Bile duct obstruction caused by an eosinophilic papillary pseudotumor.
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Adult↗
Biomedical subjects
Philippe Ruszniewski
Publications and source records attributed to Philippe Ruszniewski.
[Natural history and prognosis of pancreatic endocrine tumors].
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Biomarkers, Tumor↗
[Frequency of CFTR gene mutations in idiopathic pancreatitis].
UNLABELLED: The prevalence of mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene in idiopathic chronic pancreatitis has been shown to be increased. The aim of this study was to prospectively analyze the frequency of such mutations in a homogeneous group of patients with idiopathic pancreatitis studied in a French Gastroenterology department. PATIENTS AND METHODS: Between April 1999 and December 2001, all patients with unexplained chronic or recurrent acute pancreatitis were studied. Other causes of pancreatitis were excluded and no patient had personal signs or family history compatible with cystic fibrosis. Following informed written consent, genetic analysis for CFTR was performed using an oligonucleotid ligation assay, on the 31 most frequently known mutations of the CFTR gene. A complementary analysis for variants in exons 9, 10 and 17a, thought to be implicated in atypical cystic fibrosis, was also performed using denaturing gradient gel electrophoresis. RESULTS: Idiopathic pancreatitis occurred in 64 patients (chronic, n=30; recurrent acute, n=34) with a median age of 36 years. Eighteen CFTR mutations or variants were detected in 16 patients (25%): DeltaF508 (n=7), L997F (n=2), E528E (n=4), 5T (n=5). Two patients were compound heterozygous. The frequency of DeltaF508 mutations was greater than that of the general population (10.9 vs 2.4%; P<0.001). Pancreatitis was diagnosed at a median age of 32 years in mutation-positive patients compared to 39 in mutation-negative patients. The prevalence of CFTR mutations was 35.5% in patients < or =35 years against 15.1% in those > 35 years (P=0.06). The clinical course of pancreatitis (severity and complication rates) was not altered by the presence of a mutation. CONCLUSION: One-quarter of all patients and one-third of those < or =35 years with idiopathic pancreatitis have at least one mutation of the CFTR gene. The presence of a CFTR mutation appears to predict the development of pancreatitis at an earlier age.
Acute Disease↗
[Are the causes similar for benign and severe forms of acute pancreatitis?].
UNLABELLED: The frequency of severe acute pancreatitis not due to alcohol or biliary causes is not well known. AIMS: To evaluate the distribution of causes responsible for benign and severe cases of acute pancreatitis in an effort to identify causes to search for in patients with severe acute pancreatitis. PATIENT: All patients hospitalized for acute pancreatitis between January 1994 and May 2001 with a good quality CT scan. METHODS: All patients had a complete, standardized evaluation to look for all possible causes of acute pancreatitis. The following severity criteria were retrospectively reviewed: maximal C-reactive protein level, Ranson's score, Balthazar's score, percentage of patients hospitalized in intensive care unit or a high-dependency unit, hospitalization duration, and local or general complications. RESULTS: One hundred thirty-nine patients were included. The cause of acute pancreatitis were: alcohol (34%), biliary (27%), obstructive (16%), miscellaneous (10%), unknown (9%), post endoscopic retrograde cholangio-pancreatography (4%). The studied severity factors did not differ with respect to the cause of acute pancreatitis with the exception of Balthazar's score. Non-alcoholic non-biliary causes were found in 19 (27%) of the 71 patients with severe necrotic acute pancreatitis (Balthazar > or =D) and 35 (51%) of the 68 patients with acute pancreatitis with Balthazar score or =D). For the other severity scores, the distribution of causes was similar. After exclusion of biliary and alcoholic causes, a careful search for other etiologies should be carried out in both benign and severe cases of acute pancreatitis.
Acute Disease↗
[Weber Christian syndrome due to pancreaticomesenteric fistula secondary to acute alcoholic pancreatitis: treatment with pancreatic sphincterotomy alone].
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Humans↗
[Treatment of pancreatic-duodenal endocrine tumors].
Control of symptoms due to hormonal secretion is an integral first-step in the management of patients with functional tumours of the pancreatico-duodenal region. Symptomatic drugs should be employed, as well as systemic chemotherapy for nonresectable tumours on the basis of histological differentiation and tumour evolution. Hepatic chemoembolization yields objective response rate of 50%. Surgery however remains the only curative option. Well-differentiated and resectable tumours should be treated by resection, associated with hepatectomy in cases of resectable metastases. Liver transplantation should be reserved for patients with well-differentiated slowly-progressive tumours demonstrating multiple hepatic metastases with a resectable primary in the absence of other metastatic disease. When medical options are inefficacious in the control of hormonal symptoms, cytoreductive surgery can be an effective option.
