Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
World Neurosurg. 2011 May-Jun;75(5-6):699-703. doi: 10.1016/j.wneu.2010.10.046.
The simultaneous presence of Lhermitte-Duclos disease (LDD) with focal areas of nodular dysembryoplastic neuroepithelial tumor (DNET) differentiation in the cerebellar hemisphere is reported in a patient who showed the characteristic magnetic resonance imaging (MRI) appearance of "tiger striping".
A 25-year-old man presented with a 7-month history of holocranial headache, progressive vision diminution, and right-sided cerebellar signs. Computed tomography (CT) scan revealed a hypodense, nonenhancing right cerebellar lesion effacing the fourth ventricle. There were thin hyperdense, linear striations across the tumor with hypodense streaks between them. The third and lateral ventricles were dilated with periventricular lucency. MRI showed the hypointense-to-isointense right cerebellar lesion with linear striations on T1-weighted images. The lesion was hyperintense on T2-weighted images with areas of linear hypointensity streaks running throughout the lesion characteristic of the "tiger striping" effect of LDD. Sagittal MRI revealed tonsillar herniation. Gross total removal of the tumor was accomplished with right paramedian suboccipital craniectomy. The postoperative course was unremarkable, and the patient remained symptom-free at 3-month follow-up examination. Histology revealed the simultaneous presence of LDD with DNET.
To the best of the authors' knowledge, the simultaneous presence of cerebellar LDD with DNET has never been reported before. This case report may point to their common genesis with cortical dysplasia and neuronal migrational abnormalities playing an important role. LDD and DNET may represent different points in the spectrum of the lesion-from being purely hamartomatous to having a greater proliferative potential. The simultaneous presence of LDD and DNET was perhaps responsible for the unusually rapid neurologic deterioration in this patient before surgical intervention.
报告了一例同时存在小脑半球 Lhermitte-Duclos 病（LDD）和局灶性结节性发育不良性神经上皮肿瘤（DNET）分化的病例，该患者具有特征性的磁共振成像（MRI）“虎纹”外观。
一名 25 岁男性，表现为全颅头痛 7 个月，视力逐渐下降，以及右侧小脑体征。计算机断层扫描（CT）显示右小脑低密度、非增强病变，使第四脑室变形。肿瘤内有细的高密度线性条纹，其间有低密度条纹。第三脑室和外侧脑室扩张，伴有脑室周围透亮。MRI 显示右小脑低信号至等信号病变，T1 加权图像上有线性条纹。病变在 T2 加权图像上呈高信号，线性低信号条纹贯穿整个病变，具有 LDD 的“虎纹”效应特征。矢状位 MRI 显示扁桃体疝。通过右旁正中枕下入路完成肿瘤的全切除。术后过程无明显异常，患者在 3 个月随访检查时仍无症状。组织学显示同时存在 LDD 和 DNET。
据作者所知，小脑 LDD 与 DNET 同时存在的情况以前从未报道过。本病例报告可能表明它们具有共同的起源，皮质发育不良和神经元迁移异常起着重要作用。LDD 和 DNET 可能代表病变谱中的不同点——从纯粹的错构瘤到具有更大的增殖潜力。LDD 和 DNET 的同时存在可能是导致该患者在手术干预前神经功能迅速恶化的原因。
