Boissonneau S, Terrier L-M, De La Rosa Morilla S, Troude L, Lavieille J P, Roche P-H
Department of Neurosurgery, Assistance publique-Hôpitaux de Marseille, Aix-Marseille University, 13015 Marseille, France.
Department of Neurosurgery, CHRU de Tours, University François-Rabelais, 37000 Tours, France.
Neurochirurgie. 2016 Oct;62(5):266-270. doi: 10.1016/j.neuchi.2016.07.001. Epub 2016 Oct 19.
Gangliogliomas are rare tumors of the central nervous system. We report two unusual cases of gangliogliomas located in the cerebellopontine angle (CPA).
The first patient was a 57-year-old woman, who presented with dizziness and harbored a non-enhanced heterogeneous mass located in the cisternal space of the CPA. A partial microsurgical removal was performed, and the pathological examination concluded a grade I ganglioglioma according to the WHO Classification. The postoperative course was uneventful without any adjuvant treatment and the 5-year imaging follow-up indicated a stable remnant tumor. The second patient was a 35-year-old male who presented with acute vertigo and imbalance associated with recent prominent headaches; MR imaging showed a large heterogeneous and post-contrast enhanced tumor mass located in the CPA cistern with a mass effect on the brain. An optimal subtotal surgical resection was performed. The pathologists concluded a WHO grade III ganglioglioma. In spite of adjuvant radiotherapy and chemotherapy, the evolution proved unfavorable and patient died from cancer complications within a 2-year period. In both cases, the precise origin of the tumor could not be clearly identified even if the major component was present in the cisternal space.
Gangliogliomas growing into the cisternal spaces are exceedingly rare particularly in the CPA. Due to its infiltrating behavior and major difficulties to identify the tumor margins, total resection is not routinely feasible. The histological grading is the most important predictor for oncological prognosis.
神经节胶质瘤是中枢神经系统的罕见肿瘤。我们报告两例位于小脑脑桥角(CPA)的不寻常神经节胶质瘤病例。
首例患者为一名57岁女性,表现为头晕,在CPA脑池间隙有一个未强化的不均匀肿块。进行了部分显微手术切除,病理检查根据世界卫生组织分类判定为I级神经节胶质瘤。术后过程顺利,未进行任何辅助治疗,5年的影像学随访显示残余肿瘤稳定。第二例患者为一名35岁男性,表现为急性眩晕和失衡,伴有近期明显头痛;磁共振成像显示CPA脑池内有一个大的不均匀且增强后的肿瘤肿块,对脑有占位效应。进行了最佳的次全手术切除。病理学家判定为世界卫生组织III级神经节胶质瘤。尽管进行了辅助放疗和化疗,但病情进展不利,患者在2年内死于癌症并发症。在这两例中,即使肿瘤的主要部分位于脑池间隙,其确切起源也无法明确确定。
生长至脑池间隙的神经节胶质瘤极为罕见,尤其是在CPA。由于其浸润性生长行为以及确定肿瘤边界的主要困难,全切除通常不可行。组织学分级是肿瘤学预后的最重要预测指标。