Budd-Chiari Syndrome as the Primary Manifestation of a Fibrolamellar Hepatocellular Carcinoma

Lamberts, R.; Nitsche, R.; Vivie, Rainer E. de; Peitsch, W.; Schauer, A.; Schuster, R.; Tebbe, Ulrich; Kreuzer, H; Creutzfeldt, W.

doi:10.1159/000200995

Cited by 17 publications

(6 citation statements)

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“…Although aggressive surgical treatment has been successfully performed in occasional patients with IVC with or without RA tumor thrombi [9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20], most such patients will die of tumor recurrence within 1 year after surgical procedures [10, 11, 12, 13, 14, 15, 17, 19]. In addition, such aggressive procedures are highly risky: 4 out of the 27 (15%) patients died within 4 weeks after their operation [17, 28, 30, 33].…”

Section: Discussionmentioning

confidence: 99%

“…Articles were excluded if they were mainly composed of an imaging study without the mention of specific therapy for cardiac tumors. A total of 27 cases with RA tumor thrombi in HCC who had received various treatments were identified [9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 28, 29, 30, 31, 32, 33]. Including the 3 patients in this presentation, there were 19 men and 11 women, with a median age of 58.5 years (range, 18–83).…”

Section: Literature Reviewmentioning

confidence: 99%

“…The likely outcome for HCC patients with symptomatic, intra-atrial tumor growth was dismal, with a median survival ranging from 1 to 4 months [4, 5, 6, 7, 8]. Although heroic aggressive surgical treatments, including the use of extracorporeal cardiopulmonary bypass and hepatic vascular exclusion techniques, as well as several nonsurgical local treatment modalities had been successfully performed in occasional patients [9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23], such procedures are usually prohibited in most advanced HCC patients because of coexisting underlying cirrhosis, an advanced stage of the primary tumor, the presence of metastatic disease, or a combination of the above.…”

Section: Introductionmentioning

confidence: 99%

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Hepatocellular Carcinoma with Intra-Atrial Tumor Thrombi

Chang

Chao

et al. 2004

Oncology

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Background: In patients with advanced hepatocellular carcinoma (HCC), inferior vena cava/intra-right atrial (IVC/RA) tumor thrombi are not uncommon findings and are usually associated with extremely poor outcome. Surgical interventions as well as nonsurgical approaches, such as transcatheter arterial chemoembolization and radiotherapy, have been used in the treatment of patients with symptomatic IVC/RA tumor thrombi. However, such therapeutic modalities are usually not feasible when a patient shows poor general performance, the presence of metastatic disease, and underlying hepatic dysfunction. Such patients show limited survival. Material and Methods: Herein we describe 3 patients with advanced-stage HCC whose IVC/RA tumor thrombi and primary tumors regressed remarkably after low-dose thalidomide (200–400 mg/day) therapy. An Entrez PUBMED search of English Literature articles was performed to identify other cases of RA tumor thrombi in HCC who had received various treatments. Results: Two of our patients survived for more than 15 months after the diagnosis of IVC/RA tumor thrombi, while the other had effective symptomatic palliation associated with a drastic fall of AFP serum levels and significant tumor regression within 4 weeks of thalidomide therapy. A literature review suggested that the survival of our patients is comparable with that of occasional patients who had received aggressive surgical intervention. Conclusions: Our results suggest that, despite the low tumor response rate in earlier studies, thalidomide therapy may sometimes provide effective palliation for patients with far advanced HCC with symptomatic IVC/RA tumor thrombi and who are not candidates for alternative treatment options.

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Section: Discussionmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

Section: Introductionmentioning

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Hepatocellular Carcinoma with Intra-Atrial Tumor Thrombi

Chang

Chao

et al. 2004

Oncology

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“…This infrequency of vascular invasion is in direct contrast with the portal and hepatic vein invasion that commonly occurs in patients with hepatocellular carcinoma (23). Rarely, fibrolamellar carcinoma has directly invaded and occluded the hepatic veins, resulting in Budd-Chiari syndrome (24). Rarely, fibrolamellar carcinoma has directly invaded and occluded the hepatic veins, resulting in Budd-Chiari syndrome (24).…”

Section: Learning Objectivesmentioning

confidence: 99%

From the Archives of the AFIP

McLarney

Rucker²,

Bender³

et al. 1999

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Fibrolamellar carcinoma is a malignant hepatocellular tumor with distinct clinical and pathologic differences from hepatocellular carcinoma. It differs from hepatocellular carcinoma in demographics, condition of the affected liver, tumor markers, and prognosis. Fibrolamellar carcinoma characteristically manifests as a large hepatic mass in adolescents or young adults (without gender predominance). Cirrhosis; elevated alpha-fetoprotein levels; and typical risk factors for hepatocellular carcinoma such as viral hepatitis, alcohol abuse, and metabolic disease are typically absent. Fibrolamellar carcinoma is characterized pathologically by cords of tumor cells surrounded by abundant collagenous fibrous tissue arranged in a parallel or lamellar distribution. Fibrotic lamellae often coalesce to form a central scar. Fibrolamellar carcinoma characteristically appears on radiologic images as a lobulated heterogeneous mass with a central scar in an otherwise normal liver. Radiologic evidence of cirrhosis, vascular invasion, or multifocal disease--findings typical of hepatocellular carcinoma--is uncommon in fibrolamellar carcinoma. Imaging features of fibrolamellar carcinoma overlap with those of other scar-producing lesions including focal nodular hyperplasia (FNH), hepatocellular adenoma and carcinoma, hemangioma, metastases, and cholangiocarcinoma. FNH, in particular, may simulate fibrolamellar carcinoma, since both have similar demographic and clinical characteristics. Because some believe that radiologic diagnosis of FNH is possible, it is important to understand the imaging appearance of fibrolamellar carcinoma to avoid misdiagnosing this malignant tumor as a FNH.

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“…1 Our patient presented with rare manifestations that included secondary Budd‐Chiari syndrome and significant clot burden causing lower extremity edema and ascites, bilateral pulmonary emboli causing shortness of breath, and a right atrial thrombus. 2 He had both tumor thrombus and bland thrombus, with the latter likely related to a hypercoagulable state. Alpha‐fetoprotein levels are usually normal.…”

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confidence: 99%