乙状窦后径路听神经瘤切除术中内镜辅助应用价值的探讨

目的：探讨内镜辅助在行乙状窦后径路听神经瘤切除术中的应用和意义。方法：在内镜辅助下经乙状窦后径路听神经瘤切除术12例,术中行面、听神经监测,手术前后面听功能评估,并与未采用内镜辅助的乙状窦后径路手术进行综合比较。结果：12例听神经瘤完全切除,手术完成顺利,术后无并发症。术中面听神经均获解剖保留,术中神经监测以观测面听神经功能保留情况,术后半年面神经功能与术前相比无明显改变,所有患者听觉功能与术前相比均有不同程度下降。内镜辅助下手术组术后面神经功能及听觉功能改变与未采用内镜辅助手术组差异无统计学意义。结论：在乙状窦后径路手术中应用内镜辅助能为桥小脑角区手术提供精确的信息,在避免后半规管、颈静脉球损伤的前提下,完全切除内耳道底的肿瘤,减少并发症的发生,但对面听神经功能保留无明显作用。     

Giant trigeminal schwannoma with parapharyngeal extension: Report of a case

The authors present their experience in the treatment of a giant trigeminal schwannoma with wide extension in the parapharyngeal space using a combination of the orbito-zygomatic and the transcervical-transmandibular approaches. The clinical and radiological findings, advantages of surgical approach and clinical outcome will be discussed.     

额面部丛状神经鞘瘤1例

报告额面部丛状神经鞘瘤1例。患者女,48岁,因“额面部左侧肿物17年,左眉弓皮下肿物3年”就诊。皮肤专科检查：额面部左侧见一约8 mm×6 mm×3 mm大小黄红色类圆形结节,隆起于皮面,表面光滑,质地韧,无压痛。左眉弓上方皮下扪及一约9 mm×8 mm×4 mm大小的结节,质地韧,轻度压痛,活动度可。两处皮损组织病理均为：真皮中下层内可见多个由梭形细胞组成的肿瘤团块,呈结节状,结节间被透明疏松的纤维结缔组织所分隔。免疫组化结果：S-100(+++),Vimentin(+),SMA(-),CD34(-),Ki-67(5%+)。诊断为丛状神经鞘瘤。予以手术切除,随访3年未复发。     

Soft tissue sarcoma as second malignant lesion after therapy for Hodgkin's disease

Summary Two cases of soft tissue sarcoma following treatment of nodular sclerosing Hodgkin's disease are described. One patient developed a malignant schwannoma after radiotherapy, the other was diagnosed as having a malignant fibrous histiocytoma after treatment with radiation and chemotherapy. Both secondary malignancies arose within the irradiated field after a latent period rangig from 9.5 years in the first to 21 years in the second case. A review of the pertinent literature is given and previous reports of malignant tumors and leukemias following therapy for Hodgkin's disease are summarized.     

Schwannomas and Their Pathogenesis

Schwannomas may occur spontaneously, or in the context of a familial tumor syndrome such as neurofibromatosis type 2 (NF2), schwannomatosis and Carney's complex. Schwannomas have a variety of morphological appearances, but they behave as World Health Organization (WHO) grade I tumors, and only very rarely undergo malignant transformation. Central to the pathogenesis of these tumors is loss of function of merlin, either by direct genetic change involving the NF2 gene on chromosome 22 or secondarily to merlin inactivation. The genetic pathways and morphological features of schwannomas associated with different genetic syndromes will be discussed. Merlin has multiple functions, including within the nucleus and at the cell membrane, and this review summarizes our current understanding of the mechanisms by which merlin loss is involved in schwannoma pathogenesis, highlighting potential areas for therapeutic intervention.     

Plexiform schwannoma of the ulnar nerve

Plexiform schwannoma is a rare benign neurogenic tumour; we report a case that arose in the ulnar nerve of a 59-year-old woman. Exploration showed a continuous multinodular tumour that involved the ulnar nerve from the hand to the upper arm; the length of the tumour was 35 cm.