■毛细血管扩张骨肉瘤(TOS),一种罕见的骨肉瘤变种,易误诊为动脉瘤样骨囊肿(ABC)。这项研究的目的是通过回顾我们对TOS的经验来研究TOS的诊断和预后因素,并建立可以区分TOS和ABC的诊断模型。
■我们确定了2001年3月至2016年1月在中山大学附属第一医院治疗的51例TOS,并回顾了他们的记录,影像学信息和病理研究。通过Bayes判别分析建立了区分TOS和ABC的诊断模型并进行了评估。采用对数秩检验分析TOS的预后因素,比较TOS与其他高级别骨肉瘤亚型的预后差异。
■采用多学科诊断方法,结合临床,成像,病理研究提高了诊断的准确性。与ABC患者相比,TOS患者中年龄在18岁或以下且病理性骨折更为常见(分别为P=.004和.005)。平均白细胞(WBC),血小板,乳酸脱氢酶(LDH),TOS患者的碱性磷酸酶(ALP)值高于ABC患者(分别为P=.002,.003,.007和.007)。我们的诊断模型,包括上述因素,准确预测了训练和验证集中62%和78%的TOS患者,分别。TOS患者的5年无事件生存率和总生存率分别为52.5±9.4%和54.9±8.8%。分别,与其他骨肉瘤亚型患者相似(分别为P=.950和.615)。肿瘤体积和LDH水平是预测预后因素(P=.040和.044),但不是病理性骨折或误诊的存在(分别为P=.424和.632)。
■基于预测因素的多学科诊断方法和诊断模型,即,年龄,病理性骨折的存在,和血小板,LDH,ALP和WBC水平,有助于TOS和ABC的区分。较小的肿瘤和正常的LDH水平与更好的预后相关。
OBJECTIVE: Telangiectatic osteosarcoma (TOS), a rare variant of osteosarcoma, may be easily misdiagnosed as aneurysmal bone cyst (ABC). The aims of this study were to investigate the diagnostic and prognostic factors of TOS by reviewing our experience with TOS and to develop a diagnostic model that may distinguish TOS from ABC.
METHODS: We identified 51 cases of TOS treated at the First Affiliated Hospital of Sun Yat-Sen University from March 2001 to January 2016 and reviewed their records, imaging information and pathological studies. A diagnostic model was developed to differentiate TOS and ABC by Bayes discriminant analysis and was evaluated. The log-rank test was used to analyze the prognostic factors of TOS and to compare the outcome differences between TOS and other high-grade osteosarcoma subtypes.
RESULTS: The multi-disciplinary diagnostic method employed that combined clinical, imaging, and pathological studies enhanced the diagnostic accuracy. Age 18 years or younger and pathologic fracture were more common among the TOS patients than among the ABC patients (P = .004 and .005, respectively). The average white blood cell (WBC), platelet, lactate dehydrogenase (LDH), and alkaline phosphatase (ALP) values of the TOS patients were higher than those of the ABC patients (P = .002, .003, .007, and .007, respectively). Our diagnostic model, including the aforementioned factors, accurately predicted 62% and 78% of the TOS patients in the training and validation sets, respectively. The 5-year estimates of event-free survival and overall survival of the TOS patients were 52.5 ± 9.4% and 54.9 ± 8.8%, respectively, which were similar to those of patients with other osteosarcoma subtypes (P = .950 and .615, respectively). Tumor volume and the LDH level were predictive prognostic factors (P = .040 and .044) but not the presence of pathologic fracture or misdiagnosis (P = .424 and .632, all respectively).
CONCLUSIONS: The multi-disciplinary diagnostic method and diagnostic model based on predictive factors, i.e., age, the presence of pathologic fracture, and platelet, LDH, ALP and WBC levels, aided the differentiation of TOS and ABC. Smaller tumors and normal LDH levels were associated with better outcomes.