HPB P02 Leiomyosarcoma of IVC

Abstract

Abstract Background Leiomyosarcoma of the inferior vena cava (IVC) is a very rare entity with poor 5-year prognosis. Less than 400 cases have been reported in literature. The extent of mass forming lesions is quite progressed upon discovery and multiorgan resection is often required. Here we present our 5year experience and surgical management of this malignancy. Methods From 2000 - 2023, 7 cases with IVC Leiomyosarcoma were treated in our institution. The median patient age was 59 years old with female gender predominance involvement. All patients presented with vague abdominal symptoms prompted imaging studies discovering the tumour, or the latter was an incidental finding of a retroperitoneal mass involving the IVC and adjacent organs (liver, kidney, duodenum). Only 4 patients (56%) had preoperative CT guided biopsy confirming the diagnosis and 3 cases were confirmed from post operative histology. Results All cases were reviewed in our multidisciplinary meeting and 4 patients (56 %) underwent surgical resection with caval replacement using a graft apart from one case which underwent IVC ligation. Liver resection was required after portal flow manipulation in two cases. Postoperative complications were ascites and chyle leak which were managed conservatively. Resection margins were negative (R0) to all cases. One case recurred and underwent further liver resections. Chemotherapy was offered only to unresectable cases (42%). From resected patients three of them (42%) are still alive with longest follow up of 4.5years post-resection and one died after multiple resections. Conclusions IVC Leiomyosarcoma is an aggressive malignancy and requires major surgery upon diagnosis. Surgery is the gold standard treatment since unrespectability upon diagnosis has poor prognosis. Due to rarity of the disease, large studies are difficult to be conducted.