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Portal vein leiomyosarcoma: a case report and review of the literature
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 presen...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5007823/ https://www.ncbi.nlm.nih.gov/pubmed/27580598 http://dx.doi.org/10.1186/s12893-016-0174-x |
Sumario: | 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. |
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