Serous cystic neoplasm of the pancreas: A multinational study of 2622 patients under the auspices of the International Association of Pancreatology and European Pancreatic Club (European Study Group on Cystic Tumors of the Pancreas)

OBJECTIVES: Serous cystic neoplasm (SCN) is a cystic neoplasm of the pancreas whose natural history is poorly known. The purpose of the study was to attempt to describe the natural history of SCN, including the specific mortality. DESIGN: Retrospective multinational study including SCN diagnosed between 1990 and 2014. RESULTS: 2622 patients were included. Seventy-four per cent were women, and median age at diagnosis was 58\u2005years (16-99). Patients presented with non-specific abdominal pain (27%), pancreaticobiliary symptoms (9%), diabetes mellitus (5%), other symptoms (4%) and/or were asymptomatic (61%). Fifty-two per cent of patients were operated on during the first year after diagnosis (median size: 40\u2005mm (2-200)), 9% had resection beyond 1\u2005year of follow-up (3\u2005years (1-20), size at diagnosis: 25\u2005mm (4-140)) and 39% had no surgery (3.6\u2005years (1-23), 25.5\u2005mm (1-200)). Surgical indications were (not exclusive) uncertain diagnosis (60%), symptoms (23%), size increase (12%), large size (6%) and adjacent organ compression (5%). In patients followed beyond 1\u2005year (n=1271), size increased in 37% (growth rate: 4\u2005mm/year), was stable in 57% and decreased in 6%. Three serous cystadenocarcinomas were recorded. Postoperative mortality was 0.6% (n=10), and SCN's related mortality was 0.1% (n=1). CONCLUSIONS: After a 3-year follow-up, clinical relevant symptoms occurred in a very small proportion of patients and size slowly increased in less than half. Surgical treatment should be proposed only for diagnosis remaining uncertain after complete workup, significant and related symptoms or exceptionally when exists concern with malignancy. This study supports an initial conservative management in the majority of patients with SCN

A misquoted mutation in exon16 of the BRCA2 gene

A pathogenic mutation in the BRCA2 gene, nt7602del16, has been misquoted as a mutation, possibly due to the incorrect inclusion of the last 16 nucleotides of exon15 of the BRCA2 gene as part of the intron15-exon16 BRCA2 gene sequence in publicly available databases. This was concluded following mutational screening by sequencing and enzymatic mapping of the BRCA2 gene exon15-exon16 region in DNA from peripheral blood samples from a total of 74 breast cancer and non-breast cancer patients as well as healthy individuals and the cell line MCF7. Careful interpretation of genetic variants and direct feedback to the corresponding sequence databases prevent systemic errors by integrating updated data into broadly referenced sources. © The Japan Society of Human Genetics and Springer-Verlag 2006

Laparoscopic or open cholecystectomy in patients with sickle cell disease: which approach is superior?

To compare laparoscopic with open cholecystectomy in patients with sickle cell disease. Retrospective clinical study. University hospital, Greece. 41 patients (22 men and 19 women) with sickle cell disease had laparoscopic cholecystectomy between September 1991 and June 1998. Each patient was matched for age, sex, year of operation, and number of preoperative transfusions with control patients with sickle cell disease who had open cholecystectomy. Duration of operation, postoperative stay in hospital, incidence of complications, and conversion to open operation. The mean operation time was 81.4 min (range 55-125) for open cholecystectomy and 64.2 min (range 45-90) for laparoscopic cholecystectomy (p < 0.01). Complications occurred in 5% (2/41) of the patients in the laparoscopic group and in 20% (8/41) of the patients in the open group (p = 0.04). The mean length of stay in hospital was 5.6 days (range 3-9) in the open group and 2.7 days (range 2-5) in the laparoscopic group (p < 0.01). Conversion to open operation was necessary in 2 (5%) patients. Laparoscopic cholecystectomy resulted in a shorter hospital stay with fewer postoperative complications than open operation in patients with sickle cell disease and may be the procedure of choice in the treatment of cholelithiasis in such patients

Subclinical peritonitis due to perforated sigmoid diverticulitis 14 years after heart-lung transplantation

