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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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Contributor : Laurent Jonchère Connect in order to contact the contributor
Submitted on : Tuesday, April 30, 2019 - 1:37:38 PM
Last modification on : Wednesday, November 3, 2021 - 9:06:26 AM



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, Medknow Publications, 2019, 56 (1), pp.83-85. ⟨10.4103/ijc.IJC_199_18⟩. ⟨hal-02115614⟩



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