ABSTRACT
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease strongly associated, in the western series, to inflammatory bowel disease (IBD) and particularly to chronic ulcerative colitis. North African data are rare. aim : To study the epidemiological, clinicobiological and morphological profile of PSC in Tunisia. methods: A retrospective multicenter study extended over a period of 14 years (1995-2009), including all patients suffering from PSC, hospitalized in the four participants departments. We collected epidemiological, clinico-biological, histopathologic and morphological data for each patient. results: We brought together 33 patients (22 men and 11 women), middle aged 44 years. The disease was symptomatic in 73% of cases. Laboratory tests showed cholestasis (100%) associated with hyperbilirubinemia (72%) and a moderate cytolysis (78%). Morphological analysis of bile ducts by cholangioMRI or endoscopic retrograde cholangio-pancreatography objectified diffuse damage of the biliary tract in 61% of cases. Association with IBD was found in 33% of cases (Crohn's Disease: 27%, chronic ulcerative colitis: 6%). An association with autoimmune hepatitis was found in 6% of cases and primary biliary cirrhosis in 3% of cases. Conclusion : PSC is rare in Tunisia and affects men more often than women. The association with IBD is less frequent than in literature. It concerns essentially Crohn's disease. These data require confirmation by prospective multicenter studies.
Subject(s)
Cholangitis, Sclerosing/diagnosis , Cholangitis, Sclerosing/epidemiology , Adolescent , Adult , Aged , Cholangiopancreatography, Endoscopic Retrograde , Cholangitis, Sclerosing/complications , Cholestasis/etiology , Colitis, Ulcerative/epidemiology , Comorbidity , Diagnosis, Differential , Female , Humans , Hyperbilirubinemia/etiology , Incidence , Inflammatory Bowel Diseases/epidemiology , Magnetic Resonance Imaging , Male , Middle Aged , Retrospective Studies , Risk Factors , Sex Distribution , Tunisia/epidemiologySubject(s)
Neuroectodermal Tumors, Primitive, Peripheral/diagnosis , Pelvic Neoplasms/diagnosis , Sigmoidoscopy , Tomography, X-Ray Computed , Adult , Diagnosis, Differential , Fatal Outcome , Humans , Male , Neoplasm Staging , Neuroectodermal Tumors, Primitive, Peripheral/pathology , Sigmoidoscopy/methodsABSTRACT
Hepatoid adenocarcinoma is a rare variant of extrahepatic adenocarcinoma which behaves like hepatocellular carcinoma in morphology and functionality. We present a rare case of hepatoid adenocarcinoma of the gallbladder which invades deeply the liver bed, in a 59-year-old woman. Histologically, most of the mass in the gallbladder was composed of cells with eosinophilic cytoplasm arranged in a trabecular pattern, which resembled hepatocellular carcinoma. The main differential diagnosis was hepatocellular carcinoma with invasion into the gallbladder. The gallbladder origin of the hepatoid adenocarcinoma was verified by the presence of foci of conventional adenocarcinoma, the recognition of high-grade dysplasia in the adjacent epithelium and the absence of cirrhosis.
