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      Common bile duct adenocarcinoma in a patient with situs inversus totalis: report of a rare case

      case-report

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          Abstract

          Background

          Situs inversus totalis represents an unusual anomaly characterized by a mirror-image transposition of the abdominal and thoracic viscera. It often occurs concomitantly with other disorders that make difficult diagnosis and management of abdominal pathology. The relationship between situs inversus totalis and cancer remains unclear.

          Case presentation

          We describe a 33-year old Guinean man with situs inversus totalis who presented with obstructive jaundice. Imaging and endoscopic modalities demonstrated a mass of distal common bile duct which biopsy identified an adenocarcinoma. The patient was successfully treated by cephalic pancreaticoduodenectomy followed by adjuvant chemoradiation and he is doing well without recurrence 8 months after surgery.

          Conclusion

          The occurrence of bile duct adenocarcinoma in patient with situs inversus totalis accounts as a rare coincidence. In this setting, when the tumor is resectable, surgical management should be considered without contraindication and must be preceded by a careful preoperative staging.

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          Most cited references21

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          Nuclear factor-kappa B and cancer: its role in prevention and therapy.

          Cancer is a hyperproliferative disorder in which invasion and angiogenesis lead to tumor metastasis. Several genes that mediate tumorigenesis and metastasis are regulated by a nuclear transcription factor, nuclear factor kappa B (NF-kappaB). A heterotrimeric complex consisting of p50, p65, and IkappaBalpha, NF-kappaB is present in its inactive state in the cytoplasm. When NF-kappaB is activated, IkappaBalpha is degraded and p50-p65 heterodimer is translocated to the nucleus, binds the DNA (at the promoter region), and activates gene. Research within the last few years has revealed that NF-kappaB is activated by carcinogens, tumor promoters, inflammatory cytokines, and by chemotherapeutic agents. The activation of NF-kappaB can suppress apoptosis, thus promoting chemoresistance and tumorigenesis. Interestingly, however, most chemopreventive agents appear to suppress the activation of the NF-kappaB through inhibition of NF-kappaB signaling pathway. These chemopreventive agents also sensitize the tumors to chemotherapeutic agents through abrogation of NF-kappaB activation. Overall, these observations suggest that NF-kappaB is an ideal target for chemoprevention and chemosensitization. This article reviews evidence supporting this hypothesis.
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            The primary cilium at the crossroads of mammalian hedgehog signaling.

            Cilia function as critical sensors of extracellular information, and ciliary dysfunction underlies diverse human disorders including situs inversus, polycystic kidney disease, retinal degeneration, and Bardet-Biedl syndrome. Importantly, mammalian primary cilia have recently been shown to mediate transduction of Hedgehog (Hh) signals, which are involved in a variety of developmental processes. Mutations in several ciliary components disrupt the patterning of the neural tube and limb bud, tissues that rely on precisely coordinated gradients of Hh signal transduction. Numerous components of the Hh pathway, including Patched, Smoothened, and the Gli transcription factors, are present within primary cilia, indicating that key steps of Hh signaling may occur within the cilium. Because dysregulated Hh signaling promotes the development of a variety of human tumors, cilia may also have roles in cancer. Together, these findings have shed light on one mechanism by which primary cilia transduce signals critical for both development and disease.
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              • Article: not found

              Human laterality disorders.

              Heterotaxia is a group of congenital disorders characterized by a misplacement of one or more organs according to the left-right axis. Bilateral asymmetry of internal organs is conserved among all vertebrate species. Analyses in animal models such as mouse, chicken, frog and zebrafish allowed for a remarkable progress of knowledge on the embryonic and genetic mechanisms underlying internal left-right asymmetry. In this review we focus on the insights from these model organisms that are useful for a better understanding of the etiology and pathogenesis of human heterotaxia. The known causes of human heterotaxia are reviewed and situated within the conceptual framework that originates from vertebrate model organisms. Furthermore, we attempt to apply the rapidly increasing insights gained from both animal models and human genetics to clinical practice in order to contribute to a more accurate conceptual classification, genetic diagnosis and counseling.
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                Author and article information

                Journal
                BMC Res Notes
                BMC Res Notes
                BMC Research Notes
                BioMed Central
                1756-0500
                2012
                12 December 2012
                : 5
                : 681
                Affiliations
                [1 ]Department of medical oncology, Hassan II University Hospital, Fez, Morocco
                [2 ]Department of radiology, University Hospitals of Geneva, Geneva, Switzerland
                [3 ]Department of general surgery, University Hospitals of Geneva, Geneva, Switzerland
                [4 ]Department of gastroenterology, University Hospitals of Geneva, Geneva, Switzerland
                [5 ]Department of pathology, University Hospitals of Geneva, Geneva, Switzerland
                [6 ]Department of medical oncology, University Hospitals of Geneva, Geneva, Switzerland
                Article
                1756-0500-5-681
                10.1186/1756-0500-5-681
                3532423
                23234596
                8f0c8dc7-cc60-4950-a6d7-7efe7652ab3c
                Copyright ©2012 Benhammane et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 September 2012
                : 27 November 2012
                Categories
                Case Report

                Medicine
                situs inversus totalis,bile duct cancer,preoperative staging,surgical management
                Medicine
                situs inversus totalis, bile duct cancer, preoperative staging, surgical management

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