Genomic stability prevails in North-African hepatocellular carcinomas.

The molecular pathogenesis of hepatocellular carcinoma, a tumour characterized by a vast clinical heterogeneity, remains unexplored outside Europe and Eastern Asia. We analysed by direct sequencing or loss of heterozygosity assay, the common targets of genomic alterations in 42 hepatocellular carcinomas collected in western North-Africa. Overall, genomic instability was uncommon, allelic losses affecting mostly chromosomes 1p, 4q, 8p and 17p (24-28% of cases). CTNNB1 and TP53 were infrequently mutated (9 and 17% of cases, respectively). Surprisingly, TP53 mutation R249S, diagnostic of aflatoxin B1 exposure, usually frequent in Africa, was exceptional (one case), indicating that in western North-Africa, hepatocellular carcinoma genetics differs markedly from that of the remainder of the continent.

[Congenital cystic dilatation of bile ducts]. / Les dilatations kystiques congénitales des voies biliaires.

Congenital cystic dilatation of bile ducts is a rare condition. We report a retrospective study about 18 patients having congenital bile duct cysts. According to Todani's classification, 11 cases were type I and 7 were type V. Six patients from the first group had a pancreatobiliary maljunction. A total resection of the cyst was conducted in the type I cysts. Anatomopathologic examination showed an adenocarcinoma of a common bile duct cyst. In one case, a cancer of the gall bladder associated to a common bile duct cyst in another case. Three patients with segmental dilatation of intrahepatic bile ducts (type V) underwent liver resection. Four patients had a diffuse form, one of them was treated by percutaneous drainage, and in the other cases a hepatojejunostomy was performed. Postoperative course was complicated with acute cholangitis in these four cases. Percutaneous drainage and antibiotics allowed a positive outcome in most of the cases. In one case, secondary biliary cirrhosis occurred as a long-term complication. Congenital cystic dilatation of bile ducts is considered to be a precancer state. Enterocystic anastomosis is proscribed and the resection has to be as complete as possible.

[Survival and prognostic factors of colorectal adenocarcinoma: analytic multifactor review of 150 cases]. / Survie et facteurs pronostiques des adénocarcinomes colorectaux: étude analytique uni- et multifactorielle de 150 cas.

INTRODUCTION: Prognostic factors have a pivotal role in clinical oncology. They are helpful in the selection of treatment; provide insights into the disease process and the therapic response. The number of possibility useful prognosis factors in the colorectal cancer is large. This study attempts to observe the survival of colorectal adenocarcinoma and to find prognostic factors and other variables potentially associated with outcome of colorectal adenocarcinoma. MATERIAL AND METHODS: It's a retrospective study based on 150 patients with colorectal adenocarcinoma from 1990 to 2002. There were 150 patients aged of 58 years (median 61 years) with 1.4 sex-ratio. 84 patients had colon adenocarcinoma and 66 patients had rectal adenocarcinoma. In histological exam the adenocarcinoma was well differenced in 69 cases (46%), and undifferentiated in 17 cases (18, 3%). RESULTS: Locoregional extension was found in 18 cases and metastatic extension in 45 cases with hepatic metastasis in 37 cases and pulmonary metastasis in 8 cases. There were 6 cases of peritoneal localized carcinosis and 6 cases of ovary metastasis. There were 6 patients (4%) Dukes stage I TNM, 61 stage II (40, 7%), 51 stage III TNM (34%) and 32 patients stage IV TNM (34%). All patients had surgical curative resection associated with adjuvant chemotherapy in 60 cases of colon adenocarcinoma and preoperative radiotherapy in 33 cases of rectal adenocarcinoma. After a follow up of 46 months, 52 patients was died (10 operative mortality), 35 patients were lost of view and 63 patients were still alive at the point date. Median survival was 20 months with 95% confidence interval: (4, 2-7, 8). Overall one year and 5 years survival were respectively 92,8% and 26,3%. Various prognostic factors had been identified through univariate (Kaplan-Meier) then multivariate (Cox) analyze. In addition to the clinical factors, we found of significant prognostic value undifferentiated adenocarcinoma and an elevated value of serum carcinoembryonic antigen>5 ng/ml.

Nodular hyperplasia of the gastrointestinal tract after liver transplantation: role of immunosuppressive therapy? A case report.

