Desmoplastic small round cell tumor (DSRCT) is a very rare but aggressive malignancy. It is usually observed in males during adolescent and early adulthood. The tumor primarily affects the intra-abdominal serosal and is characterized by distinctive histological and immunophenotypic features and by the specific reciprocal translocation EWS-WT1. Prognosis is mainly poor with a mean survival approximately of 2.5 years. However, long-term survivals have been reported using aggressive multimodal therapy based on complete surgical excision, systemic chemotherapy and radiotherapy. The addition of hyperthermic intraperitoneal chemotherapy in the multimodal approach has been reported in very few cases but no effect on survival has been clearly demonstrated. We report a case of a 51-year old adult patient presenting with a DSRCT treated with aggressive therapy based on systemic chemotherapy, complete cytoreductive surgery associated with hyperthermic intraperitoneal chemotherapy, resulting in a long term survival of 4 years.
Objectives. - Tumor necrosis factor alpha (INF-alpha) plays a key role in the immune response. An elevated plasma level of INF-alpha was repeatedly observed in patients with active liver injury or cirrhosis regardless of the aetiology. The G/A transition at position -308 in the promoter region have been shown to influence TNF-alpha expression. In this study, we aimed to evaluate the impact of TNF-alpha -308 G/A functional polymorphism on fibrosis severity in Tunisian Hepatitis C Virus (HCV)-infected patients.Methods. - TNF-alpha -308 G/A polymorphism was evaluated by polymerase chain reaction (PCR) amplification followed by Restriction Fragment Length Polymorphism (RFLP) method in 53 chronic hepatitis C patients Single-nucleotide polymorphism (SNP) frequencies were compared with regard to liver fibrosis severity as assessed by the METAVIR scoring system (F1-F2; n=22 versus F3-F4; n=31).Results. - The genotype distribution of the INF-alpha -308 G/A polymorphism among the HCV-infected patients was as follows : GG : 67.9%, GA: 32.1%, AA: 0%. With regard to fibrosis score, no significant differences in TNF-alpha genotype distribution were observed between F1-F2 and F3-F4 patients (p=0.15)Conclusion. - No significant association between TNF-alpha -308 polymorphism and and the severity of liver fibrosis was found in our Tunisian cohort. (C) 2010 Elsevier Masson SAS. All rights reserved.
In theory, the term of cholangiocarcinoma is reserved for the tumours originating from the intrahepatic bile ducts. The problems of classification of the most frequent hilar tumours and the absence of histopathological confirmation in a large percentage of cases in cancer registries from many countries show the difficulty of establishing the specific epidemiologic behaviour of intrahepatic cholangiocarcinoma (ICC). There are clearly two types of ICC: the first one is the consequence of the recurrent infection of the biliary ducts by the parasites Opisthorchis viverrini and Clonorchis sinensis, and is only seen in the areas of Southeast Asia where liver flukes are endemic. In these areas, incidence and mortality rates of ICC are extremely high. Both parasites have been classified class I carcinogens by the International Agency for Research on Cancer. The other type of ICC is a cancer much rarer but present in the whole world. Some risk factors have been well-established (chronic inflammation of biliary ducts, hepatitis, thorotrast, etc) but many patients do not have any of these factors. An increase in incidence and mortality of this second type of ICC has been seen in recent years, mostly in developed countries. There is an ongoing discussion in the literature about its authenticity and potential causes.
The 18-fluorine-18-fluoro-2-deoxyglucose Positron Emission Tomography coupled with computed tomography is a non invasive exploration. Several studies have shown that PET-CT has superior efficacy over conventional imaging techniques in distinguishing a benign pancreatic tumor from a malignant one. It contributes to the diagnosis of cancer in patients with a doubtful mass, much more in case of chronic pancreatitis. PET-CT is also an important help for the diagnosis of cystic tumors of the pancreas; the results can affect the management strategy. It is interesting for the endocrine tumors, particularly since the emergence of new markers. The aim of this paper is to summarize the role and limitations of 18-F-FDG PET-CT in the management of patients with pancreatic lesions (adenocarcinoma, cystic tumors, endocrine tumors, etc…) concerning the malignancy diagnosis, the detection of metastases, the monitoring after non surgical treatments and to evaluate interpretation difficulties, particularly in case of diabetes or chronic pancreatitis.
