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Journal Articles Indian Journal of Cancer Year : 2019

Portal vein leiomyosarcoma A rare case of hepatic hilar tumor with review of the literature

Abstract

Portal vein leiomyosarcoma (LMS) is a very rare entity with poor prognosis. Only few cases have been reported in the literature. We report the case of a 53-year-old man with portal vein LMS presenting as a hilar tumor and causing cholestasis. The tumor measured 10 cm and was responsible for right portal vein thrombosis and compression of the biliary convergence. A right hepatectomy with hilar en bloc resection was performed with portal vein and biliary reconstruction. Pathology confirmed the complete resection of a well-differentiated leiomyosarcoma of the portal vein. The postoperative outcome was uneventful and the patient received no adjuvant treatment. Six months after the procedure, the patient is doing well with a normal quality of life and without any sign of recurrence. Aggressive and radical surgery should be proposed in first intention for the treatment of portal vein LMS.
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hal-02115614 , version 1 (30-04-2019)

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Elodie Gaignard, Damien Bergeat, Nathalie Stock, Fabien Robin, Karim Boudjema, et al.. Portal vein leiomyosarcoma A rare case of hepatic hilar tumor with review of the literature. Indian Journal of Cancer, 2019, 56 (1), pp.83-85. ⟨10.4103/ijc.IJC_199_18⟩. ⟨hal-02115614⟩
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