Leiomyosarcoma of the inferior vena cava. Clinical observation

Abstract

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor observed in 2 % of all leiomyosarcomas. Less than 400 cases are described in the literature, in Russian publications not more than 30. Computed tomography is the most accurate diagnostic method for determination of tumor location, presence or absence of thrombosis and its severity, evaluation of resectability. We present a case of a patient with leiomyosarcoma of the IVC with preoperative diagnosis of adrenocortical carcinoma of the right adrenal gland with tumor thrombosis of the IVC and tumor advancement into the right kidney. During intraoperative revision the tumor was evaluated as primary leiomyosarcoma of the IVC with thrombosis of the iliac veins up to the retrohepatic segment of the IVC and advancement into the right adrenal gland and vascular structures of the right kidney and its pole which was confirmed by histological examination. Surgery is the main method of treatment of leiomyosarcoma of the IVC. Long-term results depend on the volume and radicality of operation. Reconstruction of the IVC is not always necessary. Despite high recurrence rate, there is no consensus on adjuvant treatment. The best results are achieved through interdisciplinary approach involving an oncological surgeon, chemotherapist, vascular surgeon, anesthesiologist. Perioperative planning, coordination, and adherence to oncological methods are crucial for recovery.