 14 years old), and 45 patients without biliary stenosis (22 men, 23 women; 70 ± 13 years old); out of those, 25 subjects have and 20 do not have choledocholithiasis. Twenty-three different bile acids were investigated by high-performance liquid chromatography/mass spectrometry. Serum total bilirubin was significantly higher in patients with malignant biliary stenosis compared with nonstenotic controls (p = 0.005). Significant relationship (r > 0.7) was found between several pairs of bile acids. Significantly lower bile acid concentrations in malignant biliary stenosis compared to controls without stenosis were found for GLCA (p = 0.032), GUDCA (p = 0.032), GCDCA (p = 0.006), GDCA (p = 0.031), GHCA (p = 0.005), TUDCA (p = 0.044), and TDCA (p = 0.036). Significant difference in cholic acid was found between benign and malignant stenosis (p = 0.022). Analysis of bile acids might be helpful in the differential diagnosis of malignant and benign biliary stenosis. More patients need to be enrolled in further studies so that the real diagnostic yield of bile acids can be determined.

Histopathologic Features are more Important Prognostic Factors than Primary Tumour Location in Gastro-oesophageal Adenocarcinoma Treated with Preoperative Chemoradiation and Surgery.

The aim of present study was to evaluate the impact of primary tumour location and other factors on the outcome of preoperative chemoradiation followed by surgery in adenocarcinomas of distal oesophagus, gastro-oesophageal junction and stomach. We retrospectively reviewed the institutional patient database. The therapeutic response was re-evaluated as a percentage of residual tumor cells in surgical resection specimens. Overall survival (OS) and disease-free survival (DFS) were assessed. The effect primary tumour location, clinical and pathological TNM stage, and histopathological factors (histological type, grade, angioinvasion, perineural invasion, tumour response) on treatment outcome were evaluated. A total of 108 patients underwent preoperative chemoradiation for adenocarcinoma of distal oesophagus, gastro-oesophageal junction or stomach. The median prescribed dose of radiation was 45 Gy. The concurrent chemotherapy consisted of 5-fluorouracil +/- cisplatin +/- taxanes. R0 resection was achieved in 80 patients (74%). The complete response was observed in 19%. The median follow-up was 50.8 months. Three-year and 5-year OS and DFS were 36.2% and 25.3%; and 28.1% and 23.7%, respectively. Pretreatment T-stage, pathological N-stage, radicality of resection, histological subtype, grade, angioinvasion and perineural invasion, were identified as statistical significant OS predictors in univariate analysis; pathological N-stage, radicality of resection and angioinvasion, in multivariate analysis. The primary tumor location did not influence the prognosis. The pathologic response to chemoradiation had borderline significance. In conclusion, no prognostic impact of primary tumour location, in contrast to other investigated factors, was evident in the present study. The most important predictors of prognosis were angioinvasion status and pN-stage.

The first European family with gastric adenocarcinoma and proximal polyposis of the stomach: case report and review of the literature.

BACKGROUND AND AIMS: Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) has to date been recognized in only 8 families worldwide. Recently, different point mutations within the Ying Yang 1 (YY1) binding motif in promoter 1B of the APC gene were assigned as causal in 6 families with GAPPS. METHODS: We diagnosed GAPPS across 3 generations in a Czech white family. RESULTS: The proband's mother died of gastric cancer at 49 years of age. The proband died of gastric cancer at 56 years of age. All 3 of the proband's daughters inherited polyposis, involving exclusively the gastric fundus and body, with relative sparing of the lesser curve. The daughters have all been regularly surveyed endoscopically. Polyposis progressed rapidly with intestinal differentiated low-grade and high-grade dysplasia present on polypectomy specimens 5 years after the original diagnosis. On this basis, all 3 of the proband's daughters were scheduled for prophylactic total gastrectomy. Unfortunately, the middle daughter presented with generalized gastric adenocarcinoma and died at the age of 26 years. The other 2 daughters (aged 30 and 23 years) underwent total gastrectomy within 6 weeks of their sister's death; histology of surgical specimens showed gastric adenocarcinoma stage IA (pT1a, N0, M0) in both cases. Bi-directional Sanger sequencing of promoter 1B revealed a point mutation (c.-191 T>C) in all 3 daughters of the proband. CONCLUSIONS: Atypical endoscopic progression of the fundic gland polyposis, with the presence of dysplasia on polypectomy specimens and genetic testing with recently discovered mutations in promoter 1B of the APC gene might help clinicians to decide whether prophylactic gastrectomy should be performed.

Cryptogenic multifocal ulcerous stenosing enteritis: a review of the literature.

