Li Cheng-Bei, Xie Ming-Guo, Ma Jun-Peng, Wang Liang, Hao Shu-Yu, Zhang Li-Wei, Jia Wang, Jia Gui-Jun, Zhang Jun-Ting, Li Da, Li Huan, Wu Zhen
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China.
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China.
World Neurosurg. 2018 May;113:1-13. doi: 10.1016/j.wneu.2018.01.207. Epub 2018 Feb 7.
Primary intracranial angioleiomyoma is a rare and distinct neoplasm. Only 29 cases have been reported previously, and we aimed to investigate the clinical and radiopathologic features of these lesions.
Medical records and radiographs of 8 patients (7 male and 1 female; mean age: 48.7 years) at our institution were reviewed retrospectively. Patient follow-up and a literature review were performed.
The most common preoperative symptom was a visual defect (n = 2), followed by diplopia (n = 1) and abducens paralysis (n = 1). Three patients were asymptomatic. The parasellar area (particularly the cavernous sinus) was the predilection site (n = 4; 50.0%). Radiographically, all lesions were solid without cystic degeneration. All lesions appeared with T1 hypointensity and T2 hyperintensity, and they were gradually heterogeneously enhanced after the administration of gadolinium. Complete resection was achieved in 7 patients (87.5%) without recurrence after 26.8 months of follow-up. Mitosis was rarely observed, and the Ki-67 labeling index was less than 1%; pathologically, the cavernous type was the most common.
Primary intracranial angioleiomyomas were prevalent in middle-aged men, and they usually involved the cavernous sinus and were frequently pathologically identified as the cavernous type. Preoperative symptoms varied depending on lesion location. The preoperative diagnosis of primary intracranial angioleiomyomas is difficult without pathology. Digital subtraction angiography and preoperative embolization are useful for differential diagnosis and surgery. Given the indolent biology of these tumors, a favorable outcome can be achieved using total resection without recurrence. A larger sample size with long-term follow-up is needed to verify our findings.
原发性颅内血管平滑肌瘤是一种罕见且独特的肿瘤。此前仅报道过29例，我们旨在研究这些病变的临床和放射病理学特征。
回顾性分析了我院8例患者（7例男性，1例女性；平均年龄：48.7岁）的病历和影像学资料。对患者进行了随访并进行了文献回顾。
最常见的术前症状是视力缺损（n = 2），其次是复视（n = 1）和展神经麻痹（n = 1）。3例患者无症状。鞍旁区域（尤其是海绵窦）是好发部位（n = 4；50.0%）。影像学上，所有病变均为实性，无囊性变。所有病变在T1加权像上呈低信号，T2加权像上呈高信号，注射钆对比剂后逐渐呈不均匀强化。7例患者（87.5%）实现了完全切除，随访26.8个月后无复发。很少观察到有丝分裂，Ki-67标记指数小于1%；病理上，海绵状型最常见。
原发性颅内血管平滑肌瘤在中年男性中较为常见，通常累及海绵窦，病理上常被鉴定为海绵状型。术前症状因病变位置而异。若无病理检查，原发性颅内血管平滑肌瘤的术前诊断困难。数字减影血管造影和术前栓塞对鉴别诊断和手术有用。鉴于这些肿瘤的生物学行为惰性，采用全切除可获得良好预后且无复发。需要更大样本量和长期随访来验证我们的发现。
