Portal vein leiomyosarcoma: a case report and review of the literature.
BMC Surg
; 16(1): 60, 2016 Sep 01.
Article
em En
| MEDLINE
| ID: mdl-27580598
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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Veia Porta
/
Neoplasias Vasculares
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Hepatectomia
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Leiomiossarcoma
Tipo de estudo:
Etiology_studies
Limite:
Aged
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Female
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Humans
Idioma:
En
Ano de publicação:
2016
Tipo de documento:
Article