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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][2][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][4][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 transient right limb edema. Pathology confirmed R0 resection of a T1N0M0 leiomyosarcoma, Fédération 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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We report the case of a patient with exceptional survival over 8 years after left trisectionectomy combined with portal vein and hepatic artery resection and reconstruction for advanced perihilar cholangiocarcinoma. Such extended hepatectomy with vascular resection is the only way to obtain free tumor margin. It can be performed with acceptable morbidity and mortality and it is the only hope to prolong survival.