Preusser Matthias, Dietrich Wolfgang, Czech Thomas, Prayer Daniela, Budka Herbert, Hainfellner Johannes A
Institute of Neurology, University of Vienna, AKH 4J, Währinger Gürtel 18-20, POB 48, 1097, Vienna, Austria.
Acta Neuropathol. 2003 Nov;106(5):506-8. doi: 10.1007/s00401-003-0758-2. Epub 2003 Aug 12.
Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle has been reported recently as a novel type of primary CNS neoplasm. We present the case of a 35-year-old male patient with RGNT of the fourth ventricle. The tumor was found incidentally; the patient did not suffer from any neurological symptoms. The tumor mass involved the caudal cerebellar vermis, filled the fourth ventricle and protruded into the caudal part of the mesencephalic aquaeduct. Smaller tumor nodules were visible in the adjacent right cerebellar hemisphere. Histologically, prominent neurocytic rosettes with synaptophysin expression were embedded in a glial tumor component resembling pilocytic astrocytoma. Clinicopathological features of our case closely resemble those reported in the original description. Thus, our case confirms RGNT as a new distinct type of primary CNS neoplasm. Due to its distinct features, adoption of RGNT as a new entity into the WHO classification of tumors should be considered.
第四脑室的菊形团形成性胶质神经元肿瘤（RGNT）最近被报道为一种新型的原发性中枢神经系统肿瘤。我们报告一例35岁男性第四脑室RGNT患者。肿瘤为偶然发现；患者无任何神经症状。肿瘤块累及小脑蚓尾，充满第四脑室并突入中脑导水管尾部。在相邻的右侧小脑半球可见较小的肿瘤结节。组织学上，具有突触素表达的显著神经细胞菊形团包埋于类似毛细胞型星形细胞瘤的胶质肿瘤成分中。我们病例的临床病理特征与最初描述中报道的特征极为相似。因此，我们的病例证实RGNT是一种新的独特类型的原发性中枢神经系统肿瘤。由于其独特特征，应考虑将RGNT作为一个新的实体纳入世界卫生组织肿瘤分类中。
