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. Cavo-hepatic confluence invasion is a poor prognostic situation, requiring extreme liver surgery for selected patients to achieve R margins (a crucial prognostic factor). Ex situ liver resection and autotransplantation (ELRA), developed by Pichlmayr et al., permits to achieve such R margin. 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 TNM R, 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 r-IVC leiomyosarcoma invading cavo-hepatic confluence in selected patients.