Yu Shenping, He Li, Zhuang Xiaozhao, Luo Boning
Department of Radiology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
Acta Radiol. 2011 Mar 1;52(2):223-8. doi: 10.1258/ar.2010.100221.
Pleomorphic xanthoastrocytoma (PXA) is a rare and special type of astrocytoma which occurs in childhood and adolescence, and usually with a favorable prognosis. Since its initial description, PXA cases have been reported infrequently in the literature, mostly as single cases or small series.
To review the imaging characteristics of PXA.
MR imaging findings of 19 pathologically confirmed PXAs were retrospectively analyzed and correlated with the clinical and pathological features.
The clinical presentation in 18 patients included: dizziness (8); headache (9); and epilepsy (8). One patient was asymptomatic. On MR imaging, the tumors were located in the brain surface (17), thalamus (1) or deep in the right frontal lobe (1). The tumors were cystic (4), mixed cystic-solid (7), or solid (8) with well-defined (11) or poorly-defined borders (8). Peritumoral edema was marked (8), mild (9) or absent (2). Cystic components of tumors were hypointense on T1- and hyperintense on T2-weighted images whereas the solid components of tumors were hypointense or isointense on T1- and slightly hyperintense on T2-weighted images. There was marked (10), moderate (7) or no (2) contrast enhancement in the solid tumors with surrounding leptomeningeal enhancement (7). The tumors were located in the frontal lobes (8), temporal lobes (7), occipital lobe (1), cerebellum (1), thalamus (1), and sellar region (1). Histologically, 18 tumors were classified as WHO grade 2 comprising of pleomorphic giant cells, spindle cells and foamy cells. One PXA with anaplastic features was composed of pleomorphic polygonal cells and spindle cells, and with high mitotic activity ( ≥5 mitoses per 10 HPF). Immunohistochemical reactions to glial fibrillary acidic protein (GFAP) were positive in all 19 cases.
The pathological appearances of PXA are distinctive. MR imaging could display the morphologic features exactly, and has important diagnostic value for PXA.
多形性黄色星形细胞瘤(PXA)是一种罕见且特殊的星形细胞瘤类型,发生于儿童和青少年,通常预后良好。自首次描述以来,PXA病例在文献中报道较少,大多为单个病例或小系列病例。
回顾PXA的影像学特征。
回顾性分析19例经病理证实的PXA的磁共振成像(MR)表现,并与临床和病理特征进行相关性分析。
18例患者的临床表现包括:头晕(8例);头痛(9例);癫痫(8例)。1例患者无症状。在MR成像上,肿瘤位于脑表面(17例)、丘脑(1例)或右额叶深部(1例)。肿瘤为囊性(4例)、囊实性混合(7例)或实性(8例),边界清晰(11例)或不清晰(8例)。瘤周水肿明显(8例)、轻度(9例)或无(2例)。肿瘤的囊性成分在T1加权像上呈低信号,在T2加权像上呈高信号,而实性成分在T1加权像上呈低信号或等信号,在T2加权像上呈轻度高信号。实性肿瘤有明显(10例)、中度(7例)或无(2例)强化,周围软脑膜强化(7例)。肿瘤位于额叶(8例)、颞叶(7例)、枕叶(1例)、小脑(1例)、丘脑(1例)和鞍区(1例)。组织学上,18例肿瘤被分类为世界卫生组织2级,由多形性巨细胞、梭形细胞和泡沫细胞组成。1例具有间变特征的PXA由多形性多边形细胞和梭形细胞组成,有高有丝分裂活性(每10个高倍视野≥5个有丝分裂象)。19例病例对胶质纤维酸性蛋白(GFAP)的免疫组化反应均为阳性。
PXA的病理表现具有特征性。MR成像能够准确显示其形态特征,对PXA具有重要的诊断价值。