Tang Hailiang, Hua Wendi, Wang Ying, Xiong Ji, Cheng Haixia, Xu Ming, Xu Jian, Zhong Ping
Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China.
Department of Operation Center, Huashan Hospital, Fudan University, Shanghai, China.
Transl Cancer Res. 2024 Jul 31;13(7):3922-3927. doi: 10.21037/tcr-24-151. Epub 2024 Jul 26.
Angioleiomyoma is a benign lesion of mesenchymal origin, which always occurs in the uterine system. Pathologically, angioleiomyoma is usually composed of well-differentiated smooth muscle cells with few mitotic features. However, primary intracranial angioleiomyoma represents an exceedingly rare tumor, since the first case reported in 1994.
Here, we reported a case of primary intracranial angioleiomyoma, which mimicking meningioma in pre-operative images. The patient was a 42-year-old male, presented with dizziness and unsteady walking for about 6 months, without symptoms of cranial nerve deficit. Head computer tomography scan showed a well-defined lesion adjacent to right brain stem with high intensity. Contrast brain magnetic resonance imaging (MRI) scan exhibited an extra-axial mass with homogeneous enhancement located at the right pontine, presented as meningioma features; however, other tumors including lymphoma should be differentiated as well. The patient underwent sub-temporal craniotomy for the tumor resection. Histological analysis confirmed the diagnosis of angioleiomyoma. Follow-up brain MRI scan (6 months after surgery) showed total resection of the lesion without residual.
In summary, primary intracranial angioleiomyoma is rare. Thus, diagnosis and differential diagnosis are important before surgical resection, which was mimicking meningioma in our case. Pathological analysis could reveal spindle shaped cells with few mitotic features, and confirm the diagnosis of angioleiomyoma. Currently, the optimal therapy for primary intracranial angioleiomyoma is surgical resection, and adjuvant radiation therapy for the residual tumor. However, long-term prognosis of the disease should be monitor in the future.
血管平滑肌瘤是一种间叶源性良性病变，通常发生于子宫系统。病理上，血管平滑肌瘤通常由分化良好的平滑肌细胞组成，有丝分裂特征较少。然而，原发性颅内血管平滑肌瘤是一种极为罕见的肿瘤，自1994年首例报道以来。
在此，我们报告一例原发性颅内血管平滑肌瘤，其在术前影像中酷似脑膜瘤。患者为一名42岁男性，出现头晕和行走不稳约6个月，无颅神经缺损症状。头部计算机断层扫描显示右脑干旁有一个边界清晰的高密度病变。对比增强脑磁共振成像（MRI）扫描显示位于右脑桥的一个轴外肿块，呈均匀强化，表现为脑膜瘤特征；然而，也应鉴别包括淋巴瘤在内的其他肿瘤。患者接受颞下开颅肿瘤切除术。组织学分析确诊为血管平滑肌瘤。术后6个月的脑部MRI随访扫描显示病变全切无残留。
总之，原发性颅内血管平滑肌瘤罕见。因此，在手术切除前进行诊断和鉴别诊断很重要，在我们的病例中其酷似脑膜瘤。病理分析可显示有丝分裂特征较少的梭形细胞，并确诊血管平滑肌瘤。目前，原发性颅内血管平滑肌瘤的最佳治疗方法是手术切除，对残留肿瘤进行辅助放疗。然而，未来应监测该疾病的长期预后。
