Lim Sungryong, Kim Jeong Hoon, Kim Sun A, Park Eun Suk, Ra Young Shin, Kim Chang Jin
Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
J Korean Neurosurg Soc. 2013 May;53(5):281-7. doi: 10.3340/jkns.2013.53.5.281. Epub 2013 May 31.
Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor classified as WHO II. It is generally benign, but disease progression and malignant transformation have been reported. Prognostic factors for PXA and optimal therapies are not well known.
The study period was January 2000 to March 2012. Data on MR findings, histology, surgical extents and adjuvant therapies were reviewed in twenty-two patients diagnosed with PXA.
The frequent symptoms of PXA included seizures, headaches and neurologic deficits. Tumors were most common in the temporal lobe followed by frontal, parietal and occipital lobes. One patient who died from immediate post-operative complications was excluded from the statistical analysis. Of the remaining 21 patients, 3 (14%) died and 7 (33%) showed disease progression. Atypical tumor location (p<0.001), peritumoral edema (p=0.022) and large tumor size (p=0.048) were correlated with disease progression, however, Ki-67 index and necrosis were not statistically significant. Disease progression occurred in three (21%) of 14 patients who underwent GTR, compared with 4 (57%) of 7 patients who did not undergo GTR, however, it was not statistically significant. Ten patients received adjuvant radiotherapy and the tumors were controlled in 5 of these patients.
The prognosis for PXA is good; in our patients overall survival was 84%, and event-free survival was 59% at 3 years. Atypical tumor location, peritumoral edema and large tumor size are significantly correlated with disease progression. GTR may provide prolonged disease control, and adjuvant radiotherapy may be beneficial, but further study is needed.
多形性黄色瘤型星形细胞瘤(PXA)是一种罕见的原发性低级别星形细胞瘤,被归类为世界卫生组织(WHO)II级。它通常为良性,但已有疾病进展和恶性转化的报道。PXA的预后因素及最佳治疗方法尚不明确。
研究时间段为2000年1月至2012年3月。回顾了22例诊断为PXA患者的磁共振成像(MR)表现、组织学、手术切除范围及辅助治疗的数据。
PXA常见症状包括癫痫发作、头痛和神经功能缺损。肿瘤最常见于颞叶,其次为额叶、顶叶和枕叶。一名死于术后即刻并发症的患者被排除在统计分析之外。在其余21例患者中,3例(14%)死亡,7例(33%)出现疾病进展。非典型肿瘤位置(p<0.001)、瘤周水肿(p=0.022)和肿瘤体积大(p=0.048)与疾病进展相关,然而,Ki-67指数和坏死在统计学上无显著意义。14例行大体肿瘤切除(GTR)的患者中有3例(21%)出现疾病进展,而7例未行GTR的患者中有4例(57%)出现疾病进展,但差异无统计学意义。10例患者接受了辅助放疗,其中5例患者的肿瘤得到控制。
PXA的预后良好;在我们的患者中,3年总生存率为84%,无事件生存率为59%。非典型肿瘤位置、瘤周水肿和肿瘤体积大与疾病进展显著相关。GTR可能提供更长时间的疾病控制,辅助放疗可能有益,但仍需进一步研究。