Toronto Sarcoma Program at Mount Sinai Hospital, Toronto, Canada.
Princess Margaret Cancer Centre, Toronto, Canada.
Cancer Med. 2020 Dec;9(24):9282-9292. doi: 10.1002/cam4.3531. Epub 2020 Oct 16.
Non-osteogenic, non-Ewing soft-tissue sarcoma (NONE-STS) of bone is a rare presentation of primary bone cancers. Optimal treatments and outcomes for this heterogenous group are poorly described. We evaluated the factors associated with long-term outcomes in patients with this disease. Patients with localized NONE-STS of bone treated at the Toronto Sarcoma Program from 1987 to 2017 were identified. Clinical characteristics, treatment, and survival information were collected. Kaplan-Meier (log-rank) survival estimates from the time of definitive surgery, with uni-/multivariate analyses (Cox) of sarcoma-specific survival were performed. A total of 106 patients (60.4% male; median age 46 years) with NONE-STS of bone were identified. Common histologies included undifferentiated pleomorphic sarcoma [UPS]/malignant fibrous histiocytoma [MFH] (UPS/MFH, 41.5%), leiomyosarcoma (LMS, 20.8%), and fibrosarcoma (FS, 11.3%). Tumors were often high grade (59.4%) and involved the extremities (88.7%), with most receiving chemotherapy (67.9%) with cisplatin/doxorubicin-based regimens (73.6%). In the full cohort, 10-year DFS (45.7%, [95%CI: 35.7-55.8%]), OS (53.4%, [95%CI: 41.7-62.2%]), and SSS (63.9%, [95%CI: 53.9-72.5%]) were moderate. Histology specific, 10-year SSS was 70.7% [95%CI: 56.1-85.5%] for UPS/MFH, 51.8% [95%CI: 29.8-73.8%] for LMS, and 72.2% [95%CI: 45.1-99.2%] for FS. Only UPS/MFH (n = 4) showed sarcoma-related death >10 years. Multivariate analysis identified axial location (HR = 35.5, [95%CI: 3.4-369.6]), high grade (HR = 16.9, [95%CI: 1.6-185.1]), and disease relapse (HR = 485.1, [95%CI: 36.3-6482.6]) as risk factors for death (p < 0.05). Treatment with chemotherapy (HR = 0.1, [95%CI: 0.01-0.86]) and necrosis ≥85% (HR = 0.2, [95%CI: 0.04-0.99]) showed improved survival (p < 0.05). NONE-STS of bone has favorable long-term survival similar to osteosarcoma. Patients receiving chemotherapy derive benefit in retrospective analyses. UPS/MFH histologies show sarcoma-related death beyond 10 years. Further data on histologic subgroups are needed.
非成骨性、非尤文氏软组织肉瘤（NONE-STS）是一种罕见的原发性骨癌表现。对于这种异质性群体，最佳治疗方法和结果描述得很差。我们评估了与该疾病患者长期结果相关的因素。从 1987 年到 2017 年，在多伦多肉瘤计划治疗的局部 NONE-STS 患者被确定。收集了临床特征、治疗和生存信息。从明确手术时间开始，通过单变量/多变量分析（Cox）进行肉瘤特异性生存的 Kaplan-Meier（对数秩）生存估计。共确定了 106 例 NONE-STS 患者（60.4%为男性；中位年龄 46 岁）。常见的组织学包括未分化多形性肉瘤[UPS]/恶性纤维组织细胞瘤[MFH]（UPS/MFH，41.5%）、平滑肌肉瘤（LMS，20.8%）和纤维肉瘤（FS，11.3%）。肿瘤通常为高级别（59.4%），并累及四肢（88.7%），大多数患者接受了含顺铂/多柔比星方案的化疗（67.9%）（73.6%）。在全队列中，10 年DFS（45.7%，[95%CI：35.7-55.8%]）、OS（53.4%，[95%CI：41.7-62.2%]）和 SSS（63.9%，[95%CI：53.9-72.5%]）为中等水平。特定组织学的 10 年 SSS 为 UPS/MFH 为 70.7%[95%CI：56.1-85.5%]，LMS 为 51.8%[95%CI：29.8-73.8%]，FS 为 72.2%[95%CI：45.1-99.2%]。只有 UPS/MFH（n=4）显示肉瘤相关死亡超过 10 年。多变量分析确定了轴向位置（HR=35.5，[95%CI：3.4-369.6]）、高级别（HR=16.9，[95%CI：1.6-185.1]）和疾病复发（HR=485.1，[95%CI：36.3-6482.6]）是死亡的危险因素（p<0.05）。化疗（HR=0.1，[95%CI：0.01-0.86]）和坏死≥85%（HR=0.2，[95%CI：0.04-0.99]）的治疗显示出生存改善（p<0.05）。NONE-STS 的骨有类似于骨肉瘤的良好长期生存。接受化疗的患者在回顾性分析中获益。UPS/MFH 组织学显示肉瘤相关死亡超过 10 年。需要进一步的组织学亚组数据。
