THE EFFECT OF RADIOTHERAPY ON THE OUTCOME OF PRIMARY GLIOSARCOMA: A CLINICAL STUDY OF THREE CASES AND A LITERATURE REVIEW

放射線治療於原發性神經膠質肉瘤之療效：三病例個案報告及文獻回顧

劉威麟(Wei-Lin Liu) ； 林奎利(Kuei-Li Lin) ； 張裕綱(Yu-Kang Chang) ； 盧納密(Na-Mi Lu) ； 陳昭勳(Chao-Hsun Chen) ； 張進宏(Chin-Hong Chang) ； 林立青(Li-Ching Lin) ； 何聖佑(Sheng-Yow Ho)

《放射治療與腫瘤學》 24卷4期 (2017/12) Pp. 335-348

https://doi.org/10.6316/TRO/201724(4)335

摘要

Primary gliosarcoma (GS) is a rare primary tumor of the central nervous system (CNS), and a unique entity among high-grade glioma, composed of both malignant glial and sarcomatous differentiations. The 2016 WHO classification of central nervous tumors classified GS as a grade IV astrocytic neoplasm and a variant of glioblastoma. Due to the rarity of GS, the optimal therapy is not well defined, and the clinical therapies for GS are usually in accordance with glioblastoma guidelines by the mode of maximal surgical resection followed by chemo-radiotherapy with temozolomide. This report describes three rare pathologically confirmed cases of GS in our hospital and analyzes the distinct clinical features, treatment course with more focus on radiotherapy, and their outcome. In our three GS patients, two males and one female with a median age of 61, the main tumors all occurred in the supra-tentorial brain. Two cases are presented with CNS metastasis to different cerebral lobes, and one case with widespread leptomeninges at initial diagnosis. Extra-cranial metastasis is also exhibited in the lungs and pleura during the clinical course. All patients received postoperative radiation therapy with conventional fractionation or hypofractionation regimen. The total radiation dose to the involved brain tumor bed (planning target volume) reached 50 - 60 Gy (range 50.4 to 59.4 Gy). Our results support the role of local radiotherapy after surgery, and the local control effect can be achieved or palliated without obvious side effects. Our cases survived 8 to 23 months with a median survival of 17 months. To highlight the unique clinical features of GS, we conduct a literature review of the reported clinical case series of GS patients in Taiwan and the world using Medline.

關鍵字

Gliosarcoma ； Radiotherapy ； Metastasis ； High-grade glioma ； Taiwan

並列摘要

原發性神經膠質肉瘤（gliosarcoma, GS）是中樞神經系統的罕見原發腫瘤，是一種獨特的高度惡性神經膠質瘤，同時擁有惡性神經膠質與肉瘤的分化。2016年世界衛生組織在中樞神經系統腫瘤分類中將GS歸類為膠質母細胞瘤（glioblastoma）的亞型，皆為第四級的惡性腦瘤。由於GS相當罕見，最佳療法目前仍不明確，臨床治療上多遵循膠質母細胞瘤的治療指引，在最大範圍手術切除後，搭配temozolomide化療併同步放射治療。本報告介紹了三位經由病理確診的GS案例，分析其臨床特徵，治療過程，放射治療及其療效。三名GS患者，有兩名男性和一名女性，中位年齡為61歲，腫瘤全部發生在天幕上區域。兩例患者有中樞神經系統轉移至對側或其他腦葉，另一例在診斷時即有廣泛的腦脊膜轉移，其中一例發生肺和胸膜的顱外轉移。所有患者均接受傳統分次或低分次高劑量的術後放射線治療。目標腦腫瘤體積的總放射劑量達到50至60 Gy（範圍50.4至59.4 Gy）。我們的研究結果指出手術後局部放療可以達到腦腫瘤局部控制或緩解效果，並且沒有明顯的放療副作用。三名病例存活期為8至23個月，存活期中位數17個月。由於GS的獨特臨床和治療特徵相當值得研究，我們在Medline蒐集台灣和世界各地的GS患者文獻報告進行整理及文獻回顧。

並列關鍵字

神經膠質肉瘤 ； 放射線治療 ； 轉移 ； 高度惡性神經膠質瘤 ； 台灣