Gusho Charles A, Tepper Sarah C, Gitelis Steven, Blank Alan T
Department of Orthopedic Surgery, Division of Orthopedic Oncology, Rush University Medical Center; and Midwest Orthopaedics at Rush, Chicago, IL, USA.
Rare Tumors. 2021 Apr 11;13:20363613211005593. doi: 10.1177/20363613211005593. eCollection 2021.
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that may arise in bone. The purpose of this investigation was to determine the clinicopathological features and outcomes of osseous EHE in a large patient series, and to assess whether survival is impacted by demographics, tumor characteristics, or treatment factors. This was a retrospective review of the Surveillance, Epidemiology and End Results (SEER) database from 1992 to 2016. Kaplan-Meier was used to estimate overall survival (OS) and disease-specific survival (DSS). A Cox regression model was used to identify prognostic factors. Fifty patients from 1992 to 2016 with a median age of 54.5 years (IQR, 37-67) were reviewed. For location, 46% ( = 23) of tumors arose from the appendicular skeleton while 38% ( = 19) occurred within the axial skeleton (overlapping EHE: 16%, = 8). Of the cases with recorded treatment factors, 54.8% ( = 23) had surgery, 26% ( = 13) received radiation, 22% ( = 11) were treated with chemotherapy, and 26% ( = 13) had surgery plus radiation. The 5-year OS probability was 49.2% (95% CI, 23.6-70.6), and the 5-year DSS probability was 63.9% (95% CI, 33.0-83.5). No surgery (surgery: HR, 0.262; 95% CI, 0.07-0.9); = 0.041) and age older than 50 years (HR, 4.117; 95% CI, 1.1-15.4; = 0.035) were negative prognostic factors of disease-specific mortality after controlling for confounding variables. There was no association between disease-specific mortality and adjuvant or multimodal therapy. The prognosis of EHE of bone is less than favorable, and the 5-year DSS probability of 64% emphasizes the intermediate grade nature of this tumor subtype. Surgical treatment, when feasible, is associated with a better prognosis.
上皮样血管内皮瘤（EHE）是一种罕见的血管肿瘤，可发生于骨骼。本研究的目的是确定一大组患者中骨EHE的临床病理特征和预后，并评估生存是否受人口统计学、肿瘤特征或治疗因素的影响。这是一项对1992年至2016年监测、流行病学和最终结果（SEER）数据库的回顾性研究。采用Kaplan-Meier法估计总生存期（OS）和疾病特异性生存期（DSS）。使用Cox回归模型确定预后因素。回顾了1992年至2016年的50例患者，中位年龄为54.5岁（IQR，37 - 67岁）。就肿瘤位置而言，46%（n = 23）的肿瘤发生于四肢骨骼，而38%（n = 19）发生于中轴骨骼（重叠EHE：16%，n = 8）。在记录了治疗因素的病例中，54.8%（n = 23）接受了手术，26%（n = 13）接受了放疗，22%（n = 11）接受了化疗，26%（n = 13）接受了手术加放疗。5年OS概率为49.2%（95%CI，23.6 - 70.6），5年DSS概率为63.9%（95%CI，33.0 - 83.5）。未接受手术（手术：HR，0.262；95%CI，0.07 - 0.9；P = 0.041）和年龄大于50岁（HR，4.117；95%CI，1.1 - 15.4；P = 0.035）是控制混杂变量后疾病特异性死亡的负性预后因素。疾病特异性死亡与辅助治疗或多模式治疗之间无关联。骨EHE的预后欠佳，5年DSS概率为64%，强调了该肿瘤亚型的中级性质。可行时，手术治疗与较好的预后相关。