Chemoembolization, Therapeutic↗
[Chronic pancreatitis].
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Chronic Disease↗
[Clinical spectrum of digestive neuroendocrine tumors].
Digestive neuro-endocrine tumours have a broad and initially misleading clinical spectrum. Tumours from the duodenopancreatic area should be distinguished from digestive carcinoid tumours. In the first group, insulinomas, gastrinomas, and non-functioning tumours are the most frequent. Insulinoma is responsible for hypoglycaemic symptoms (coma, confusion, seizure, psychiatric disorders) associated with adrenergic response (sweat, tachycardia, palpitations). Gastrinoma is responsible for the Zollinger-Ellison syndrome, which associates peptic ulcers in the oesophagus, stomach, and duodenum without Helicobacter pylori infection, and chronic volumogenic diarrhoea. Non-functioning tumours are recognised fortuitously or at a late stage, when large tumour mass contrasts with often unaltered general condition. Carcinoid tumours are mainly located in the appendix, the rectum, and the small bowel. In the 2 first conditions, the diagnosis is most often made on a resection specimen after uneventful appendectomy or polypectomy; in the latter, the carcinoid syndrome is frequent, combining cutaneous flushing, motor diarrhoea, tricuspid valve insufficiency and bronchospasm.
Diagnosis, Differential↗
Mucin gene expression in intraductal papillary-mucinous pancreatic tumours and related lesions.
Intraductal papillary-mucinous tumours (IPMTs) of the pancreas are heterogeneous proliferations characterized by a malignant potential. The molecular mechanisms underlying the tumourigenesis process are not well understood. Recently, it has been shown that IPMTs secreting the mucin antigen MUC2 have a better prognosis, but the complete pattern of MUC gene expression has not yet been established. The aims of this study were to evaluate the mucin gene expression in 57 IPMTs and eight related lesions surgically resected and to relate MUC gene expression to the histological diagnosis. In situ hybridization (ISH) was performed in 28 cases with probes specific for the MUC1, MUC2, MUC3, MUC4, MUC5AC, MUC5B, MUC6, and MUC7 genes. An immunohistochemical analysis was carried in all 65 cases and in 90 conventional ductal adenocarcinomas of the pancreas using MUC1, MUC2, and MUC5AC antibodies. IPMTs of adenoma (dysplasia) type exhibited high expression of MUC2 (93%), MUC5AC (97%), and, to a lesser extent, of MUC4 (71%), all of which were also observed in colloid carcinomas associated with IPMTs. In contrast, IPMTs with simple hyperplasia, intraductal oncocytic papillary neoplasms, and pyloric glandular adenomas exhibited little or no expression of MUC2. The mucin expression profile supports the existence of two types of invasive tumour associated with IPMTs: a colloid and an ordinary form. The latter shows a pattern similar to the conventional ductal adenocarcinomas with a loss of MUC2 and a gain of MUC1 and has a greater tendency to metastasize. In conclusion, the altered expression of mucin, characteristic of IPMT of adenoma type and of colloid carcinomas, may contribute to the better clinical outcome of these neoplasms, compared to conventional pancreatic ductal adenocarcinomas.
Adenocarcinoma, Mucinous↗
Characterization of gene expression profiles in intraductal papillary-mucinous tumors of the pancreas.
The molecular pathology of precursor lesions leading to invasive pancreatic ductal adenocarcinomas remains relatively unknown. We have applied cDNA microarray analysis to characterize gene expression profiles in a series of intraductal papillary-mucinous tumors (IPMTs) of the pancreas, which represents one of the alternative routes of intraepithelial progression to full malignancy in the pancreatic duct system. Using a cDNA microarray containing 4992 human genes, we screened a total of 13 IPMTs including nine noninvasive and four invasive cases. Expression change in more than half of the tumors was observed for 120 genes, ie, 62 up-regulated and 58 down-regulated genes. Some of the up-regulated genes in this study have been previously described in classical pancreatic carcinomas such as lipocalin 2, galectin 3, claudin 4, and cathepsin E. The most highly up-regulated genes in IPMTs corresponded to three members of the trefoil factor family (TFF1, TFF2, and TFF3). Immunohistochemistry performed on five genes found to be differentially expressed at the RNA level (TFF1, TFF2, TFF3, lipocalin 2, and galectin 3) showed a good concordance between transcript level and protein abundance, except for TFF2. Hierarchical clustering organized the cases according to the dysplastic and invasive phenotype of theIPMTs. This analysis has permitted us to implicate several genes (caveolin 1, glypican 1, growth arrest-specific 6 protein, cysteine-rich angiogenic inducer 61) in tumor progression. The observation that several genes are differentially expressed both in IPMTs and pancreatic carcinomas suggests that they may be involved at an early stage of pancreatic carcinogenesis.
