Heese O, Lamszus K, Grzyska U, Westphal M
Department of Neurosurgery, University Hospital Hamburg-Eppendorf, Germany.
Acta Neurochir (Wien). 2002 Jul;144(7):723-8. doi: 10.1007/s00701-002-0960-3.
The case of a 38-year old man with a histologically benign choroid plexus papilloma arising within the fourth ventricle with en plaque growth around the brain stem and medulla is described in detail. Up to this point this particular growth pattern has not been published and is a rare presentation for this tumour.
The patient presented with a 1.5 year history of headache, nausea, and vomiting in the morning. Additional symptoms like blurred vision and gait ataxia lead to hospital admission. MRI demonstrated a homogeneously contrast-enhancing tumour completely filling the fourth ventricle and subsequent obstructive hydrocephalus. In addition Gd enhancement encasing the brain stem, the lower aspect of the medulla and the conus medullaris was seen suggesting a disseminated ependymoma or medulloblastoma.
An extensive resection of the tumour in the fourth ventricle and CP angle was performed. Infiltrative growth into the structures of the left CP angle and into the rhomboid fossa hampered complete removal. Surprisingly histological examination revealed a well-differentiated papillary choroid plexus papilloma without signs of anaplasia. On follow up imaging the Gd enhancement encasing the pons vanished completely. A growing cyst adjacent to a small tumour residuum left behind on the floor of the fourth ventricle led to re-operation after 8 months with complete removal.
This case presents several biological, neuroradiological and surgical aspects which make it noteworthy and we hope that the informations provided add to the understanding of these tumours, expand the differential diagnostic thinking of lesions which present with diffuse arachnoid Gd enhancement upon first presentation.
详细描述了一名38岁男性病例，其组织学表现为良性脉络丛乳头状瘤，起源于第四脑室，呈斑块状生长，环绕脑干和延髓。截至目前，这种特殊的生长模式尚未见报道，对于该肿瘤来说是一种罕见的表现。
患者有1.5年的晨起头痛、恶心和呕吐病史。其他症状如视力模糊和步态共济失调导致其入院。磁共振成像（MRI）显示一个均匀强化的肿瘤完全充满第四脑室，并继发梗阻性脑积水。此外，可见钆（Gd）强化包绕脑干、延髓下部和脊髓圆锥，提示为播散性室管膜瘤或髓母细胞瘤。
对第四脑室和小脑脑桥角的肿瘤进行了广泛切除。肿瘤浸润性生长至左侧小脑脑桥角结构及菱形窝，妨碍了完全切除。令人惊讶的是，组织学检查显示为分化良好的乳头状脉络丛乳头状瘤，无间变迹象。随访成像显示包绕脑桥的Gd强化完全消失。第四脑室底部遗留的一个小肿瘤残端旁出现一个不断增大的囊肿，导致8个月后再次手术并完全切除。
该病例呈现出几个生物学、神经放射学和手术方面的特点，使其值得关注，我们希望所提供的信息有助于增进对这些肿瘤的理解，拓展对初次表现为弥漫性蛛网膜Gd强化病变的鉴别诊断思路。
