脊髓血管母细胞瘤的显微手术治疗(附11例分析)

目的探讨脊髓血管母细胞瘤的临床特点和外科治疗技巧。方法回顾性分析11例脊髓髓内血管母细胞瘤的诊治经验。本组临床表现主要为感觉和运动障碍、肌肉萎缩及大小便失禁。均经后正中入路切除肿瘤,术中及术后常规使用甲基强的松龙。结果肿瘤均获全切除。术后短时间内症状改善8例,无变化2例,加重1例,无手术死亡。结论本病根据术前典型的影像资料均可确诊。术中尽可能避免活检或分块切除肿瘤,以免引起难以控制的出血和盲目止血引起的脊髓损害。大剂量甲基强的松龙可减轻脊髓水肿。     

Stromal cells in hemangioblastoma: neuroectodermal differentiation and morphological similarities to ependymoma

The histogenesis of stromal cells in hemangioblastoma is inconclusive despite a long-term controversy. An immunohistochemical and ultrastructural study was conducted for 17 cases of cerebellar hemangioblastoma. A wide range of immunohistological markers, targeting epithelial, mesenchymal, endothelial and neuroectodermal tissues, was used. In all cases, the microscopic hallmark characterizing hemangioblastomas, that is, lipid-containing stromal cells and a fine capillary network, known as a reticular variant, was noted. Stromal cells showed a variable immunoreactivity for neuroectodermal markers, such as S-100 protein, CD56, CD57, CD99, and neuron-specific enolase. This result, in conjunction with the absence of immunoreactivity for epithelial, mesenchymal, and endothelial markers, likely suggests neuroectodermal differentiation of stromal cells. In three cases, another component, known as a cellular variant, where epithelioid tumor cells were arranged in nests encircled by capillaries and/or in pseudorosette-like structures, was noted. Glial fibrillary acidic protein-immunoreactivity, which was totally absent in cases only showing the reticular pattern, was noted in two of them, suggesting a distinctive sign of glial differentiation in a proportion of hemangioblastomas. Ultrastructurally, microvilli-like projections in intracytoplasmic vacuoles were demonstrated in stromal cells. This result, taken together with the neuroectodermal hypothesis of stromal cells, suggests that hemangioblastomas may occasionally exhibit morphological similarities to ependymomas.     

Supratentorial hemangioblastoma: A rare case report and literature review

Hemangioblastomas are rare and benign tumors of the central nervous system. They account for 1.5%-2.5% of all intracranial tumors and have an incidence of 3.2%. The resemblance of hemangioblastomas to other tumors renders preoperative diagnosis and management challenging. Herein, we report a case of a supratentorial hemangioblastoma accompanied by extensive reactive gliosis and diagnosed through magnetic resonance imaging. In addition, we review the relevant literature.     

侧脑室实性血管母细胞瘤1例并文献复习

目的 探讨侧脑室实性血管母细胞瘤(HBS)的临床特点与治疗方法 .方法 回顾分析1例目前国内外年龄最小的侧脑室实性血管母细胞瘤患者的临床资料;并复习相关文献.结果 患者为女性,15岁;表现间断性头痛3个月,头痛加重伴发热1周;查体无异常神经系统体征.头颅MRI检查示,右侧脑室体部不规则形实性混杂信号影,增强扫描病灶呈明...     

脊髓血管母细胞瘤MRI诊断

目的:探讨脊髓血管母细胞瘤磁共振成像(MRI)影像学特征。方法:回顾性分析经手术病理证实12例脊髓血管母细胞瘤的MRI表现。结果:在12例中,病灶位于颈髓7例,胸髓3例,颈胸段、胸腰段水平各1例。其中3例为多发灶,1例合并脑内血管母细胞瘤。MRI表现:受侵脊髓局限性增粗,与正常脊髓信号相比,T1WI呈等低信号结节性肿块,其中1例伴斑片状高信号(因出血);T2WI为混杂高信号;增强扫描,肿瘤结节显著强化,边界清晰。在T2WI或增强扫描上,见特征性瘤内或瘤周迂曲的畸形血管流空信号9例,肿瘤上下继发性脊髓空洞或囊肿10例。结论:MRI检查是诊断脊髓血管母细胞瘤的有效手段,有助于诊断和鉴别诊断。     

脑血管母细胞瘤的CT和MRI诊断

目的 分析血管母细胞瘤的CT、MRI影像特征及鉴别诊断。方法 30例经手术病理证实的血管母细胞瘤,19例行CT检查,11例MRI检查。结果 25例病灶位于幕下,5例位于幕上。单发28例,多发2例。囊结节型22例,囊肿型5例,实质肿块或混合型3例。CT平扫表现球形低密度影,囊腔壁结节和实质肿块均呈等密度或稍高于囊液密度。MRI示T1WI高于囊液信号,T2WI呈稍高或等信号。壁结节和实质肿块明显强化,强化密度曲线呈速升缓降型,MRI信号增强率升高并可见流空信号。结论 血管母细胞瘤有特征性,CT和MRI表现,CT动态增强扫描、密度曲线测定以及MRI增强率的表达有助于诊断和鉴别诊断。     

颅内血管母细胞瘤的诊断和显微手术治疗

目的探讨颅内血管母细胞瘤诊断方法和手术治疗策略。方法回顾性分析28例经手术和病理证实的血管母细胞瘤的临床资料,并从影像学诊断方法、特点及显微手术治疗中注意事项予以分析。结果本组血管母细胞瘤好发年龄为20-45岁,肿瘤位于小脑半球20例,位于中线蚓部、Ⅳ脑室内累及延髓背侧7例,幕上1例。除3例多发外,余均为单发。显微镜下手术全切除23例,次全切除3例,部分切除2例。未全切除病例术后加行放射治疗。本组手术无死亡。随访期内3例患者复发。结论外科治疗应在不加重神经功能障碍的前提下尽量全切除肿瘤。显微手术技术、CTA的应用、肿瘤术前栓塞以及立体定向放射外科等可提高手术疗效。     

小脑血管母细胞瘤的诊断和治疗

目的:分析小脑血管母细胞瘤的临床特点及治疗。方法:回顾性分析小脑血管母细胞瘤并经病理及手术证实的患者资料。结果:小脑血管母细胞瘤患者21例,发病年龄为28～57岁,男性多于女性,肿瘤位于小脑半球19例,小脑蚓部2例。21例行CT检查,囊性肿瘤19例,实质性肿瘤2例。行MRI检查13例,肿瘤为囊性者11例,实质性者2例。19例囊性结节型肿瘤中,手术行瘤结节全切除17例,大部切除2例;2例实质性肿瘤行部分切除。瘤结节部分切除加放射治疗3例。术后恢复良好20例,死亡1例。结论:本病临床表现多为颅高压症状、共济失调等。CT、MRI检查可以定位或定性。治疗首选手术切除,预后良好。     

Cerebellar hemangioblastoma simulating arachnoid cyst on imaging and surgery

A case of posterior fossa hemangioblastoma simulating arachnoid cyst on imaging and peroperatively is presented. In vivo proton MR spectroscopy showed evidence of large lactate and resonance at 2.37 ppm not observed earlier in the cystic lesions, including arachnoid cyst. The demonstration of this resonance may help in characterization of these lesions that may be confused with arachnoid cysts on imaging and during surgery. Electronic Publication