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[后颅窝血管外皮细胞瘤:病例报告]

[Hemangiopericytoma of the posterior fossa: case report].

作者信息

Gutiérrez-González R, Boto G R, Pérez-Zamarrón A, Rivero-Garvía M

机构信息

Servicio de Neurocirugía, Hospital Clínico San Carlos, Madrid, Spain.

出版信息

Neurocirugia (Astur). 2008 Oct;19(5):446-52.

Abstract

Hemangiopericytoma is an uncommon mesenchymal neoplasm arising from Zimmerman's pericytes, which usually locates in soft tissues. Meningeal hemangiopericytoma accounts for less than 1% of all intracranial tumours. Typically, it behaves aggressively, showing distinct tendency to recur locally or distantly along the neural axis and to present extraneural metastases. We describe a 74-year-old patient who presented unspecific symptoms and whose physical exam revealed a painless retroauricular mass which was adhered to skin. Neuroimaging studies showed a large posterior fossa tumour with intense enhancement after contrast infusion that caused striking occipital-mastoid osteolysis and which was exclusively fed by external carotid artery branches. The patient underwent gross total resection of the tumour, and once the histological diagnosis of hemangiopericytoma was confirmed, she underwent initial adjuvant radiotherapy. Sixteen months after surgery, the patient remains recurrence free. The treatment of choice of intracranial hemangiopericytoma is gross total resection, which must be attempted when technically feasible, followed by adjuvant radiotherapy providing total doses over 50 Gy. This combination has demonstrated increasing recurrence- free interval in these patients. Close and longterm follow-up is mandatory in order to achieve early diagnosis of recurrence or metastases in these patients, since they can appear several years, even decades, after initial proper treatment.

摘要

血管外皮细胞瘤是一种罕见的间叶性肿瘤,起源于齐默尔曼周细胞,通常位于软组织中。脑膜血管外皮细胞瘤占所有颅内肿瘤的比例不到1%。典型情况下,其行为具有侵袭性,表现出明显的局部或沿神经轴远处复发以及出现神经外转移的倾向。我们描述了一名74岁的患者,该患者出现非特异性症状,体格检查发现一个与皮肤粘连的无痛性耳后肿块。神经影像学研究显示后颅窝有一个大肿瘤,注入造影剂后有明显强化,导致枕骨-乳突显著骨质溶解,且仅由颈外动脉分支供血。患者接受了肿瘤全切术,在组织学诊断为血管外皮细胞瘤得到证实后,接受了初始辅助放疗。手术后16个月,患者无复发。颅内血管外皮细胞瘤的首选治疗方法是全切术,在技术可行时必须尝试,随后进行总剂量超过50 Gy的辅助放疗。这种联合治疗已证明可延长这些患者的无复发生存期。由于这些患者在最初适当治疗后数年甚至数十年可能出现复发或转移,因此必须进行密切和长期随访,以便早期诊断复发或转移。

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