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

Elodie Gaignard 1, 2 Damien Bergeat 1, 2 Nathalie Stock 1 Fabien Robin 1, 2 Karim Boudjema 1, 2 Laurent Sulpice 1, 2 Michel Rayar 1, 2 Bernard Meunier 1, 2
1 CHU Pontchaillou [Rennes]
2 CIC - Centre d'Investigation Clinique [Rennes]
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.
Keywords : portal vein Leiomyosarcoma liver resection
Document type :
Journal articles
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https://hal-univ-rennes1.archives-ouvertes.fr/hal-02115614
Contributor : Laurent Jonchère <>
Submitted on : Tuesday, April 30, 2019 - 1:37:38 PM
Last modification on : Monday, May 13, 2019 - 4:00:16 PM

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UNIV-RENNES1 | CIC | CIC203 | BIOSIT | UR1-UFR-SVE | UNIV-RENNES

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

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