La colite collagène est une colite microscopique dont le mécanisme physiopathologique exact est inconnu, différentes hypothèses étiopathogéniques auto-immunes, infectieuses, alimentaires et médicamenteuses ayant été évoquées, cette dernière étant probablement la plus fréquente. La gastrite collagène est une entité rare dont l’association avec une colite collagène a été exceptionnellement rapportée, seuls six cas ayant jusque là été publiés. Nous rapportons une observation de gastrite collagène associée à une iléite et une colite collagène chez une femme de 73 ans présentant une diarrhée chronique et une perte de poids massive évoluant depuis quatre mois. Cet épaississement collagène diffus du tube digestif est apparu dans un contexte auto-immun associant un antécédent de thyroïdite d’Hashimoto et une gastrite atrophique de Biermer probable. L’évolution clinique et histologique a été favorable sous traitement par budésonide.
Gut microbiota contains about 10(14) bacterial cells classified within 4 bacterial phyla, namely Firmicutes, Bacteroidetes, Actinobacteria, and Proteobacteria. Much of the information has been generated through the application of nucleic acid-based methodologies (16S rRNA) which provide a cornerstone of microbial taxonomy. Inflammatory bowel disease (IBD) involves a dysregulated immune response to the gut microbiota in genetically predisposed hosts. Experimental animal models of colitis provide the best evidence that bacteria present in the bowel of the animals have an essential role in the pathogenesis of colitis since in most models, germ-free animals do not develop disease. Moreover, in the immunodeficient mouse model of colitis called TRUC (T-bet-/- x RAG2-/-), a colitogenic gut microbiota is selected and can be transmitted to mice with intact immunity and induce colitis. Current interest therefore focuses on the bacterial community as the source of antigens that fuel the chronic inflammation seen in IBD. Dysbiosis, an imbalance between harmful and protective bacteria, has been evoked and investigated in IBD. Thus, besides the classical pathogens, gut microbiota can drive pathogenicity via two mechanisms: an expansion of 'pro-inflammatory' species or a restriction in the protective compounds of the microbiota. Complexity of the microbiota suggests that both mechanisms may contribute to chronic gut inflammation in IBD.
Two studies show that the risk of drug-induced liver injury (DILI) is increased when the daily dose of a drug given by oral route is higher than 10mg per day and/or when the drug undergoes a significant hepatic metabolism. If confirmed, these data suggest that developing drugs with high potency and low hepatic metabolism will reduce the risk of idiosyncratic DILI in man.
A major challenge in the human metagenomics field is to identify associations of the bacterial genes and human phenotypes and act to modulate microbial populations in order to improve human health and wellbeing. MetaHIT project addresses this ambitious challenge by developing and integrating a number of necessary approaches within the context of the gut microbiome. Among the first results is the establishment of a broad catalog of the human gut microbial genes, which was achieved by an original application of the new generation sequencing technology. The catalog contains 3.3 million non-redundant genes, 150-fold more than the human genome equivalent and includes a large majority of the gut metagenomic sequences determined across three continents, Europe, America and Asia. Its content corresponds to some 1000 bacterial species, which likely represent a large fraction of species associated with humankind intestinal tract. The catalog enables development of the gene profiling approaches aiming to detect associations of bacterial genes and phenotypes. These should lead to the speedy development of diagnostic and prognostic tools and open avenues to reasoned approaches to the modulation of the individual's microbiota in order to optimize health and well-being.
