San Millan Beatriz, Kaci Rachid, Polivka Marc, Robert Gilles, Héran Françoise, Gueguen Antoine, Mokhtari Karima, Gray Françoise
Service d'anatomie et de cytologie pathologiques, hôpital Lariboisière, AP-HP, université Paris 7, 2 rue Ambroise-Paré, Paris cedex 10, France.
Ann Pathol. 2010 Feb;30(1):25-9. doi: 10.1016/j.annpat.2009.10.020. Epub 2009 Nov 25.
Gliomatosis cerebri is a rare glial neoplasm, characterized by diffuse brain infiltration with relative preservation of the underlying cytoarchitecture. Its clinical and radiologic features are not specific and its antemortem diagnosis is difficult. We report a case of gliomatosis cerebri in a 68-year-old woman presenting with gait disturbances and episodic seizures. MRI showed bilateral white matter hypersignal intensities on Flair sequences and brain biopsy revealed a poorly cellular proliferation of neoplasic glial cells strongly expressing OLIG-2, Ki-67 and occasionally GFAP, without alpha-internexin expression. The patient status worsened rapidly and she died 2 months after the initial symptoms. Postmortem brain examination confirmed gliomatosis cerebri and revealed a focal glioblastoma in the frontal cortex, with nuclear p53 expression in the highest malignant areas. Gliomatosis cerebri should be included in the differential diagnostic of diffuse brain lesions. Antemortem diagnosis, although difficult, can be assessed by IRM and careful biopsy examination. Progression to glioblastoma has been seldom reported, enhancing the controversy about the etiopathogenesis of this rare tumour.
大脑胶质瘤病是一种罕见的神经胶质肿瘤,其特征是肿瘤弥漫浸润大脑,而其下的细胞结构相对保留。其临床和放射学特征不具有特异性,生前诊断困难。我们报告一例68岁女性大脑胶质瘤病患者,该患者表现为步态障碍和发作性癫痫。磁共振成像(MRI)在液体衰减反转恢复序列(Flair)上显示双侧白质高信号强度,脑活检显示肿瘤性神经胶质细胞细胞增殖不良,强烈表达少突胶质细胞转录因子2(OLIG-2)、增殖细胞核抗原(Ki-67),偶尔表达胶质纤维酸性蛋白(GFAP),无α-中间丝蛋白表达。患者病情迅速恶化,初始症状出现2个月后死亡。尸检证实为大脑胶质瘤病,并在额叶皮质发现一个局灶性胶质母细胞瘤,在最高恶性区域有核p53表达。大脑胶质瘤病应列入弥漫性脑病变的鉴别诊断。生前诊断虽然困难,但可通过磁共振成像(IRM)和仔细的活检检查来评估。进展为胶质母细胞瘤的情况鲜有报道,这增加了关于这种罕见肿瘤病因发病机制的争议。