Adenocarcinoma, Mucinous↗
Determination of the relative contribution of three genes-the cystic fibrosis transmembrane conductance regulator gene, the cationic trypsinogen gene, and the pancreatic secretory trypsin inhibitor gene-to the etiology of idiopathic chronic pancreatitis.
In the last 5 years, mutations in three genes, the cystic fibrosis transmembrane conductance regulator (CFTR) gene, the cationic trypsinogen (PRSS1) gene, and the pancreatic secretory trypsin inhibitor (PSTI) gene, have been found to be associated with chronic pancreatitis (CP). In this study, using established mutation screening methods, we systematically analysed the entire coding sequences and all exon/intron junctions of the three genes in 39 patients with idiopathic CP (ICP), with a view to evaluating the relative contribution of each gene to the aetiology of the disease. Our results demonstrate that, firstly, 'gain-of-function' mutations in the PRSS1 gene may occasionally be found in an obvious ICP subject. Secondly, presumably 'loss-of-function' mutations in the PSTI gene appear to be frequent, with a detection rate of at least 10% in ICP and, finally, abnormal CFTR alleles are common: at least 20% of patients carried one of the most common CFTR mutations, and about 10% of patients were compound heterozygotes, having at least one 'mild' allele. Thus, in total, about 30% of ICP patients carried at least one abnormal allele in one of the three genes, and this is the most conservative estimate. Moreover, a trans-heterozygous state with sequence variations in the PSTI/CFTR genes was found in three patients. However, an association between the 5T allele in intron 8 of the CFTR gene and ICP remains unproven.
Chronic Disease↗
Macrocystic form of serous pancreatic cystadenoma.
OBJECTIVES: Macrocystic serous cystadenoma of the pancreas are benign lesions with sometimes difficult diagnostic issues. We aimed to describe clinicopathological and imaging features with cyst fluid analysis in a series of patients undergoing surgery for macrocystic serous cystadenoma. METHODS: Eight patients underwent pancreatic resection for a macrocystic lesion of the pancreas diagnosed on ultrasonography or CT. Endoscopic ultrasonography and preoperative fine-needle aspiration were performed in seven patients. Immunohistochemical analysis of the surgical specimen with antibodies to carcinoembryonic-antigen (CEA), carbohydrate antigen (CA) 19-9, estrogen receptor, and progesterone receptor antibodies was performed in all cases. RESULTS: Patients included seven women and one man, with a mean age of 48 yr. Lesions were incidentally discovered on ultrasonography in six patients and had a mean size of 3 cm (range, 1.5-5 cm). Endoscopic ultrasonography revealed millimetric cysts in three cases. In the seven aspirated cysts, cytological analysis was non-contributive, but biochemical analysis showed low content of CEA (< 5 ng/ml) and CA72.4 (< 40U/ml) in all but two. At histology, cysts were lined by clear cuboidal cells. They focally expressed CA19-9 but were negative for anti-CEA, antiestrogen receptor, and antiprogesterone receptor antibodies. Microscopic cysts in the wall of the lesions were demonstrated in five cases. CONCLUSIONS: Macrocystic serous cystadenoma is a particular variant of pancreatic serous cystadenoma. Endoscopic ultrasonography may be useful in detecting peripherally located millimetric cysts in unilocular lesions, and measurement of enzymes and tumor markers in cyst fluid may also contribute to the diagnosis showing low concentrations.
Adult↗
Benign inflammatory pancreatic mucinous cystadenomas mimicking locally advanced cystadenocarcinomas. Presentation of 3 cases.
We report 3 cases of benign mucinous cystadenoma of the pancreas mimicking, both clinically and on imaging findings, locally advanced cystadenocarcinoma spreading to neighbouring organs (stomach, splenic and mesenteric vessels, and diaphragm). Surgical resection was performed in light of the suspicion of invasive carcinoma in all 3 cases. Histological examination of the resected specimens showed entirely benign mucinous cystadenomas associated with marked peri-tumoural inflammation that accounted for the pre-operative misdiagnoses. All 3 patients are alive 40, 47 and 54 months after surgery without evidence of tumour relapse. These cases indicate that surgery must be considered in patients with cystic tumour of the pancreas suggesting locally advanced cystadenocarcinoma, even when pre-operative imaging findings suggest tumour extension into neighbouring organs.
Adult↗
[Acinar cystic transformation of the pancreas (or acinar cell cystadenoma), a rare and recently described entity].
