Extra Luminal Leiomyosarcoma of Inferior Venacava: a Hepatobiliary Surgeon Perspective

Excerpt

Inferior venacava (IVC) leiomyosarcoma (LMS) is a rare, but most common malignant vascular tumor originating in the smooth muscle of the vein wall [1]. The incidence is 1 per 100,000 cases per annum [2]. The entity was first described in 1871, but successful resection and ligation of IVC was done only in 1951, approximately a decade later [3]. Due to its rarity, the disease poses a unique surgical challenge in diagnosis and management. Frequently, a hepatobiliary surgeon may encounter this rare entity and be unable to differentiate from duodenal gastrointestinal stromal tumor, caudate lobe hepatocellular carcinoma, or paraganglioma [2, 4]. Hence, it requires multidisciplinary planning and management involving surgical oncologists, radiologists, urologists, radiation oncologists, and cardiovascular and hepatobiliary surgeons [5]. Here, we describe a rare case of extra luminal leiomyosarcoma of infra renal IVC managed successfully by focal resection and primary repair. …