Nakajima M, Kidooka M, Nakasu S
Department of Neurosurgery, Shiga University of Medical Science, Seta, Ohtsu, Japan.
Surg Neurol. 1998 Apr;49(4):445-8. doi: 10.1016/s0090-3019(97)00293-0.
Gangliogliomas are rare tumors that generally arise in the temporal lobe. Although most are benign, malignant gangliogliomas have been reported. The clinical course of anaplastic gangliogliomas has not been well understood.
An anaplastic ganglioglioma of the right parieto-occipital lobe is reported in a 7-year-old girl who presented with left homonymous hemianopsia and papilledema. Neurologic examination revealed a choked disc and a left homonymous hemianopsia. A computed tomographic scan and magnetic resonance imaging showed a large enhancing mass with calcification. Radiation therapy was administered after subtotal resection of the tumor. Histologic and immunohistochemical studies showed a typical appearance of anaplastic ganglioglioma. Spinal dissemination developed 3 months after the operation. In spite of spinal axis radiation and chemotherapy, she expired 15 months after the diagnosis.
Although the clinical course of anaplastic gangliogliomas is not always aggressive, our case indicates the importance of strict follow-up assessments of the whole craniospinal axis.
神经节胶质瘤是一种罕见肿瘤，通常发生于颞叶。虽然大多数为良性，但也有恶性神经节胶质瘤的报道。间变性神经节胶质瘤的临床病程尚未完全明了。
本文报道一名7岁女孩，患有右侧顶枕叶间变性神经节胶质瘤，表现为左侧同向性偏盲和视乳头水肿。神经系统检查发现视乳头水肿和左侧同向性偏盲。计算机断层扫描和磁共振成像显示一个伴有钙化的巨大强化肿块。肿瘤次全切除后给予放射治疗。组织学和免疫组化研究显示为典型的间变性神经节胶质瘤表现。术后3个月出现脊髓播散。尽管进行了全脊髓轴放疗和化疗，患者在诊断后15个月死亡。
虽然间变性神经节胶质瘤的临床病程并非总是侵袭性的，但我们的病例表明对整个颅脊髓轴进行严格随访评估的重要性。