Nodular lymphoid hyperplasia (NLH) of the gastrointestinal tract is a rare disease usually reported in patients with congenital or acquired immunodeficiency and chronic gastrointestinal infections. However, no case of NLH in a patient receiving immunosuppressive therapy has been reported to date. We describe the case of a woman who developed chronic diarrhea related to NLH 9 years after liver transplantation. Other causes of diarrhea and NLH were excluded. Her immunosuppressive regimen consisted on mycophenolate mofetil (MMF) and tacrolimus. Reduction of MMF dose improved symptoms but led to a rising aminotransferase level. Given the risk of graft rejection, MMF at full dose was resumed and she was started on symptomatic treatment for diarrhea. The role of immunosuppressive drugs in the pathogenesis of NLH may be related to the reduction of T- and B-lymphocyte proliferation and decreasing antibody production. NLH will further develop to compensate functionally inadequate lymphoid tissue, as reported in congenital immunodeficiency states.

Intrahepatic Osler's disease: report of two cases and review of the literature.

Osler-Weber-Rendu disease is a hereditary vascular disease with multiple manifestations. The liver is involved rarely. Vascular abnormalities include telangiectasis and arteriovenous fistulas, sometimes associated with fibrosis and cirrhosis. Hepatic arteriovenous shunting may include secondary portal hypertension, reduced liver function and high cardiac output. Two cases of Osler-Weber-Rendu disease with extensive hepatic arteriovenous fistulation were described in detail and we report on their clinical features. In the first patient, treatment was symptomatic since liver transplantation is not indicated because the patient was asymptomatic. Embolization treatment of hepatic arteries was indicated in the second patient because he had biliary disease and recurrent cholangitis secondary to vasculo-biliary shunts. Therapy with arterial embolization, banding, or ligature of hepatic arteries is still limited and provides unsatisfactory long-term results. Liver transplantation offers now another therapeutic option for patients with intrahepatic high shunting and secondary pulmonary hypertension.

Cystic mesenchymal hamartoma of the liver report of a case and review of the literature.

Mesenchymal Hamartoma (MH) of the liver constitutes the third or the fourth most common tumour of the liver in childhood and occurs most commonly in the first two years of life. It is often misdiagnosed clinically as a malignant tumour because of its rapid increase in size within a short period of time, or as a hepatic collections or abscess because of its cystic appearance. Although a benign lesion, MH may cause heart failure due to arteriovenous shunts, or death as a result of respiratory complications. A typical case of MH was recently encountered in a 6-years-old-boy. The patient presented with progres sive abdominal distension; surgery revealed a large mass arising from the right lobe of the liver. The mass was predominantly formed by fluid collections. Loose mesenchymal tissue and branched, tortuous bile ducts were the key diagnostic features. When predominantly cystic, MH may mimic lymphangioma both grossly and microscopically. Prudent examination of the cystic structures can establish a correct diagnosis.

[Synovial lipoma arborescens]. / Lipomes arborescents de la synoviale.

The synovial lipoma are uncommon articular tumors of unknown origin. We report two cases of synovial lipoma arborescens arisen in a man and a woman respectively 41 and 27 years old. Both had already consulted for a tumefaction progressively increasing in volume. It was accompanied in the first case by a laxity in the knee joint. Radiography and arthroscopy showed an important hyperplasia of the articular synovia. A total synovectomy was performed in both cases. The synovia had a hairy aspect and was extremely thickened. It weighted 1.5 kg in the first case and its section had a fatty aspect. The histological examination confirmed the diagnosis of synovial lipoma arborescens. The observed aspect of the two tumors and particularly their volume, which was very important in the first case are arguments in favor of their tumoral nature.

Case report: Primary pure squamous cell carcinoma of the gallbladder.

Squamous cell carcinoma of the gallbladder is rare, accounting for only 0.5-12.7% of all malignant gallbladder tumors. Tumor progression is rapid but silent and therefore usually discovered at an advanced stage, hence its poor prognosis. We report the observation of a 69-year-old woman with known cholelithiasis, admitted for biliary colic. CT scan highlighted a thick-walled gallbladder harboring a tumor invading segments IV and V of the liver. The patient underwent cholecystectomy associated with bisegmental hepatic resection. Pathology showed a well-differentiated, keratinizing squamous cell carcinoma, infiltrating the entire gallbladder wall and the adjacent hepatic parenchyma.