Right Hepatectomy with Vascular and Biliary Reconstruction for a Portal Vein Leiomyosarcoma
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ORIGINAL ARTICLE – SARCOMA
Right Hepatectomy with Vascular and Biliary Reconstruction for a Portal Vein Leiomyosarcoma Stylianos Tzedakis, MD, Heithem Jeddou, MD, Haitham Triki, MD, Laurent Sulpice, MD, and Karim Boudjema, MD Department of Hepatobiliary and Digestive Surgery, Pontchaillou University Hospital, Rennes, France
ABSTRACT Background. Vascular leiomyosarcomas (LMS) are rare malignant mesenchymal tumors arising from vessel smooth muscle cells. They represent only 2% of soft tissue sarcomas and most commonly (50%) originate from the inferior vena cava.1–3 Portal vein LMS are very rare, and their resection combines en bloc negative margin sarcoma surgery principles and complex liver surgery procedures with vascular reconstructions.3–5 Methods. This is the case of a 42-year-old female presenting with a moderate cholestasis. Imaging revealed a 3-cm tumor originating from the main portal vein and its right branch while being in contact with both the right hepatic artery and biliary confluence. No metastases were identified. Core needle biopsy confirmed the diagnosis and tumor board decision was surgical resection. Results. The procedure included anatomic right hepatectomy with en bloc resection of the portal vein bifurcation, common bile duct, and biliary confluence. Portal venous reconstruction was performed using an autogenous external iliac vein interposition graft, while biliary reconstruction was performed via a Roux-en-Y end-to-side hepatico-jejunostomy. Duration of surgery was 300 min, and blood loss was 300 ml. Postoperative outcomes were uneventful and patient was discharged on postoperative day 8 with a
Electronic supplementary material The online version of this article (https://doi.org/10.1245/s10434-020-09021-4) contains supplementary material, which is available to authorized users.
transient right limb edema. Pathology confirmed R0 resection of a T1N0M0 leiomyosarcoma, Fe´de´ration Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade 2 tumor. Patient was free of disease at 20 months post-surgery. Conclusions. Portal vein leiomyosarcomas are extremely rare. En bloc extensive surgical resection should be proposed to obtain R0 resection, and achieve prolonged survival.4,6,7
DISCLOSURE
The authors declare no conflict of interest.
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