Hollenbeck Scott T, Grobmyer Stephen R, Kent K Craig, Brennan Murray F
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
J Am Coll Surg. 2003 Oct;197(4):575-9. doi: 10.1016/S1072-7515(03)00433-2.
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.
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.
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.
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.
下腔静脉（IVC）是原发性软组织肉瘤的罕见发病部位。关于下腔静脉原发性软组织肉瘤的手术治疗及这些患者的长期生存率，文献中的数据有限。
1982年至2002年，共有25例原发性下腔静脉平滑肌肉瘤患者在纪念斯隆-凯特琳癌症中心作为住院患者接受治疗，并纳入前瞻性数据库进行随访。评估了每位患者的临床表现、肿瘤特征、手术治疗、术后并发症及疾病特异性生存率。
这25例原发性下腔静脉平滑肌肉瘤患者占同期接受治疗的所有成年软组织肉瘤患者的0.5%。患者中位年龄为56岁（范围41至79岁）。最常见的三种临床表现为腹痛（52%）、腹胀（20%）和深静脉血栓形成（12%）。其中21例（84%）患者肿瘤得以完全切除。下腔静脉的处理方式有三种：结扎（n = 11）、一期/补片修复（n = 8）和膨体聚四氟乙烯人工血管移植（n = 2）。在下腔静脉结扎和一期/补片修复的患者中（n = 19），11%出现严重术后水肿，且无肾功能恶化情况。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3年和5年疾病特异性生存率分别为76%和33%。未完全切除的患者中无3年生存者。
原发性下腔静脉平滑肌肉瘤的完全切除是可行的，且与生存率提高相关。下腔静脉可通过一期修复或结扎处理，术后发生严重水肿的风险较低。