Acute complicated diverticulitis, particularly with colon perforation, is a rare but serious condition in transplant recipients with high morbidity and mortality. Neither acute diverticulitis nor colon perforation has been reported in young heart-lung grafted patients. A case of subclinical peritonitis due to perforated acute sigmoid diverticulitis 14 years after heart-lung transplantation is reported. A 26-year-old woman, who received heart-lung transplantation 14 years ago, presented with vague abdominal pain. Physical examination was normal. Blood tests revealed leukocytosis. Abdominal X-ray showed air-fluid levels while CT demonstrated peritonitis due to perforated sigmoid diverticulitis. Sigmoidectomy and end-colostomy (Hartmann's procedure) were performed. Histopathology confirmed perforated acute sigmoid diverticulitis. The patient was discharged on the 8th postoperative day after an uneventful postoperative course. This is the first report of acute diverticulitis resulting in colon perforation in a young heart-lung transplanted patient. Clinical presentation, even in peritonitis, may be atypical due to the masking effects of immunosuppression. A high index of suspicion, urgent aggressive diagnostic investigation of even vague abdominal symptoms, adjustment of immunosuppression, broad-spectrum antibiotics, and immediate surgical treatment are critical. Moreover, strategies to reduce the risk of this complication should be implemented. Pretransplantation colon screening, prophylactic pretransplantation sigmoid resection in patients with diverticulosis, and elective surgical intervention in patients with nonoperatively treated acute diverticulitis after transplantation deserve consideration and further studies. © 2008 The WJG Press. All rights reserved

The Mount Sinai experience with orthotopic liver transplantation for benign tumors: Brief report and literature review: Case reports

Orthotopic liver transplantation (OLT) is performed for benign hepatic lesions that are symptomatic, too large to be resected, have a malignant transformation potential, cause debilitating/life-threatening manifestations, or in patients experiencing posthepatectomy acute liver failure. Among benign tumors, polycystic liver disease (PLD) is the most common indication for OLT alone, or combined liver-kidney transplantation. Our 10-year experience with OLT for benign tumors includes two patients with PLD and one with a benign giant fibrous tumor. In this report, we present our experience with OLT for benign liver tumors, commenting on relevant published studies

Breast cancer nodal metastasis correlates with tumour and lymph node methylation profiles of Caveolin-1 and CXCR4

DNA methylation is the best characterised epigenetic change so far. However, its role in breast cancer metastasis has not as yet been elucidated. The aim of this study was to investigate the differences between the methylation profiles characterising primary tumours and their corresponding positive or negative for metastasis lymph nodes (LN) and correlate these with tumour metastatic potential. Methylation signatures of Caveolin-1, CXCR4, RAR-β, Cyclin D2 and Twist gene promoters were studied in 30 breast cancer primary lesions and their corresponding metastasis-free and tumour-infiltrated LN with Methylation-Specific PCR. CXCR4 and Caveolin-1 expression was further studied by immunohistochemistry. Tumours were typified by methylation of RAR-β and hypermethylation of Cyclin-D2 and Twist gene promoters. Tumour patterns were highly conserved in tumour-infiltrated LN. CXCR4 and Caveolin-1 promoter methylation patterns differentiated between node-negative and metastatic tumours. Nodal metastasis was associated with tumour and lymph node profiles of extended methylation of Caveolin-1 and lack of CXCR4 hypermethylation. Immunodetection studies verified CXCR4 and Caveolin-1 hypermethylation as gene silencing mechanism. Absence of Caveolin-1 expression in stromal cells associated with tumour aggressiveness while strong Caveolin-1 expression in tumour cells correlated with decreased 7-year disease-free survival. Methylation-mediated activation of CXCR4 and inactivation of Caveolin-1 was linked with nodal metastasis while intratumoral Caveolin-1 expression heterogeneity correlated with disease progression. This evidence contributes to the better understanding and, thereby, therapeutic management of breast cancer metastasis process. © 2014 Springer Science+Business Media Dordrecht

Apoptotic and proliferative status in HPV (+) and HPV (-) inverted papilloma patients. Correlation with local recurrence and clinicopathological variables

Inverted papilloma (IP) is a rare sinonasal benign lesion characterized by aggressive biological behavior. Our aim was to evaluate the expression of various proliferation and apoptotic markers and the presence of HPV genotypes in paraffin sections gathered from surgically treated IP patients.Immunohistochemistry for PCNA, bax, cytochrome c and caspase-8 and flow cytometry for the detection of apoptosis, necrosis and ki67 expression were performed. The identification of various HPV subtypes was achieved by nested PCR amplification. Nasal polyps (NP) and specimens from normal nasal epithelium (NE) were used as controls.PCNA was more frequently expressed in IP compared to NE (p=0.04) and caspase-8 and bax staining were less frequently observed in IP compared to NP (p=0.004 and p=0.01 respectively) and NE (p=0.003 and p=0.01, respectively). IP and NP presented significantly higher Ki67 flow cytometry values compared to NE (p<0.001 and p=0.02 respectively). Cytochrome c was more frequently expressed in IP specimens with more prominent inflammation (p=0.02). A low HPV DNA detection rate was observed. Neither HPV status nor any of the apoptotic or proliferative markers studied was associated with the patients' clinicopathological characteristics. Increased Ki67 appeared to correlate with disease recurrence (p=0.01).Increased PCNA and Ki67 and decreased bax and caspase-8 expression indicate that cell proliferation is increased while apoptosis is inhibited in IP, explaining its biological behavior. © 2012 Elsevier GmbH