Yao Kun, Qi Xue-Ling, Mei Xi, Jiang Tao
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University Beijing, China.
Department of Pathology, Beijing San Bo Brain Hospital, Capital Medical University Haidian District, Beijng, P. R. China.
Int J Clin Exp Pathol. 2015 Feb 1;8(2):2079-84. eCollection 2015.
We describe a rare case of gliosarcoma with primitive neuroectodermal, osseous, cartilage and adipocyte differentiation. A 57-year-old man experienced a month history of headache, nausea and vomiting. Worse yet, the headache has become more severe for the past 6 days. Magnetic resonance (MR) images disclosed a lesion with operative indications located in the right frontal lobe. Then the tumor was macroscopically totally removed. Histologically, the tumor showed two kinds of components. One kind of the tumor cells appeared typical astrocytic tumor cells with anaplastic appearance. The other kind of the tumor cells appeared sheets of small round hyperchromatic cells, which presented a kind of pancreatic neuroendocrine tumor (PNET)-like structure. These sheets of small round cells were surrounded by a large number of relative-sparse-spindle cells. Multiple separate distinct areas of adipose tissue, osteoid matrix laid down and cartilage tissue were also identified. Immunohistochemically, a portion of typical astrocytic tumor cells and some small round hyperchromatic cells showed GFAP positivity. Small round hyperchromatic cells were positive for S-100, Fli-1, Nestin, MAP-2 and Syn. A large amount of relative sparse spindle cells (sarcomatous areas) were positive for vimentin. In addition, reticulin staining demonstrated expression of reticular fibers in relative-sparse-spindle cells areas but not in the astrocytic tumor cells and small round hyperchromatic cells areas. Molecular cytogenetic analyses demonstrated PTEN allele loss and no evidence of amplification of EGFR in both the astrocytic tumor cells, PNET-like structure and sparse spindle cells areas. These data suggest that this tumor was a gliosarcoma with primitive neuroectodermal, osseous, cartilage and adipocyte differentiation. To our knowledge, this is a rare gliosarcoma , reporting our additional new case would add to the better understanding of this tumor.
我们描述了一例罕见的具有原始神经外胚层、骨、软骨和脂肪细胞分化的胶质肉瘤。一名57岁男性有1个月的头痛、恶心和呕吐病史。更糟糕的是，在过去6天里头痛变得更加严重。磁共振（MR）图像显示右额叶有一个具有手术指征的病变。随后肿瘤被肉眼下完全切除。组织学上，肿瘤显示出两种成分。一种肿瘤细胞表现为具有间变外观的典型星形细胞瘤细胞。另一种肿瘤细胞表现为成片的小圆形深染细胞，呈现出一种胰腺神经内分泌肿瘤（PNET）样结构。这些成片的小圆形细胞被大量相对稀疏的梭形细胞包围。还发现了多个独立的不同区域的脂肪组织、类骨质基质沉积和软骨组织。免疫组化显示，一部分典型星形细胞瘤细胞和一些小圆形深染细胞呈GFAP阳性。小圆形深染细胞对S-100、Fli-1、Nestin、MAP-2和Syn呈阳性。大量相对稀疏的梭形细胞（肉瘤区域）对波形蛋白呈阳性。此外，网织纤维染色显示在相对稀疏的梭形细胞区域有网状纤维表达，而在星形细胞瘤细胞和小圆形深染细胞区域没有。分子细胞遗传学分析显示，在星形细胞瘤细胞、PNET样结构和稀疏梭形细胞区域均存在PTEN等位基因缺失，且无表皮生长因子受体（EGFR）扩增的证据。这些数据表明该肿瘤是一种具有原始神经外胚层、骨、软骨和脂肪细胞分化的胶质肉瘤。据我们所知，这是一例罕见的胶质肉瘤，报告我们这例新增病例将有助于更好地了解这种肿瘤。
