Ex Situ Liver Resection and Autotransplantation with Retrohepatic Inferior Vena Cava Reconstruction and Atrial Thrombectomy Under Extracorporeal Circulation for Inferior Vena Cava Leiomyosarcoma.

Background: Surgery is the only curative treatment for retrohepatic inferior vena cava (r-IVC) leiomyosarcoma. ¹ Cavo-hepatic confluence invasion is a poor prognostic situation, requiring extreme liver surgery for selected patients to achieve R ₀ margins (a crucial prognostic factor). Ex situ liver resection and autotransplantation (ELRA), developed by Pichlmayr et al., permits to achieve such R ₀ margin. ^2,3 METHODS: An 84-year-old patient in excellent condition (ECOG 0), without relevant past medical history, was referred for abdominal mass, bilateral lower limbs edema, and dyspnea. Workup revealed a large r-IVC leiomyosarcoma invading cavo-hepatic confluence and protruding in right atrium without any metastasis. After multidisciplinary consultation, surgical treatment was retained. Preoperative transoesophaegal echocardiography confirmed a 4-cm protruding tumoral thrombus in right atrium without abdominalisation possibility.
Results: A sterno-laparotomy was performed, consisting of a right nephrectomy for exposure and en bloc total hepatectomy comprising r-IVC after atriotomy for intracardiac thrombectomy under extracorporeal circulation. Tumorectomy (rIVC + segment I and IX) was performed on back table followed by a r-IVC reconstruction through a tubulized homologous venous patch. Native IVC was reconstructed as well, permitting a side-to-side cavo-caval anastomosis for liver reimplantation. Postoperative evolution was eventless except for an early bile leak that required surgical exploration. The patient was discharged on postoperative day 32. Pathological examination confirmed r-IVC-leiomyosarcoma T ₄ N ₀ M ₀ R ₀ , FNCLCC grade 2. Eight months after surgery, general status was conserved with disappearance of symptoms, and IVC was permeable without leiomyosarcoma recurrence.
Conclusion: Ex situ liver resection and autotransplantation with atrial thrombectomy is a surgical possibility for R ₀ r-IVC leiomyosarcoma invading cavo-hepatic confluence in selected patients.
(© 2024. Society of Surgical Oncology.)