We report a case of cystic acinar transformation (also known as acinar cell cystadenoma) of the pancreas. A 40-year old woman presented 3 episodes of acute pancreatitis that revealed a multicystic lesion of the ventral part of the head of the pancreas. A Whipple resection was performed. Gross examination of the specimen revealed a well-demarcated multiloculated cyst measuring 4 x 2 cm without communication with the excretory ducts. The cystic spaces contained a non-mucinous fluid. Histological analysis showed numerous various sized cysts that seemed to develop from microcystic structures in the surrounding acinar tissue. The cysts were lined by normal appearing acinar cells, with no atypia, sometimes flattened. Acinar cystic transformation is a rare lesion of the pancreas whose histogenesis is unknown. It should be distinguished from other lesions such as IPMT in order to discuss a surveillance strategy instead of surgical procedure.
Acute Disease↗
[Intraductal papillary mucinous tumor with pancreatobiliary and pancreatodigestive fistulae: a case report].
Pancreatobiliary or pancreatodigestive fistulae are rare during the course of intraductal papillary mucinous tumors. The mechanism of the fistula is either malignant involvement or mechanical. We report the case of an 81-year-old patient with intraductal papillary mucinous tumor complicated by fistulae between the main pancreatic duct and common bile duct, duodenum and stomach. These fistulae were treated endoscopically and radiologically by several metallic stents.
Adenocarcinoma, Mucinous↗
Prevalence of irritable bowel syndrome (IBS) and variability of diagnostic criteria.
OBJECTIVES: The main objectives of this study were to assess whether the use of different definitions of irritable bowel syndrome (IBS) could influence measurements of its prevalence and characterize the patient population fulfilling these different diagnostic criteria. METHOD: A telephone survey was carried out by contacting 8,221 subjects aged >or=18 Years representative of the French population. A "screening" questionnaire based on three algorithms of IBS classification (Manning, with or without a notion of a minimal duration of symptoms, Rome I and Rome II) was used by specialised inquirers. RESULTS: Twenty three percent of the subjects interviewed stated that they had suffered from abdominal pain during the previous 12 Months. The prevalence of IBS considerably varied, depending on the diagnostic criteria used: 12% based on Manning criteria without reference to the duration of symptoms; 2.5% if the notion of duration of symptoms was added to the Manning criteria, and 2.1% and 1.1% based on the Rome I and Rome II criteria, respectively (the latter including the same notion of duration). In total, 212 subjects (2.6%) met at least one of the criteria including a minimal duration of symptoms, with a predominance for women (sex-ratio close to 2). CONCLUSION: The prevalence of IBS is strongly dependent on the classification algorithm employed. The requirement of a minimum duration of symptoms eliminates IBS in a large number of subjects complaining of abdominal disorders. Once these methodological variations were taken into account, the prevalence of IBS in France was found to be comparable to that published in international literature.
Abdominal Pain↗
[Jejunal vipoma].
We report here case of a 57-year-old woman presenting with a metastatic vipoma revealed by secretory diarrhea and severe ionic disorders successfully treated by somatostatin administration. The primitive tumour, located in the jejunum, was identified peroperatively. Both the primitive lesion and the liver metastases were resected at the same time. Early tumour relapse occurred and was unsuccessfully treated by systemic chemotherapy (5-fluorouracil, streptozotocin and doxorubin) and chemoembolization. Finally, a liver transplantation was proposed.
Female↗
Quality of life scale and impact of a topical treatment on symptoms of gastro-esophageal reflux without severe esophagitis.
OBJECTIVE: Restoring a normal quality of life (QOL) should be the goal of treatments of gastro-esophageal reflux (GER) without severe esophagitis. In this analysis, carried out within the frame of a clinical study comparing a topical anti-inflammatory gel to a placebo, we assessed the relevance of the MOS SF-36 questionnaire in patients suffering from GER without severe esophagitis, and compared the scores of the studied patients to those of a representative sample of the French general population. PATIENTS AND METHODS: The patients had to suffer from GER symptoms for at least 2 months, with no or mild (< or =grade 1) esophagitis endoscopy. They were randomized to be given a 4 week treatment either with a topical gel containing gaïazulene and dimeticone or a placebo gel. Patients were asked to complete a symptom diary during the study, which allowed the calculation of mean symptom scores. The MOS SF-36 questionnaire was administered at baseline (d0) and after 4 weeks of treatment (d28). RESULTS: Two hundred and thirty three patients were enrolled in the study. At baseline, QOL scores were impaired in both groups. After 4 weeks of treatment: the treated patients displayed better improvement in all MOS SF-36 domains compared with the placebo group. The QOL profile in the treated group reached the level of the French reference population, while it remained impaired in the placebo group. CONCLUSION: This analysis evidenced the relevancy of the MOS SF-36 questionnaire to assess the impact of GER without severe esophagitis on QOL. Moreover, it demonstrated the capacity of the studied topical treatment to restore a normal QOL to the patients.
Administration, Topical↗