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) , B. Meunier (1, 2)

1 CHU Pontchaillou [Rennes]
2 CIC - Centre d'Investigation Clinique [Rennes]

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: liver resection; portal vein; Leiomyosarcoma

Domains: Life Sciences [q-bio]

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⟩