含有血管中心性排列特征的毛细胞型星形细胞瘤:病例报告并文献复习  

Pilocytic astrocytoma with angiocentric arrangement: a case report and literature review

作　　者：程海霞[1] 汪寅[1] 

机构地区：[1]复旦大学附属华山医院神经病理室,上海200040

出　　处：《中国现代神经疾病杂志》2013年第4期342-348,共7页Chinese Journal of Contemporary Neurology and Neurosurgery

摘　　要：研究背景毛细胞型星形细胞瘤是常见于小脑的低级别胶质瘤,好发于儿童和青少年。本文报告1例临床罕见、发生于成人幕上的毛细胞型星形细胞瘤病例,其病理形态与典型的毛细胞型星形细胞瘤略有不同:脑组织大部分区域肿瘤细胞围绕玻璃样变性小血管呈单层排列,即所谓的血管中心性生长。本文拟探讨含有血管中心性排列特征的毛细胞型星形细胞瘤的临床、影像学及病理学特征,并讨论其与相关肿瘤的临床病理学鉴别要点,以提高对此类肿瘤的诊断与鉴别诊断能力。方法与结果男性患者,28岁。临床表现为反复发作的癫大发作,抗癫药物治疗无效,脑电图检查未发现典型的癫样放电或局灶性δ波。影像学检查以额叶皮质边界清楚的囊性病变伴壁结节为特征性表现。术中可见肿瘤组织质地较韧,表浅为囊性结构、深部呈实质性结构。光学显微镜下观察肿瘤细胞胞核呈圆形、卵圆形,胞质两端突起细长毛发状,围绕玻璃样变性小血管单层排列,部分区域肿瘤细胞胞核伸展呈梭形,束状致密排列或疏松星网状排列,偶见Rosenthal纤维和嗜酸性颗粒小体形成。肿瘤细胞弥漫表达胶质纤维酸性蛋白、波形蛋白、S-100蛋白、巢蛋白和CD56,局灶性表达Olig2,不表达上皮膜抗原、异柠檬酸脱氢酶1、P53、神经元核抗原、神经微丝蛋白、突触素及CD34,Ki-67抗原标记指数约为1%。病理诊断:左侧额叶含有血管中心性排列特征的毛细胞型星形细胞瘤(WHOⅠ级)。术后未行放射治疗或药物化疗,随访1年余未出现癫发作,肿瘤无复发。结论毛细胞型星形细胞瘤好发于儿童,以小脑多见,发生于成人及幕上者少见,含有血管中心性排列特征的毛细胞型星形细胞瘤形态特殊,诊断时应注意与毛细胞黏液型星形细胞瘤、星形母细胞瘤、血管中心型胶质瘤、皮质室管膜瘤及乳头状型胶质神经元肿瘤相鉴Background Pilocytic astrocytoma （PA） is a low-grade glioma that occurs mainly in the cerebellum of children and young adults. This article is to report a rare case of PA with angiocentric arrangement in the supratentorial region of a 28- year-old male. The pathomorphism of this tumor is different from that of typical PA, since most tumor cells grow in single layer around the hyalinized degenerative vessels, that is to say, angiocentric pattern. This paper aims to discuss the clinical, imaging and pathological features of PA with angiocentric arrangement and clinieopathologieal differentiations from relevant tumors, so as to improve the ability of diagnosing and differentiating this kind of tumor. Methods and Results A 28-year-old male with no significant past history presented with repeated grand mal seizures and did not recover in the treatment with antiepileptic drugs. Physical and neurological examinations were normal. Eleetroencephalography （EEG） found no typical epileptic wave or focal 8 wave. CT and MRI showed a superficial cystic mass with a mural nodule and clear boundary in the left frontal cortex. During the resection of the tumor, the lesion was identified as cystic structure on the surface and solid structure within the cyst wall. Optical microscopy findings revealed that tumour cells with round oroval nuclei and cytoplasm showing slender hair-like protuberance of both ends were arranged in single layer around hyalinized blood vessels. Some tumor cells with fusiform nuclei showed compact fascicles or loose stellate reticulum. Rosenthal fibers and eosinophilic granular bodies were occasionally seen. According to immunohistochemical staining, the tumor cells were diffusely positive for glial fibrillary acidic protein （GFAP）, Vimentin （Vim）, S-100 protein （S-100）, Nestin, CD56, and focally positive for Olig2, while the tumor cells were negative for epithelial membrane antigen （EMA）, isocitrate dehydrogenase 1 （IDH1）, P53, neuronal nuclei antigen （NeuN）, neurofilament prot

关 键 词：脑肿瘤 星形细胞瘤 毛细胞 听觉 

分 类 号：R739.4[医药卫生—肿瘤]