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Budd-Chiari syndrome from pleomorphic sarcoma compressing the inferior vena cava: A case report
Brandon Camp1, Clifford N Danza1, Cameron Fateri1
1Department of Radiological Sciences, University of California, Irvine, CA 92868-3201, USA.
Insights
This report details the first known case of Budd-Chiari syndrome caused by metastatic undifferentiated pleomorphic sarcoma. The rare condition resulted from a mediastinal mass compressing the inferior vena cava, leading to liver dysfunction.
Area of Science:
- Medicine
- Oncology
- Radiology
Background:
- Budd-Chiari syndrome is a rare hepatic vascular condition.
- It involves obstruction of hepatic veins or the inferior vena cava, causing portal hypertension and liver dysfunction.
- Common causes include thrombosis and malignancy.
Observation:
- A 52-year-old male with metastatic undifferentiated pleomorphic sarcoma presented with abdominal pain and vomiting.
- Imaging revealed a mediastinal mass compressing the suprahepatic inferior vena cava.
- This compression led to severe congestive hepatopathy, consistent with Budd-Chiari syndrome.
Findings:
- This case represents the first reported instance of Budd-Chiari syndrome secondary to metastatic undifferentiated pleomorphic sarcoma in the English-language literature.
- The obstruction was caused by extrinsic compression from a metastatic mass.
- This expands the known oncological etiologies for Budd-Chiari syndrome.
Implications:
- Highlights undifferentiated pleomorphic sarcoma as a novel cause of malignancy-associated Budd-Chiari syndrome.
- Emphasizes the importance of considering extrinsic compression by metastatic disease in diagnosing Budd-Chiari syndrome.
- Underscores the diverse mechanisms by which malignancies can obstruct hepatic vasculature.
Abstract:
Budd-Chiari syndrome is a rare condition characterized by obstruction of the hepatic veins or inferior vena cava, leading to portal hypertension and liver dysfunction. While common etiologies include thrombosis or malignancy, we present, to our knowledge, the first reported case in the English-language literature of Budd-Chiari syndrome secondary to metastatic undifferentiated pleomorphic sarcoma. A 52-year-old male with an extensive history of metastatic undifferentiated pleomorphic sarcoma presented with a 10-day history of vomiting and abdominal pain. A contrast-enhanced computed tomography scan of the abdomen revealed a large mediastinal mass representing likely further metastasis compressing the suprahepatic inferior vena cava, resulting in severe congestive hepatopathy consistent with Budd-Chiari syndrome. While hepatocellular carcinoma, renal cell carcinoma, leiomyosarcoma, and other malignancies have been linked to Budd-Chiari syndrome, this report highlights a novel cause. In cases of Budd-Chiari syndrome secondary to malignancy, vessel obstruction may result from compression, infiltration, or bland thrombus formation.
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