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• W2922503858 abstract "Introduction Budd Chiari Syndrome (BCS) is defined as any pathophysiologic process that results in interruption in normal blood outflow of the liver. It encompasses total or partial occlusion of any of the hepatic veins with or without involvement of the inferior vena cava. In over 80% of patients with BCS, an underlying disorder can be identified. Among the most common causes are hematologic abnormalities such as myeloproliferative disorders, hypercoagulable states and idiopathic BCS. Malignancies account for less than 10% of BCS etiologies. The most common malignancy is hepatocellular carcinoma, followed by renal and adrenal cancers, and sarcomas of the right atrium and the inferior vena cava (IVC) or hepatic veins. We present an extraordinarily rare case of BCS caused by endovascular leiomyosarcoma. Case An 82-year-old gentleman with a remote history of stage I lymphoma, advanced prostatic adenocarcinoma status post radiation and anti-androgen therapy, and recurrent venous thromboembolisms for which he was on anticoagulation, presented with one week of somnolence, confusion and abdominal distension. Pertinent laboratory studies on admission revealed an albumin of 3.2 g/dL, an aspartate aminotransferase of 56 U/L, an alanine aminotransferase of 27 U/L, a prothrombin time of 24.7 seconds, and an ammonia of 58 micro mol/L. Computed tomography (CT) of abdomen and pelvis with contrast revealed near complete occlusion of right hepatic vein with thrombus extension into the IVC. These findings were consistent with Budd Chiari Syndrome. Hypercoagulable work up including JAK2V617F mutation study and flow cytometry was negative. Hepatic venography was performed and minimal improvement of filling defect occurred following rheolytic thrombectomy and balloon venoplasty raising concern for an intravascular mass. He subsequently underwent a CT-guided biopsy of the mass that revealed spindle cell neoplasm with smooth muscle differentiation consistent with leiomyosarcoma (LMS). Discussion Acute Budd Chiari Syndrome caused by LMS of the IVC is an exceedingly rare entity and generally entails a poor prognosis. In general, the mainstay of management in BCS is rectifying underlying disorders, initiating anticoagulation and treating complications of portal hypertension. In cases caused by LMS of the IVC, radical resection of the tumor, even though it entails a high rate of local recurrence, is the preferred management approach for long-term survival.2335_A Figure 1. Pathology of tumor revealing spindle cell neoplasm with mild cytologic atypia2335_B Figure 2. CT abdomen revealing tumor in IVC" @default.
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• W2922503858 date "2018-10-01" @default.
• W2922503858 modified "2023-10-02" @default.
• W2922503858 title "Budd-Chiari Syndrome: Is That a Clot?" @default.
• W2922503858 doi "https://doi.org/10.14309/00000434-201810001-02334" @default.
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