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Case Report: Multidisciplinary management of primary inferior vena cava leiomyosarcoma: a comprehensive case study
Wuchao Li1, Xiaoyong Zhang1, Yi Zhang2
1Department of Radiology, Guizhou Provincial People's Hospital, Guiyang, Guizhou, China.
Introduction:
Primary Inferior vena cava (IVC) leiomyosarcoma, a rare malignant tumor, presents unique challenges in diagnosis and treatment due to its rarity and the lack of consensus on surgical and adjuvant therapy approaches.
Case Report:
A 39-year-old female patient presented with lower limb swelling and mild fatigue. Contrast-enhanced CT identified a tumor mass within the dilated IVC. Abdominal MRI revealed primary IVC leiomyosarcoma extending into the right hepatic vein. A multidisciplinary consultation established a diagnosis and devised a treatment plan, opting for Ex-vivo Liver Resection and Auto-transplantation (ELRA), tumor resection and IVC reconstruction. Pathological examination confirmed primary IVC leiomyosarcoma. Postoperatively, the patient underwent a comprehensive treatment strategy that included radiochemotherapy, immunotherapy, targeted therapy, and PRaG therapy (PD-1 inhibitor, Radiotherapy, and Granulocyte-macrophage colony-stimulating factor). Despite the tumor's recurrence and metastasis, the disease progression was partially controlled.
Conclusion:
This case report emphasizes the complexities of diagnosing and treating IVC leiomyosarcoma and highlights the potential benefits of employing ELRA, IVC reconstruction, and PRaG therapy. Our study may serve as a valuable reference for future investigations addressing the management of this rare disease.
