Leiomyosarcoma of the inferior vena cava. Report of two cases and review of the literature

Abstract

Introduction: Leiomyosarcoma (LMS) occurs most frequently in the inferior vena cav (IVC). Since Perl's first description in 1871, about 300 cases of IVX have been reported in English literature. The Authors present their personal experience of two case of IVC leiomyosarcoma treated in their Institution.

Patients and methods: Clinical and pathologic data, surgical management, ourcomes and follow-up of two patients admitted nb the Authors' Division respectively on February and November 2004 were collected.

Results: There were a 49-years-old female with rare epigastric pain and a 42-years-old female with one-month history of abdominal discomfort and weight loss. After preoperative evaluation, both of the patients had localised and resectable tumors and underwent radical surgical excision, with prosthetic replacement of the IVC in the first patient and ligation of the IVC in the other one. The first patient is still alive but in an advanced disease. The second patient died of recurrence at seven months.

Discussion: LMS of IVC is a rare tumor. The diagnosis is often delayed because of non-specificic symptoms. Radical resection with surgical margins free of tumor is the treatment of choice. The extent of venous resection does not seem to affect the survival. The type of IVC reconstruction is based on the extent of venal wall involvement. The prognosis is often poor because of tumor recurrence and/or metastatic disease. The role of chemo and radio therapy is not clear yet.

Conclusion: Long term survival is related to an extensive surgery, being surgical margins free of tumor the only prognostic factor with significant influence on patients survival.