Background. - A pilot program of organized screening for colorectal cancers was conducted in Isere, an administrative district in France. A fecal occult blood test (Hemoccult II (R)) was proposed for all individuals aged greater than 50 years (women since 1991 and men since 2002), followed by colonoscopy for those testing positive. A prospective study was carried out from May to July in 2004 and compared with a similar study conducted in 1996. The goal was to investigate colonoscopy practices, especially the role of screening.Methods. - Gastroenterologists practising in Isere (n = 39/42 practitioners) completed a questionnaire including their patients' age and gender, indications, methods and results for all colonoscopies performed in those aged greater than 20 years. Any tissue samples taken were sent away for histological evaluation.Results. - The study involved 2558 colonoscopies (54% female, 73% patients aged greater or equal to 50 years), an increase of 35% from 1996 to 2004. Of the patients referred, 50.0% were symptomatic (pain; bowel problems: 28.7%; rectal bleeding: 21.3%), 23.5% had colonic disease and 22.5% came from screening (3.1% had positive stool tests, 17.8% had a family history). Recommendations related to family history (update of the 1998 consensus conference: screening indicated for patients with a first-degree relative diagnosed with cancer or advanced polyps aged less than 60 years) were well applied in terms of relatedness (81%) but, in 52% of cases, the age was greater than 60 years. Colonoscopy was carried out in almost all cases (0.1% failure), with complications in 0.4% of the examinations. Of the 2558 colonoscopies performed, 10% revealed advanced polyps or cancer: 30% were following a positive test compared with 8% for symptoms and 6% with a family history. Multivariate analyses showed that polyps greater or equal to 10 mm or malignant tumors are 1.5 times more common in men than in women, and six times more frequently seen in patients having colonoscopy following a positive test for blood in stools than in those with a family history of colorectal cancer. The number of pathologies found increased significantly in those aged greater than 50 years.Conclusion. - This cross-sectional survey of colonoscopy practices in Isere shows an increase in the number of colonoscopies performed between 1996 and 2004. This increase is not explained by expansion of the screening program, which was the reason for only 3% of colonoscopies. However, the best diagnostic yield for advanced polyps or cancers was obtained in screened patients (30%). (C) 2010 Elsevier Masson SAS. All rights reserved.
Intramural esophageal dissection is a rare disorder characterized by extensive laceration between the mucosal and submucosal layers of the esophageal wall, but without perforation. The etiology of intramural dissection of the esophagus remains uncertain. Conservative management is usually considered adequate. Only one case of circumferential intramural esophageal dissection has been reported previously. We report here on a case due to an infectious cause (paryngeal abscess) that is also an unusual example of circumferential intramural esophageal dissection, which was then treated by endoscopic transection of the true internal esophageal wall and bougienage dilation.
Incretin-based therapies have shown promise in the treatment of type 2 diabetes. Here we review our current understanding of TGR5 as a target to induce glucagon-like peptide-1 (GLP-1) secretion. These new observations suggest that TGR5 agonists may constitute a novel approach to treat type 2 diabetes, as well as complications of diabetes, such as non-alcoholic fatty liver disease.
D’un point de vue physiopathologique, le syndrome de l’intestin irritable (SII) est une affection multifactorielle. Cependant, l’accent est mis actuellement sur le rôle probablement important joué par les anomalies de la flore, en particulier dans la sensibilisation des terminaisons sensitives et l’activation des cellules immunitaires dans la paroi digestive. Epidémiologiquement, un SII, surtout à forme diarrhéique, peut apparaitre au décours d’une gastroentérite aiguë, essentiellement d’origine bactérienne. L’action métabolique de la flore parait excessive, pouvant contribuer à certain symptômes comme le ballonnement et peut-être favoriser la survenue d’une hypersensibilité viscérale. Des anomalies qualitatives de la flore à la fois endoluminale et adhérente au biofilm présent au contact de l’épithélium ont été décrites. Enfin, une pullulation bactérienne endo-luminale parait être une réalité dans un sous groupe de patients souffrant de SII, sans que l’on sache actuellement si cette anomalie est primitive ou secondaire aux troubles moteurs, notamment grêliques, qui s’observent au cours du SII. Ce rôle délétère de la flore ouvre des perspectives thérapeutiques avec un recours possible aux pré- ou probiotiques et peut-être aux antibiotiques.
