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.     

Juxtapapillary retinal capillary hemangioma: A clinical and histopathological case report

Introduction and importanceJuxtapapillary retinal capillary hemangiomas (RCHs) are vascular hamartomas that occur adjacent to the optic disc. Juxtapapillary RCHs can be found as an isolated finding or in association with Von Hippel-Lindau (VHL) disease. VHL is a dominantly inherited disease that is characterized by multiple intracranial and retinal hemangioblastomas along with benign and malignant visceral tumors. RCH is a hallmark lesion in VHL and typically presents early in the disease.Case presentationWe present the clinical and histopathological findings of a 15-month-old child with juxtapapillary RCH associated with exudative retinal detachment and a family history of VHL. The child presented initially at a late stage and lost to follow-up twice then came back with a blind painful eye secondary to neovascular glaucoma necessitating enucleation.DiscussionAlthough juxtapapillary RCHs are benign, slowly growing tumors, they pose a serious threat to central vision secondary to posterior segment complications such as intraretinal and subretinal exudation, macular edema and exudative retinal detachment and anterior segment complications such neovascular glaucoma if left untreated.ConclusionJuxtapapillary RCHs are potentially blinding tumors if not treated in early stages given their close proximity to the optic nerve (ON) and macula.     

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...     

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。     

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。     

小脑实性血管网织细胞瘤的诊断和显微外科治疗

目的 探讨小脑实性血管网织细胞瘤的影像学特征、诊断及鉴别诊断和手术注意事项。方法 对1993年3月～2003年3月治疗的48例小脑实性血管网织细胞瘤患者的临床资料进行回顾性分析。所有患者术前均进行了CT和MRI检查,20例患者同时进行了DSA检查,16例患者术前进行了肿瘤供血动脉的栓塞治疗,5例患者术前行侧脑室一腹腔分流术。48例患者的病灶部位分别为左小脑半球14例(其中2例突入同侧桥小脑角),右小脑半球12例,上蚓部8例,下蚓部7例,左天幕下6例,左小脑扁桃体1例。结果 48例患者均在显微镜下达到全切除,1例术中发生正常灌注压突破,术后第二天出现消化道出血,其他患者术中术后均顺利,术中除自体血回输外,无一例输异体血,本组患者无手术死亡率,无明显手术并发症。结论 小脑实性血管网织细胞瘤为良性肿瘤,术中严格沿肿瘤包膜分离,先阻断供血动脉,后处理引流静脉,可以达到较少出血的完整切除,而肿瘤完整切除后不存在复发,可达到治愈。     

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