Pimentel José, Resende Mário, Vaz Artur, Reis Ana M, Campos Alexandre, Carvalho Herculano, Honavar Mrinalini
Department of Neurology, Laboratory of Neuropathology, Institute of Molecular Medicine, Hospital de Santa Maria, Lisbon Faculty of Medicine, Lisbon, Portugal.
Neurosurgery. 2008 May;62(5):E1162-3; discussion E1163. doi: 10.1227/01.neu.0000325879.75376.63.
Rosette-forming glioneuronal tumor is a newly described mixed glial and neuronal tumor. We describe two cases and review the literature to better characterize this entity.
Patients were surgically treated, and tumors were diagnosed by light microscopy and immunohistochemistry using the avidin-biotin complex method. PubMed was searched for previously reported cases.
Patient 1 was a 38-year-old woman who presented with headaches and no neurological abnormality. Magnetic resonance imaging showed a solid mass in the fourth ventricle. Subtotal excision of the mass caused transient gait ataxia. Patient 2 was a 51-year-old woman with dizziness who fell and sustained head trauma. Magnetic resonance imaging revealed a right paramedian cerebellar cystic and nodular mass and a separate nodule in the vermis, which were excised gross totally with no morbidity. Microscopic examination showed neuroepithelial tumors composed of neurocytic cells focally forming well-defined rosettes that were immunopositive for neuronal markers and of elongated, glial fibrillary acidic protein-immunoreactive astrocytes. No histological anaplasia was present. Both patients were well 18 and 8 months after surgery, respectively. Eighteen rosette-forming glioneuronal tumors were identified with the literature search.
These are tumors of young adulthood (range, 12-59 yr) usually in or close to the fourth ventricle. Histologically, they are low-grade, although multiple foci or local extension may prevent total excision and account for some recurrences. On imaging, they are cystic, solid, or both, with minimal perilesional edema or mass effect. They are composed of neurocytic and glial elements, probably arising from a common progenitor in the subependymal plate, and need to be differentiated from a variety of glioneuronal tumors.
菊形团形成性胶质神经元肿瘤是一种新描述的混合性胶质和神经元肿瘤。我们描述两例病例并复习文献以更好地明确这一实体。
对患者进行手术治疗，肿瘤通过光镜和采用抗生物素蛋白-生物素复合物法的免疫组化进行诊断。检索PubMed以查找先前报道的病例。
患者1为一名38岁女性，表现为头痛且无神经功能异常。磁共振成像显示第四脑室内有一实性肿块。肿块次全切除导致短暂性步态共济失调。患者2为一名51岁女性，有头晕症状，跌倒并头部外伤。磁共振成像显示右小脑旁正中囊性和结节性肿块以及蚓部有一独立结节，将其全部切除，无并发症。显微镜检查显示神经上皮肿瘤由局部形成界限清楚的菊形团的神经细胞组成，这些神经细胞对神经元标志物免疫阳性，还有细长的、胶质纤维酸性蛋白免疫反应阳性的星形胶质细胞。无组织学间变。两名患者术后分别在18个月和8个月时情况良好。通过文献检索共确定了18例菊形团形成性胶质神经元肿瘤。
这些肿瘤好发于青年（年龄范围12 - 59岁），通常位于第四脑室内或其附近。组织学上，它们为低级别肿瘤，尽管多个病灶或局部扩展可能妨碍完全切除并导致一些复发。影像学上，它们可为囊性、实性或两者皆有，周围水肿或占位效应轻微。它们由神经细胞和胶质成分组成，可能起源于室管膜下板的共同祖细胞，需要与多种胶质神经元肿瘤相鉴别。
