Teixeira Frederico José Ribeiro, do Couto Netto Sergio Dias, Perina André Luis de Freitas, Torricelli Fabio C M, Ragazzo Teixeira Luciana, Zerati Antonio Eduardo, Ferreira Fabio de Oliveira, Akaishi Eduardo Hiroshi, Nahas William Carlos, Utiyama Edivaldo Massazo
Sarcoma and Melanoma Surgery Unit, São Paulo Cancer Institute, University of São Paulo Medical School, Cerqueira César, SP 05403-000, Brazil.
Department of General Surgery, Clinics Hospital, University of São Paulo Medical School, Cerqueira César, SP 05403-000, Brazil.
Oncol Lett. 2017 Oct;14(4):3909-3916. doi: 10.3892/ol.2017.6706. Epub 2017 Aug 2.
Leiomyosarcoma (LMS) of inferior vena cava (IVC) is a rare neoplasm affecting approximately 1/100,000 people. The prognosis is poor and potential curative intent occurs through challenging operations, such as vena cava resection, occasionally multivisceral when required, and vascular reconstruction. There are few retrospective series regarding this retroperitoneal neoplasm, and the aim of the present study was to discuss the experience at the São Paulo Cancer Institute and Clinics Hospital of University of São Paulo Medical School, São Paulo, Brazil. The current study is a retrospective review of 7 patients treated in the two tertiary hospitals between 2005 and 2013. Oncological and operative aspects were discussed, primarily regarding surgical aspects highlighting resection, vascular reconstruction, and the overall survival and recurrence rates. All the patients were treated with radical intent, 4 of whom underwent multivisceral resection, with the kidney being the most resected organ. The location of the IVC tumor was described using Kulaylat's description and the median tumor size was 10 cm. Vascular reconstruction was necessary in 4 patients. The overall survival rate at 3 and 5 years was 100, and 25%, respectively. The disease-free survival rate at 3 and 5 years was 57 and 20%, respectively. In conclusion, IVC LMS is a rare and severe retroperitoneal neoplasm, with multivisceral resections remaining a surgical challenge. The treatment requires numerous experienced surgeons and the impact of microscopic free margins remains unclear. Vascular reconstruction depends on several aspects regarding primarily the topography of the tumor.
下腔静脉平滑肌肉瘤（LMS）是一种罕见的肿瘤，发病率约为十万分之一。其预后较差，可能的治愈方法是通过具有挑战性的手术，如下腔静脉切除术，必要时偶尔进行多脏器切除，并进行血管重建。关于这种腹膜后肿瘤的回顾性系列研究较少，本研究的目的是讨论巴西圣保罗圣保罗大学医学院癌症研究所和临床医院的经验。本研究是对2005年至2013年间在两家三级医院接受治疗的7例患者的回顾性分析。讨论了肿瘤学和手术方面的问题，主要强调手术方面，包括切除、血管重建以及总生存率和复发率。所有患者均接受根治性治疗，其中4例接受了多脏器切除，肾脏是切除最多的器官。下腔静脉肿瘤的位置采用库莱拉特的描述方法，肿瘤中位大小为10厘米。4例患者需要进行血管重建。3年和5年的总生存率分别为100%和25%。3年和5年的无病生存率分别为57%和20%。总之，下腔静脉LMS是一种罕见且严重的腹膜后肿瘤，多脏器切除仍然是一项手术挑战。治疗需要众多经验丰富的外科医生，显微镜下切缘阴性的影响仍不明确。血管重建主要取决于肿瘤的位置等多个方面。
