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尽管进行了立体定向放射外科治疗,仍死于恶性小脑桥脑角蝾螈瘤。病例报告。

Death from a malignant cerebellopontine angle triton tumor despite stereotactic radiosurgery. Case report.

作者信息

Comey C H, McLaughlin M R, Jho H D, Martinez A J, Lunsford L D

机构信息

Department of Neurological Surgery, University of Pittsburgh Medical Center, Pennsylvania, USA.

出版信息

J Neurosurg. 1998 Oct;89(4):653-8. doi: 10.3171/jns.1998.89.4.0653.

DOI:10.3171/jns.1998.89.4.0653
PMID:9761063
Abstract

Malignant vestibular nerve tumors are rare: to date, only three cases have been reported in the literature. The authors report a case of an eighth cranial nerve tumor that progressed 5 years after stereotactic radiosurgery. The patient was a 44-year-old man who underwent stereotactic radiosurgery for a 27-mm cerebellopontine angle tumor that was discovered on investigation of tinnitus and hearing loss. He developed facial weakness after 5 years, and repeated imaging revealed tumor enlargement. Despite complete microsurgical excision, the tumor rapidly recurred locally and subsequently disseminated within the neuraxis. The patient died 1 year after tumor progression was detected. Histopathological analysis revealed a malignant spindle cell neoplasm with frequent mitotic figures. The presence of positive rhabdoid elements on immunohistochemical studies confirmed that it was a triton tumor. The authors review the relevant literature concerning the classification and management of malignant vestibular nerve tumors and discuss the implications of tumor progression after stereotactic radiosurgery.

摘要

恶性前庭神经肿瘤较为罕见

迄今为止,文献中仅报道过3例。作者报告了1例在立体定向放射外科治疗5年后进展的第八颅神经肿瘤病例。患者为一名44岁男性,因耳鸣和听力损失检查发现27mm的小脑脑桥角肿瘤而接受立体定向放射外科治疗。5年后他出现面部无力,重复成像显示肿瘤增大。尽管进行了完整的显微手术切除,但肿瘤迅速在局部复发,随后在神经轴内播散。在检测到肿瘤进展1年后患者死亡。组织病理学分析显示为具有频繁有丝分裂象的恶性梭形细胞瘤。免疫组织化学研究中横纹肌样阳性成分的存在证实其为蝾螈瘤。作者回顾了有关恶性前庭神经肿瘤分类和治疗的相关文献,并讨论了立体定向放射外科治疗后肿瘤进展的影响。

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