Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma
Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma
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DOI：
10.1016/s1072-7515(03)00433-2
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发表时间：
2003-10-01
影响因子：
5.2
通讯作者：
Brennan, MF
中科院分区：
文献类型：
--
作者：
Hollenbeck, ST;Grobmyer, SR;Brennan, MF
背景：下腔静脉（IVC）是原发性软组织肉瘤的罕见部位。文献中关于下腔静脉的手术治疗和这些患者的长期生存的数据有限。研究设计：从1982年到2002年，共有25例原发性下腔静脉平滑肌肉瘤患者作为住院患者接受治疗，并在Memorial Sloan-Kettering的前瞻性数据库中进行随访。评估每位患者的症状、肿瘤特征、手术处理、术后发病率和疾病特异性生存率。结果：25例原发性下腔静脉平滑肌肉瘤患者占同期治疗的所有成年软组织肉瘤患者的0.5%。患者年龄中位数为56岁（41 - 79岁）。三种最常见的症状是腹痛（52%）、腹胀（20%）和深静脉血栓形成（12%）。其中21例（84%）接受了肿瘤的完全切除。IVC采用三种方法之一进行治疗：结扎（n = 11），初级/补片修复（n = 8）和膨胀聚四氟乙烯管移植（n = 2）。在接受下腔静脉结扎和初级/补片修复的患者（n = 19）中，11%出现严重的术后水肿，没有肾功能恶化。33%的患者出现局部复发，48%的患者出现远处复发。接受完全切除的患者的3年和5年疾病特异性生存率分别为76%和33%。不完全切除的患者中没有3年存活者。结论：完全切除原发性下腔静脉平滑肌肉瘤是可行的，并可提高生存率。下腔静脉可以通过初级修复或结扎来处理，术后严重水肿的风险很低。(C) 2003年由美国外科医师学会发布。
BACKGROUND: The inferior vena cava (IVC) is a rare site for primary soft tissue sarcoma. There are limited data in the literature regarding surgical management of the IVC and longterm survival of these patients.STUDY DESIGN: From 1982 to 2002, a total of 25 patients with primary IVC leiomyosarcoma was treated as inpatients and followed in a prospective database at Memorial Sloan-Kettering. Presenting symptoms, tumor characteristics, operative management, postoperative morbidity, and disease-specific survival were assessed for each patient.RESULTS: The 25 patients with primary IVC leiomyosarcoma accounted for 0.5% of all adult patients with soft tissue sarcoma treated during this time. The median patient age was 56 years (range 41 to 79 years). The three most common presenting symptoms were abdominal pain (52%), distention (20%), and deep venous thrombosis (12%). Of the patients, 21 (84%) underwent complete resection of the tumor. The IVC was managed in one of three ways: ligation (n = 11), primary/patch repair (n = 8), and expanded polytetrafluoroethylene tube grafting (n = 2). Among patients undergoing IVC ligation and primary/patch repair (n = 19), 11% had severe postoperative edema and none had worsening renal function. Local recurrence occurred in 33% of patients and distant recurrence occurred in 48% of patients. Patients undergoing complete resection had 3-year and 5-year disease-specific survival rates of 76% and 33%, respectively. There were no 3-year survivors among patients with incomplete resections.CONCLUSIONS: Complete resection of primary IVC leiomyosarcomas is feasible and associated with improved survival. The IVC can be managed by primary repair or ligation with a low risk of severe postoperative edema. (C) 2003 by the American College of Surgeons.