Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Tiantan Xili No. 6, Beijing, 100050, China.
Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
Neurosurg Rev. 2020 Jun;43(3):911-921. doi: 10.1007/s10143-019-01089-1. Epub 2019 Feb 27.
The incidence of malignant transformation of fibrous dysplasia (FD) is very rare. Thus, the available knowledge of its characteristics, management, and survival is scarce. Here, we present a systemic review of fibrous dysplasia that had undergone malignant transformation. A comprehensive search was performed on PubMed electronic database. The survival rates and hazard ratios of age, gender, past history of previous radiotherapy, type of FD, and treatment were collected from the published articles and analyzed. Forty-eight cases were eligible for inclusion in the study. Patient's age, gender, past history radiotherapy, and type of FD did not influence the overall survival (OS). The Kaplan Meier analysis showed that the patients who had not received any treatment had poor prognosis with a median survival of 4 months. The patients that received surgery had significantly longer OS than that in the biopsy group. The prognosis of malignant transformation of FD is relatively poor, and surgery is the optimal treatment of choice. Nevertheless, the efficacy of postoperative adjuvant therapy to patient OS is still undefined.
纤维结构不良（FD）恶变的发生率非常罕见。因此，其特征、治疗和生存情况的相关知识还很匮乏。在此，我们对纤维结构不良恶变进行了系统综述。我们在 PubMed 电子数据库中进行了全面检索。从已发表的文章中收集了患者年龄、性别、既往放疗史、FD 类型和治疗对生存率和风险比的影响，并进行了分析。共有 48 例符合纳入标准。患者年龄、性别、既往放疗史和 FD 类型均不影响总生存率（OS）。Kaplan-Meier 分析显示，未接受任何治疗的患者预后较差，中位生存期为 4 个月。接受手术治疗的患者 OS 明显长于活检组。纤维结构不良恶变的预后较差，手术是最佳治疗选择。然而，术后辅助治疗对患者 OS 的疗效仍不确定。
