Guo S P, Zhang F, Li Q L, Li Q, Wang W L, Li F F
Department of Pathology, The Fourth Military Medical University, Xi an, Shan Xi province, China.
Clin Neuropathol. 2008 Mar-Apr;27(2):72-7. doi: 10.5414/npp27072.
Papillary glioneuronal tumor (PGNT) is a recently identified low-grade mixed glial-neuronal neoplasm of juvenile and young adult patients. The WHO classification does not categorize this tumor as a separate entity, but rather considers it as a variant of ganglioglioma. We present a new case of this rare entity, representing the 3rd case of this lesion in Chinese patients and review the findings in 34 patients from different case reports found in the international literature. This report describes a histologically similar-appearing lesion arising in the left frontoparietal lobe of a 23-year-old man. Its salient morphological characteristics are the presence of pseudopapillary structures composed of blood vessels, often hyalinized, lined by uniform small astrocytes and a proliferation of neurocytic cells which eventually admixed with ganglioid and ganglion cells. Sporadic Rosenthal fibers, foci of calcification, areas of hemosiderin deposition were identified. The mean Ki67 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation or necrosis were consistently lacking. It is important that every new case of PGNT is reported to allow its recognition and classification. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular low malignant potential neoplasm, whose presentation may allow for consideration as an entity.
乳头状胶质神经元肿瘤（PGNT）是一种最近才被确认的、发生于青少年和年轻成年患者的低级别混合性胶质-神经元肿瘤。世界卫生组织（WHO）分类并未将该肿瘤列为一个单独的实体，而是将其视为神经节胶质瘤的一种变体。我们报告了这一罕见实体的一例新病例，这是中国患者中的第3例该病变，并回顾了国际文献中不同病例报告里34例患者的研究结果。本报告描述了一名23岁男性左额顶叶出现的组织学表现相似的病变。其显著的形态学特征是存在由血管组成的假乳头结构，血管常发生玻璃样变，内衬均匀的小星形胶质细胞，并有神经细胞样细胞增生，最终与类神经节细胞和神经节细胞混合。可见散在的Rosenthal纤维、钙化灶、含铁血黄素沉积区。平均Ki67标记指数仍低于1%。始终未见间变迹象，尤其是有丝分裂象、内皮细胞增生或坏死。报告每一例新的PGNT病例对于其识别和分类很重要。我们认为PGNT是一种临床和形态学上界定明确的脑室外低恶性潜能肿瘤亚组，其表现可能使其可被视为一个独立的实体。
