Tenascin-W Is a Novel Stromal Marker in Biliary Tract Cancers

Extrahepatic cancers of the biliary system are typically asymptomatic until after metastasis, which contributes to their poor prognosis. Here we examined intrahepatic cholangiocarcinomas (n = 8), carcinomas of perihilar bile ducts (n = 7), carcinomas of the gallbladder (n = 11) and hepatic metastasis from carcinomas of the gallbladder (n = 4) for the expression of the extracellular matrix glycoproteins tenascin-C and tenascin-W. Anti-tenascin-C and anti-tenascin-W immunoreactivity was found in all biliary tract tumors examined. Unlike tenascin-C, tenascin-W was not detected in normal hepatobiliary tissue. Tenascin-W was also expressed by the cholangiocarcinoma-derived cell line Huh-28. However, co-culture of Huh-28 cells with immortalized bone marrow-derived stromal cells was necessary for the formation and organization of tenascin-W fibrils in vitro. Our results indicate that tenascin-W may be a novel marker of hepatobiliary tumor stroma, and its absence from many normal tissues suggests that it may be a potential target for biotherapies

Computed Tomographic presentation of obstructive jejunal adenocarcinoma associated with celiac disease and incomplete intestinal malrotation

Introduction: Small bowel adenocarcinoma is a rare entity most frequently observed with celiac disease. This is the first case report on the association of celiac disease, small bowel adenocarcinoma and intestinal malrotation. Case report: A 40 year-old male patient diagnosed with celiac disease since the age of 5 years complained of epigastric pain and vomiting for three days. Computed tomography (CT) showed a significant gastroduodenal dilatation with thickened intestinal wall proximal to the duodenojejunal flexure. The lumen contained a food bezoar in the center. The duodenojejunal angle was abnormally on the right side of the abdomen and the superior mesenteric vein was anterior to the superior mesenteric artery. Endoscopy after aspiration found a hemi-circumferential and irregular mass which bled at the contact of fibroscope. Biopsies showed an adenocarcinoma and small bowel resection was performed. Discussion: Celiac disease is associated with a high risk of small bowel cancer. The association of incomplete intestinal malrotation, duodenojejunal flexure tumor and celiac disease made the surgery challenging. Conclusion: Patients with celiac disease should be carefully monitored and endoscopic or radiologic investigations should be carried out in patients with any doubtful symptoms

Familial vertebral segmentation defects, Sprengel anomaly, and omovertebral bone with variable expressivity

A 10-year-old boy was found to have an unusual presentation of the Sprengel anomaly, omovertebral bones, and segmentation defects of the vertebral column at the cervical, thoracic, and sacral level. In addition, he showed hypertelorism, downslanting palpebral fissures, ptosis, webbing, and hypoplasia of the thenar and hypothenar areas. He had moderate mental delay. In addition to the segmentation defects and omovertebral bones, radiological studies showed a small pelvis and 11 pairs of ribs. Some of the features were present in the mother, and minimal symptoms were present in the father. The parents were consanguineous. A paternal cousin had segmentation defects, omovertebral bones, and a Sprengel deformity as well, although with milder presentation than the proband. We were unable to find a similar combination of manifestations in literature. The familial occurrence is best compatible with autosomal dominant inheritance, showing wide variability of expression. It is possible that the more notable signs in the proband can be explained by homozygosity for the disorder. (c) 2005 Wiley-Liss, In