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Radiotherapy for the Treatment of Intracranial Solitary Fibrous Tumor

放射治療用於顱內單發性纖維瘤

摘要


Purpose : Solitary fibrous tumor (SFT) is a rare mesenchymal tumor, encompassing tumor previously termed hemangiopericytoma (HPC). The aim of this study is to evaluate both histopathological and clinical factors of intracranial SFT, including tumors previously classified as HPC, and offer an analysis of treatment outcomes based on our experience. Materials & Methods : Between 2002 and 2016, 18 patients with intracranial SFT receiving radiotherapy at our hospital were eligible for this study. Hemangiopericytoma was grouped with grade II SFT, and anaplastic hemangiopericytoma was grouped with grade III SFT since they share similar genetic patterns and clinical prognosis. Clinicopathological and radiotherapy data were extracted from patient’s medical record. Radiotherapy was administered by either conventional fractionated radiotherapy or stereotactic radiosurgery. The planning system includes Varian Medical System and BrainLab system. Results : Reviewing operative records, gross total resection was performed in 14 (77.8%) cases, while the remaining 4 (22.2%) cases had subtotal resection. All patients underwent postoperative RT. Before radiation therapy at our department, ten patients received one operation for SFT, four patients received two operations and the other four patients received more than two operations. Twelve patients received conventional fractionated radiotherapy and 6 received stereotactic radiosurgery. The median dose is 55.5 Gy (range 30 - 60 Gy) for fractionated RT and 16 Gy (range 15 - 16 Gy) for SRS. The median target volume for patients who received fractionated radiotherapy was 123.8 cm^3 (49.1 - 237.0 cm^3) and 5.84 cm^3 (range 3.5 - 29.24 cm^3) for those who received stereotactic radiosurgery. Median follow-up time was 73.6 months. Five and 10 years overall survival rates were 70.5%, and 47.0%, respectively. Tumors reoccurred as intracranial recurrence only (n= 3), and extracranial metastasis alone (n= 4). Local control rate at 5 years was 90.9%. The 5-year metastasis-free rate was 100.0% for grade II (n= 9) tumors and 40.0% for grade III tumors (n= 9) (p= 0.02). There was a trend toward improved progression-free rate in low-grade tumors, with a 5-year progression-free rate of 83.3% for grade II, and 34.3% for grade III tumors (p= 0.09). There was no significant difference for overall survival, progression free rate or local control rates by extent of surgery in this study. Conclusion : For patients with intracranial SFTs, resection remains the initial treatment and postoperative radiotherapy provides an effective and safe adjuvant management option. Histological grading is the most significant prognostic factor.

並列摘要


目的:單發性纖維瘤(solitary fibrous tumor; SFT)是一類罕見的間質腫瘤,它包括了先前稱為血管外皮細胞瘤(hemangiopericytoma; HPC)的腫瘤。本篇研究的目的是在於評估單發性纖維瘤(以及血管外皮腫瘤)的臨床特徵,病理組織特徵以及本院放射治療的經驗與治療結果。材料與方法:在2002至2016年間年間,共有18位病人於放射腫瘤科接受顱內單發性纖維瘤的治療。這兩種名稱(SFT和HPC)在過去被認為是各自獨立的疾病,現在因為它們有一些共通的特殊基因被發現,根據2016年中樞神經系統WHO的新版分類,它們現在被認為是同一種類的疾病。為了分析預後因子,我們將二級的的血管外皮細胞瘤二級單發性纖維瘤歸類為一組,並將分化不良血管外皮細胞瘤與三級單發性纖維瘤歸類至為一組。病人的臨床與病理資料以及放射治療資料採自病歷記錄與治療記錄。放射治療技術包括傳統分次劑量的體外照射與立體定位放射手術。放射治療系統包括Varian Medical System以及BrainLab系統。結果:關於手術切除的程度,14位病人接受全部切除(gross total resection)(77.8%),以及另外4為病人接受了部分切除(subtotal resection)(22.2%)。十位病人在放射治療之前接受過一次外科手術,有四位在放射治療錢接受過兩次手術,而其他四位則接受過多次手術(3-4 次)。所有病人在手術後都有接受放射治療,其中12位是接受分次放射治療,而另6位是接受立體定位放射手術。對於分次治療的病人,放射劑量中位數是55.5 Gy(範圍30-60 Gy),而對於立體定位放射手術的病人,放射劑量中位數是16 Gy(範圍15 -16 Gy)。放射治療的治療靶區體積,對於分次治療的病患治療靶區體積中位數是 123.8 立方公分(範圍49.1-237.0),而立體定位放射手術則是5.84立方公分(範圍 3.5-29.24)。研究追蹤時間中位數為73.6個月,五年與十年總體存活率分別為70.5%與47.0%。有局部顱內復發的病人有3人,顱外遠端轉移的病人有4位,五年局部控制率是90.9%。病理分類上,二級腫瘤(n= 9)的遠端轉移機率較低,五年的無遠端轉移率是100%,相較於三級腫瘤(n= 9)的40.0%(p= 0.02)。另外無病惡化率方面也顯示二級腫瘤機率具有較低的一個趨勢,二級腫瘤五年無病惡化率 83.3%相較於三級腫瘤的 34.3%(p= 0.09)。因為疾病罕見以及個案數少的關係,手術切除的程度與總體存活率、局部控制率、無病惡化率都無關。結論:手術治療是單發性纖維瘤的主要治療方式,而放射治療提供良好且安全的控制率。病理的分級是一個顯著的預後因子。

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