Matyja Ewa, Grajkowska Wiesława, Kunert Przemysław, Marchel Andrzej
Department of Experimental and Clinical Neuropathology, M. Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland; Department of Neurosurgery, M. Sklodowska-Curie Memorial Cancer Centre, Warsaw, Poland.
Neuropathology. 2014 Oct;34(5):491-8. doi: 10.1111/neup.12124. Epub 2014 Apr 16.
Dysembryoplastic neuroepithelial tumors (DNTs) mostly display typical clinical, neuroimaging and histopathological features, but sometimes they reveal heterogeneous or non-specific morphology, which results in diagnostic dilemmas. We present a case of a young adult with longstanding, intractable epilepsy associated with a multinodular cystic lesion in the temporal lobe. The lesion consisted of morphologically different components. In particular, a few cortical nodules displayed a specific glioneuronal element with floating neurons typically found in DNT. Two large, well-circumscribed nodules were entirely composed of biphasic, piloid, astroglial patterns that corresponded strictly to a pilocytic astrocytoma. The well-defined areas, which contained numerous distinct neurocytic-like rosettes, were identical with rosette-forming glioneuronal tumors (RGNTs). This type of neurocytic rosette was widespread within the surrounding piloid background. Some solid nodules exhibited increased cellularity, oligodendroglioma-like elements and a focal ribbon cell arrangement. The lesion was associated with advanced reactive gliosis and foci of dysplastic changes in the adjacent cortex. The clinico-radiological and main histopathological features were consistent with a diagnosis of a complex variant of DNT composed of pilocytic and rosette-forming glioneuronal components. Although both piloid tissue and rosette-like formations have been occasionally mentioned in DNT lesions, the present case of DNT was unique in its well-circumscribed, separate pilocytic and RGNT nodules. We concluded that it represented an unusual, mixed pilocytic/RGNT variant of DNT.
胚胎发育不良性神经上皮肿瘤(DNTs)大多表现出典型的临床、神经影像学和组织病理学特征,但有时也会呈现出异质性或非特异性形态,从而导致诊断难题。我们报告一例年轻成人病例,患有长期难治性癫痫,与颞叶的多结节囊性病变相关。该病变由形态不同的成分组成。特别是,一些皮质结节显示出一种特定的神经胶质神经元成分,带有通常在DNT中发现的漂浮神经元。两个大的、边界清楚的结节完全由双相、毛细胞样星形胶质细胞模式组成,与毛细胞型星形细胞瘤完全相符。明确界定的区域含有大量独特的神经细胞样玫瑰花结,与形成玫瑰花结的神经胶质神经元肿瘤(RGNTs)相同。这种类型的神经细胞玫瑰花结在周围的毛细胞样背景中广泛存在。一些实性结节细胞增多,有少突胶质细胞瘤样成分和局灶性带状细胞排列。该病变与相邻皮质的晚期反应性胶质增生和发育异常改变灶相关。临床放射学和主要组织病理学特征与由毛细胞型和形成玫瑰花结的神经胶质神经元成分组成的DNT复杂变体的诊断一致。虽然在DNT病变中偶尔会提到毛细胞样组织和玫瑰花结样结构,但本病例的DNT其边界清楚、独立的毛细胞型和RGNT结节是独特的。我们得出结论,它代表了一种不寻常的、混合毛细胞型/RGNT的DNT变体。