Retroperitoneal leiomyosarcoma originating from the inferior vena cava: A case report and literature review

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Leiomyosarcoma of inferior vena cava is scarce retroperitoneal tumor with a poor prognosis. It is usually a misdiagnosed condition due to unspecific clinical course. Imaging is a sensitive tool in establishing the accurate preoperative diagnosis and determining the extent of the tumor. However, tumors may mimic other retroperitoneal conditions making the diagnosis challenging. Herein, we encountered a rare case of leiomyosarcoma of the inferior vena cava in a 69-year-old woman for whom the primary diagnosis was different from the pathological one and the tumor appeared in preoperative imaging as a gastrointestinal tumor. The presence intraoperatively of intimate attachments with the inferior vena cava wall raised the suspicion of vascular origin. The tumor was removed in bloc with a segment of inferior vena cava. Pathological findings led to a further diagnosis of the tumor as a leiomyosarcoma originating from the inferior vena cava. Complete surgical resection is the cornerstone of treatment. The prognosis depends directly on free tumor margins and early diagnosis. Knowing this rare entity is mandatory to assess the correct diagnosis, achieve adequate therapeutic management and improve patient’s outcome.