Portal vein leiomyosarcoma: a case report and review of the literature. Issue 1 (December 2016)

• Record Type: Journal Article
• Authors: Gohrbandt, Antje; Hansen, Torsten; Ell, Christian; Heinrich, Stefan; Lang, Hauke
• Is Part Of: BMC surgery. Volume 16:Issue 1(2016)
• Page Start: 1
• Page End: 4
• Publication Date: 2016-12
• Subjects: Leiomyosarcoma -- Portal vein -- Surgery -- Review; Surgery -- Periodicals; 617.005
• DOI: 10.1186/s12893-016-0174-x
• Languages: English
• ISSNs: 1471-2482

Abstract

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. Case Presentation 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. Conclusion 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.