Jay V, Greenberg M
Department of Pathology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
Pediatr Pathol Lab Med. 1997 Jan-Feb;17(1):105-14.
Neuronal differentiation is well documented in cerebellar primitive neuroectodermal tumors but is uncommon in other cerebellar neoplasms. Although rare, gangliogliomas and gangliocytomas have been previously described in the cerebellum. We report a cerebellar ganglioglioma in a 14-year-old boy, which revealed bizzare markedly pleomorphic cells with extremely pronounced nuclear atypia but less than one mitosis per 50 high-power fields and no necrosis. The tumor showed glial as well as neuronal differentiation, with abundant bi- and multinucleated ganglion cells. There were abundant Rosenthal fibers, eosinophilic granular bodies, focal calcification, and perivascular lymphocytic infiltrates. There has been no evidence of tumor recurrence or neurological deterioration 21 years after surgery, although the marked nuclear atypia led to an initial diagnosis of an anaplastic glioma. We stress the need for careful evaluation of cerebellar gliomas in children that show only nuclear atypia or endothelial hyperplasia in the absence of other features of malignancy.
神经元分化在小脑原始神经外胚层肿瘤中已有充分记录，但在其他小脑肿瘤中并不常见。虽然罕见，但此前曾在小脑中描述过神经节胶质瘤和神经节细胞瘤。我们报告了一名14岁男孩的小脑神经节胶质瘤，其显示出奇异的、明显多形性的细胞，核异型性极为明显，但每50个高倍视野中少于1个有丝分裂且无坏死。肿瘤显示出神经胶质和神经元分化，有大量双核和多核神经节细胞。有丰富的Rosenthal纤维、嗜酸性颗粒体、局灶性钙化和血管周围淋巴细胞浸润。尽管明显的核异型性最初导致诊断为间变性胶质瘤，但术后21年没有肿瘤复发或神经功能恶化的证据。我们强调，对于仅表现为核异型性或内皮细胞增生而无其他恶性特征的儿童小脑胶质瘤，需要进行仔细评估。
