Case Report: Multidisciplinary management of primary inferior vena cava leiomyosarcoma: a comprehensive case study.

Abstract

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. 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. 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.