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Primary leiomyosarcoma of the inferior vena cava with Budd-Chiari syndrome
M Imakita1, C Yutani, H Ishibashi-Ueda
1Division of Pathology, National Cardiovascular Center, Osaka, Japan.
Insights
A rare case of inferior vena cava leiomyosarcoma causing Budd-Chiari syndrome is presented. This aggressive tumor led to rapid hepatic failure and death in a 44-year-old woman.
Area of Science:
- Oncology
- Vascular Surgery
- Hepatology
Background:
- Leiomyosarcoma is a rare malignant mesenchymal tumor.
- Inferior vena cava (IVC) leiomyosarcomas are exceptionally rare.
- Budd-Chiari syndrome (BCS) is a condition caused by obstruction of hepatic venous outflow.
Observation:
- A 44-year-old Japanese woman presented with symptoms of Budd-Chiari syndrome.
- The patient was diagnosed with leiomyosarcoma originating from the middle segment of the IVC.
- The tumor obstructed the IVC and extended into the right atrium.
Findings:
- The patient experienced rapid disease progression, succumbing to hepatic failure approximately 3 months post-symptom onset.
- A review of 28 previously reported cases of IVC leiomyosarcoma associated with BCS was conducted.
- This case highlights the aggressive nature and poor prognosis of IVC leiomyosarcoma presenting as BCS.
Implications:
- Early diagnosis and prompt management are crucial for improving outcomes in patients with IVC leiomyosarcoma.
- Further research into the pathogenesis and treatment strategies for IVC leiomyosarcoma is warranted.
- This case contributes to the limited literature, aiding in understanding this rare oncological and vascular emergency.
Abstract:
A 44-year-old Japanese woman with leiomyosarcoma of the inferior vena cava is reported. She presented with Budd-Chiari syndrome and died of hepatic failure about 3 months after the onset of symptoms. The tumor arose from the middle segment of the inferior vena cava, occluded the inferior vena cava and projected into the right atrium. A total of 28 cases of Budd-Chiari syndrome due to primary leiomyosarcoma of the inferior vena cava are reviewed.