Department of Surgical Pathology, Northwestern Memorial Hospital, Chicago, IL 60611, USA.
Am J Surg Pathol. 2010 Jun;34(6):873-81. doi: 10.1097/PAS.0b013e3181ddf569.
This report details the clinicopathologic features and follow-up data on 40 cases of inferior vena cava leiomyosarcoma, a rare sarcoma with a poor prognosis. Study cohort consisted of 31 females and 9 males (mean age, 53 y), whose material was accessioned to the Armed Forces Institute of Pathology between 1976 and 2008. Inferior vena cava leiomyosarcomas ranged in size from 3.5 to 15.0 (median, 8.5) cms, and most involved the middle segment of the vessel and grew extraluminally. Eleven leiomyosarcomas were French Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) histologic grade I; 21, grade II; and 5 were grade III. Eleven of 33 patients managed by complete or radical resection had involved surgical margins. Twenty of the 34 patients (59%) with clinical follow-up data (mean, 33.5; median, 51 mo) died of sarcoma-related complications and 9 (26%) of unknown causes. The 5-year and 10-year survival rates after resection without documented residual macroscopic disease were 50% and 22%, respectively. Two patients are alive without disease 9 and 18 years after last surgical intervention. Suprahepatic vena caval and right atrial involvement by tumor, predominant intraluminal tumor growth, and residual postsurgical macroscopic disease were factors that statistically correlate with death within 2 years. By univariate analysis, intraluminal tumor (P=0.03), liver injury or failure (compromised liver) (P=0.01), and moderate to poor tumor differentiation (P=0.03) were associated with increased tumor-related mortality, whereas a compromised liver (P=0.01) was the only factor correlated with mortality by multivariate analysis. Our study concludes that a macroscopic resection of localized inferior vena cava leiomyosarcoma provides the best chance for long-term survival, suprahepatic tumors often result in early death, and a compromised liver correlates with overall poor survival, but French Federation Nationale des Centres de Lutte Contre le Cancer grading does not affect prognosis.
本报告详细介绍了 40 例下腔静脉平滑肌肉瘤的临床病理特征和随访数据，这是一种预后不良的罕见肉瘤。研究队列包括 31 名女性和 9 名男性（平均年龄 53 岁），其标本于 1976 年至 2008 年期间被收录于美国武装部队病理学研究所。下腔静脉平滑肌肉瘤的大小范围为 3.5 至 15.0（中位数为 8.5）cm，大多数累及血管的中段并向腔外生长。11 例平滑肌肉瘤为法国国家癌症中心联合会（FNCLCC）组织学分级 I；21 例为 II 级；5 例为 III 级。33 例接受完全或根治性切除术的患者中，有 11 例手术切缘受累。34 例有临床随访数据（平均随访时间 33.5 个月，中位数随访时间 51 个月）的患者中，20 例（59%）死于肉瘤相关并发症，9 例（26%）死因不明。无残留肉眼疾病的切除术后 5 年和 10 年生存率分别为 50%和 22%。最后一次手术干预后 9 年和 18 年无疾病生存的患者有 2 例。肿瘤累及肝上腔静脉和右心房、肿瘤主要腔内生长和残留术后肉眼疾病是 2 年内死亡的统计学相关因素。单因素分析显示，腔内肿瘤（P=0.03）、肝损伤或衰竭（受损肝脏）（P=0.01）和中-差肿瘤分化（P=0.03）与肿瘤相关死亡率增加相关，而受损肝脏（P=0.01）是多因素分析中与死亡率相关的唯一因素。本研究表明，局限性下腔静脉平滑肌肉瘤的大体切除术为长期生存提供了最佳机会，肝上肿瘤常导致早期死亡，受损肝脏与整体预后不良相关，但法国国家癌症中心联合会分级不影响预后。