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is an extremely rare illness characterised by chronic or relapsing subileus status resulting from multiple small intestinal fibrous strictures and multiple shallow ulcers of the small bowel. The etiology is unknown and pathogenesis is not fully understood. Therapy with systemic glucocorticosteroids is the treatment of choice. However, most patients develop corticosteroid dependence. Deep enteroscopy enables precise diagnostic work, possible endoscopic treatment of stenoses; may obviate the need for surgery and prevent excessive small bowel resections.

Pancreatic Castleman disease treated with laparoscopic distal pancreatectomy.

BACKGROUND: Castleman disease is an uncommon lymphoproliferative disorder most frequently occurring in the mediastinum. Abdominal forms are less frequent, with pancreatic localization of the disease in particular being extremely rare. Only seventeen cases have been described in the world literature. METHOD: This report describes an interesting and unusual case of pancreatic Castleman disease treated with laparoscopic resection. RESULTS: A 48-year-old woman presented with epigastric pain. CT scan showed a well-encapsulated mass on the ventral border of the pancreas. Endosonography with fine needle aspiration biopsy was performed. Biopsy showed lymphoid elements and structures of a normal lymph node. The patient was treated with laparoscopic distal pancreatectomy. The pancreas was transected with a Ligasure device and the pancreatic stump was secured with a manual suture. One year after surgery the patient was complaint-free and showed no signs of recurrence of the disease. CONCLUSIONS: Laparoscopic distal pancreatectomy is a feasible and safe method for the treatment of lesions in the body and tail of the pancreas. Transection of the pancreas with a Ligasure device offers the advantages of low bleeding and low risk of pancreatic fistula.

Severe cryptogenic multifocal ulcerous stenosing enteritis. A report of three cases and review of the literature.

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare condition characterised by chronic or relapsing moderate ileous episodes resulting from multiple small intestinal strictures, multiple shallow ulcers of the small bowel and favourable therapeutical effect of glucocorticosteroids. The aim of this paper was to evaluate three cases of CMUSE diagnosed within 10 years at a tertiary gastroenterology centre. Three females (35, 50, 60 years) were presented with colicky pain, repeated moderate ileous episodes and weight loss. Multiple fibrous strictures and ulcers of the small bowel were found. All three patients responded to glucocorticosteroid treatment. Tandem tight jejunal stenoses were dilated endoscopically by means of double balloon enteroscopy. In conclusion, CMUSE should always be considered when chronic moderate ileous episodes and multiple small intestinal strictures and ulcers of uncertain aetiology are found. Double balloon enteroscopy enables precise diagnostic work, possible endoscopic treatment of stenoses, may obviate the need for surgery and prevent excessive small bowel resections.

Endoscopic ultrasonography (EUS) and EUS-guided fine-needle aspiration with cyst fluid analysis in pancreatic cystic neoplasms.

BACKGROUND/AIMS: Pancreatic cystic neoplasms represent a heterogeneous group of tumors with varied malignant potential. The aim of this prospective study was to evaluate EUS, EUS-FNA and cyst fluid analysis in distinguishing serous cystadenomas (SCA) and mucinous cystic neoplasms (MCN). METHODOLOGY: Twelve patients with SCA (4 men, 8 women, mean age 58), 16 with MCN (3 men, 13 women, mean age 53) and 10 pancreatic non-tumorous cysts as controls (1 man, 9 women, mean age 43) were investigated by EUS-FNA from January 2003 to February 2006. Cyst fluid evaluation was done for cytology, amylase, CEA, CA 19-9, CA 72-4 and CA 15-3 (RIA). The final diagnosis was based on surgery & histology (14 patients) and/or follow-up after EUS-FNA (mean 15 months). RESULTS: In the MCN-group 13 mucinous cystadenomas, 2 cystadenocarcinomas and 1 malignant IPMT were found. EUS-FNA results: cytology (including staining for mucin) was diagnostic in 2/12 SCA (17%), 10/16 MCN (63%) and negative in all controls. Fluid CEA in MCN-group (mean 9487, 95%CI 0-23637) was significantly higher compared both with SCA-group (mean 22, 95%CI 0-54, p<0.001) and controls (mean 4, 95%CI 0.5-8, p<0.001). Similar results were found in fluid CA72-4 and fluid CA19-9. Accuracy of EUS-FNA with final diagnosis was 93%. CONCLUSIONS: EUS-FNA with cyst fluid CEA, CA72-4, CA19-9 and cytology are useful tools in differentiating SCA, MCN and non-tumorous cysts.