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      An Uncommon Presentation of Extrahepatic Cholestasis due to Single Biliary Stricture From Kaposi Sarcoma

      case-report

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          Abstract

          Kaposi sarcoma is one of the acquired immunodeficiency syndrome (AIDS) defining diseases. AIDS-associated Kaposi sarcoma affects primarily the skin and the lungs. Although gastrointestinal involvement is relatively common, biliary tract involvement has rarely been reported. It has been associated mostly with extension from liver disease. We describe an uncommon presentation of disseminated Kaposi sarcoma causing extrahepatic cholestasis due to extrahepatic biliary tract involvement that resolved after sphincterotomy with biliary stenting.

          We present a case of a 35-year-old African American male diagnosed with human immunodeficiency virus (HIV) infection in 2005. He presented with AIDS after discontinuation of antiretroviral therapy for one year, subsequently being diagnosed with systemic Kaposi sarcoma. He presented with signs and symptoms of obstructive biliary disease, including jaundice, abdominal pain, fatigue, and fever. We encountered a rare presentation of malignant single extrahepatic biliary stenosis secondary to biliary Kaposi sarcoma. The biochemical pattern markedly improved after endoscopic retrograde cholangiopancreatography with sphincterotomy and stenting. However, and despite the resumption of combined antiretroviral therapy, deep immunosuppression caused worsening clinical condition and death five months after initial presentation.

          Certainly, among the multiple etiologies of biliary obstruction in AIDS, Kaposi sarcoma is one to consider.

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          Most cited references20

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          Kaposi sarcoma.

          Kaposi sarcoma (KS) is a low-grade vascular tumor associated with Kaposi sarcoma herpesvirus/human herpesvirus 8 (KSHV/HHV8) infection. Kaposi sarcoma lesions predominantly present at mucocutaneous sites, but may involve all organs and anatomic locations. Recognized epidemiologic-clinical forms of KS include classic, African (endemic), AIDS-associated (epidemic), and iatrogenic KS. New clinical manifestations have been described, such as antiretroviral therapy-related KS regression or flares. Kaposi sarcoma lesions evolve from early (patch stage) macules into plaques (plaque stage) that grow into larger nodules (tumor stage). Newer histologic variants include anaplastic, hyperkeratotic, lymphangioma-like, bullous, telangiectatic, ecchymotic, keloidal, pyogenic granuloma-like, micronodular, intravascular, glomeruloid and pigmented KS, as well as KS with sarcoidlike granulomas and KS with myoid nodules. Latency-associated nuclear antigen (HHV8) is the most specific immunohistochemical marker available to help distinguish KS from its mimics. Since KS remains one of the most common AIDS-defining malignancies, it is important that pathologists be able to recognize KS and its contemporary manifestations.
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            HIV-associated Kaposi sarcoma and related diseases.

            : The search for the etiologic agent for Kaposi sarcoma led to the discovery of Kaposi sarcoma-associated herpesvirus (KSHV) in 1994. KSHV, also called human herpesvirus-8, has since been shown to be the etiologic agent for several other tumors and diseases, including primary effusion lymphoma (PEL), an extracavitary variant of PEL, KSHV-associated diffuse large B-cell lymphoma, a form of multicentric Castleman disease, and KSHV inflammatory cytokine syndrome. KSHV encodes several genes that interfere with innate and specific immunity, thwart apoptosis, enhance cell proliferation and cytokine production, and promote angiogenesis, and these play important roles in disease pathogenesis. HIV is an important cofactor in Kaposi sarcoma pathogenesis, and widespread use of antiretroviral therapy has reduced Kaposi sarcoma incidence. However, Kaposi sarcoma remains the second most frequent tumor arising in HIV-infected patients in the United States and is particularly common in sub-Saharan Africa. KSHV prevalence varies substantially in different populations. KSHV is secreted in saliva, and public health measures to reduce its spread may help reduce the incidence of KSHV-associated diseases. Although there have been advances in the treatment of Kaposi sarcoma, KSHV-multicentric Castleman disease, and PEL, improved therapies are needed, especially those that are appropriate for Kaposi sarcoma in resource-poor regions.
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              Randomized trial of paclitaxel versus pegylated liposomal doxorubicin for advanced human immunodeficiency virus-associated Kaposi sarcoma: evidence of symptom palliation from chemotherapy.

              Paclitaxel and pegylated liposomal doxorubicin (PLD) are active cytotoxic agents for the treatment of human immunodeficiency virus (HIV)-associated Kaposi sarcoma (KS). A randomized trial comparing the efficacy and toxicity of paclitaxel and PLD was performed, and the effects of therapy on symptom palliation and quality of life were determined. Patients with advanced HIV-associated KS were randomly assigned to receive paclitaxel at a dose of 100 mg/m2 intravenously (iv) every 2 weeks or PLD at a dose of 20 mg/m2 iv every 3 weeks. The KS Functional Assessment of HIV (FAHI) quality of life instrument was used before and after every other treatment cycle. The study included 73 analyzable patients enrolled between 1998 and 2002, including 36 in the paclitaxel arm and 37 in the PLD arm; 73% of patients received highly active antiretroviral therapy (HAART) and 32% had an undetectable viral load (<400 copies/mL). Treatment was associated with significant improvements in pain (P=.024) and swelling (P<.001). Of the 36 patients who reported that pain interfered with their normal work or activities at baseline, 25 (69%) improved. Of the 41 patients who reported swelling at baseline, 38 (93%) improved. Comparing the paclitaxel and PLD arms revealed comparable response rates (56% vs 46%; P=.49), median progression-free survival (17.5 months vs 12.2 months; P=.66), and 2-year survival rates (79% vs 78%; P=.75), but somewhat more grade 3 to 5 toxicity for paclitaxel (84% vs 66%; P=.077). Treatment with either paclitaxel or PLD appears to produce significant improvements in pain and swelling in patients with advanced, symptomatic, HIV-associated KS treated in the HAART era. Copyright (c) 2010 American Cancer Society.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                29 June 2020
                June 2020
                : 12
                : 6
                : e8913
                Affiliations
                [1 ] Internal Medicine, Cook County Health and Hospital System, Chicago, USA
                [2 ] Internal Medicine, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA
                [3 ] Gastroenterology and Hepatology, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA
                Author notes
                Article
                10.7759/cureus.8913
                7389956
                70231436-9ab6-4fd5-8496-3bebca8f4f12
                Copyright © 2020, Flores Córdova et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 15 May 2020
                : 29 June 2020
                Categories
                Gastroenterology
                HIV/AIDS
                Infectious Disease

                extrahepatic cholestasis,acquired immunodeficiency syndrome,biliary tract,case report,kaposi sarcoma

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