title: Portal vein leiomyosarcoma: a case report and review of the literature.
abstract: BACKGROUND
  Leiomyosarcoma of vascular smooth muscle is a very rare entity. A fair number of cases of vascular leiomyosarcomas have been reported, and the vast majority of these tumors arose from the inferior vena cava.
  
  
  METHODS
  We report the case of a 71-year-old female patient who presented with recurrent upper abdominal pain. A CT-scan demonstrated a heterogenous mass in the liver hilum. Liver function tests and hematology parameters as well as the tumor markers were normal. Due to the unclear diagnosis a percutaneous biopsy of this mass was performed and revealed leiomyosarcoma. The patient was treated by a right sided hemihepatectomy with portal vein reconstruction and an end-to-side hepatico-jejunostomy. Final histology confirmed complete (R0) resection of a moderately differentiated leiomyosarcoma of the portal vein. After complete (R0) resection of the lesion, the patient remained without any signs of tumor recurrence for a total of 36 months until detection of an unresectable local recurrence. After surgical re-exploration the patient was finally referred to palliative radiotherapy.
  
  
  CONCLUSIONS
  Vascular leiomyosarcoma of the portal vein is an extremely rare tumor entity. We have described a case with no evidence-based neo/adjuvant treatment options, where aggressive surgery achieved a tumor-free margin (R0), performed in a specialized center for sarcoma and hepatobiliary surgery.
authors: Department of General, Visceral and Transplantation Surgery, University Hospital of the Johannes Gutenberg University Mainz, Mainz, Germany. antje.gohrbandt@unimedizin-mainz.de.
authors: Antje E
authors: AE
authors: Gohrbandt
authors: Department of Pathology, University Hospital of the Johannes Gutenberg University Mainz, Mainz, Germany.
authors: Torsten
authors: T
authors: Hansen
authors: Department of Internal Medicine II/IV, Sana Klinikum, Offenbach, Germany.
authors: Christian
authors: C
authors: Ell
authors: Department of General, Visceral and Transplantation Surgery, University Hospital of the Johannes Gutenberg University Mainz, Mainz, Germany.
authors: Stefan S
authors: SS
authors: Heinrich
authors: Department of General, Visceral and Transplantation Surgery, University Hospital of Mainz, Mainz, Germany.
authors: Hauke
authors: H
authors: Lang
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000368:Aged; D000714:Anastomosis, Surgical; D005260:Female; D006498:Hepatectomy; D006801:Humans; D007890:Leiomyosarcoma / Q000175:diagnosis* / Q000601:surgery*; D011169:Portal Vein; D014057:Tomography, X-Ray Computed; D019043:Vascular Neoplasms / Q000175:diagnosis* / Q000601:surgery*
keywords: Leiomyosarcoma; Portal vein; Review; Surgery