Portal vein leiomyosarcoma: a case report and review of the literature

Abstract

BackgroundLeiomyosarcoma 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 PresentationWe 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.ConclusionVascular 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.

Primary hepatic leiomyosarcoma presenting as a thick-walled cystic mass resembling a liver abscess

“…Hepatic leiomyosarcoma patients show common symptoms of abdominal pain, weight loss, appetite loss, and weakness, [3–8] and uncommon symptoms of fever, abdominal distension, back pain, and jaundice. [4,5,9,10] Hepatic leiomyosarcoma originating from the hepatic vein may lead to the Budd–Chiari syndrome owing to the obvious space-occupying effect of large tumors.…”

“…Laboratory examinations, including liver function and plasma indexes, showed no obvious abnormality. The levels of plasma tumor markers were also within normal ranges, [3–10] except for 1 case with elevated CEA as a result of the combined colorectal cancer. [11]…”

“…[9,12] And routine CT scans of hepatic leiomyosarcomas can display either well- or ill-defined hypodense masses, that generally are necrotic and occasionally show bleeding or heterogeneous enhancement. [3,6–11,13,15,16] In rare instances, hepatic leiomyosarcomas present as cystic masses with internal or marginal solid enhancement. [12,14,16,17] Magnetic resonance imaging of the tumor may reveal lobulated masses with hypo-intensity on T1-weighted imaging, heterogeneous hyperintensities on T2-weighted imaging, high signal on diffusion-weighted imaging, heterogeneous enhancement, and occasional capsules.…”

“…Needle biopsies can be used to obtain tissue samples for making definitive diagnoses of hepatic leiomyosarcomas, but the actions involved in taking the biopsy are linked with the risk of tumor cell implantation. [3,5,7,10,11] Hepatic leiomyosarcomas are known for having poor prognoses and short survival times because of easy recurrence and metastases.…”

“…[2] Unlike the common hepatocellular carcinoma (HCC) that originate in hepatocytes, hepatic leiomyosarcomas derive from smooth muscle cells in hepatic vessels, bile ducts, or ligaments. [3]…”