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

BackgroundSurgery 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 R0 margins (a crucial prognostic factor). Ex situ liver resection and autotransplantation (ELRA), developed by Pichlmayr et al., permits to achieve such R0 margin.2,3MethodsAn 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.ResultsA 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 T4N0M0 R0, FNCLCC grade 2. Eight months after surgery, general status was conserved with disappearance of symptoms, and IVC was permeable without leiomyosarcoma recurrence.ConclusionEx situ liver resection and autotransplantation with atrial thrombectomy is a surgical possibility for R0 r-IVC leiomyosarcoma invading cavo-hepatic confluence in selected patients.