Tashjian Vartan S, Khanlou Negar, Vinters Harry V, Canalis Rinaldo F, Becker Donald P
Division of Neurosurgery, David Geffen School of Medicine at UCLA, NPI, Box 957039, Los Angeles, CA 90095-7039, USA.
Surg Neurol. 2009 Sep;72(3):290-5. doi: 10.1016/j.surneu.2008.06.016. Epub 2008 Sep 10.
Intracranial hemangiopericytoma represents a rare intracranial tumor that is typically difficult to distinguish from meningioma based on clinical presentation and radiographic findings. These inherently aggressive neoplasms have been observed to occur in numerous intracranial compartments; however, isolated involvement of the CPA is essentially unreported. The authors present a case of a young lady with presumed right acoustic schwannoma, which proved to be HPC on histopathology. The case is described; and a review of the literature pertaining to the diagnosis, optimal management, and follow-up for these lesions is provided.
A 37-year-old Asian woman presented with a 7-month history of right ear and mandible numbness, as well as subjective hearing loss involving the right ear. Magnetic resonance imaging demonstrated the presence of a homogeneously enhancing extraaxial lesion in the right CPA, radiographically suggestive of an acoustic schwannoma. The lesion proved to be an intracranial HPC on histologic sections. Review of the neurosurgical literature yielded only one prior detailed account of HPC confined to the CPA. The patient underwent right retrosigmoid craniotomy for gross total resection of the mass, followed by stereotactic radiotherapy several weeks postoperatively.
Given the fundamentally different treatment approach for HPCs over other more common CPA tumors, it is imperative that the treating surgeon consider this rare diagnosis when evaluating patients with lesions localized to this area. Specifically, gross total resection, followed by adjuvant SRT, provides patients with the highest probability for disease-free survival, based on current evidence in the neurosurgical literature.
颅内血管外皮细胞瘤是一种罕见的颅内肿瘤,基于临床表现和影像学检查结果,通常很难与脑膜瘤区分开来。这些本质上具有侵袭性的肿瘤已被观察到发生在多个颅内腔室;然而,孤立累及小脑脑桥角(CPA)的情况基本上未见报道。作者报告了一例年轻女性病例,最初推测为右听神经鞘瘤,组织病理学检查证实为血管外皮细胞瘤。本文描述了该病例,并对有关这些病变的诊断、最佳治疗和随访的文献进行了综述。
一名37岁的亚洲女性,有7个月的右耳及下颌麻木病史,以及右耳主观听力丧失。磁共振成像显示右侧CPA区有一个均匀强化的轴外病变,影像学表现提示为听神经鞘瘤。组织学切片显示该病变为颅内血管外皮细胞瘤。回顾神经外科文献,仅发现一篇之前关于局限于CPA的血管外皮细胞瘤的详细报道。该患者接受了右侧乙状窦后开颅术以全切肿瘤,术后数周接受立体定向放射治疗。
鉴于血管外皮细胞瘤与其他更常见的CPA肿瘤的治疗方法根本不同,治疗外科医生在评估该区域病变患者时必须考虑到这种罕见的诊断。具体而言,根据神经外科文献的现有证据,全切肿瘤后辅助立体定向放射治疗为患者提供了最高的无病生存概率。