Department of Neurosurgery, University of Illinois at Chicago, Chicago, Illinois, USA.
Department of Neurosurgery, University of Illinois at Chicago, Chicago, Illinois, USA.
World Neurosurg. 2020 Sep;141:284-290. doi: 10.1016/j.wneu.2020.05.157. Epub 2020 May 23.
Primary intracranial leiomyoma is a rare smooth muscle tumor often associated with Epstein-Barr virus (EBV), with <30 cases reported worldwide. These tumors commonly occur in patients with immunocompromised status, especially those with human immunodeficiency virus. In the present report, we have described the case of an EBV-associated leiomyoma at the cerebellopontine angle. The patient had presented with trigeminal neuralgia, which, to the best of our knowledge, is the first reported anatomical location and presentation for this tumor type.
A 41-year-old male patient had presented with right-sided facial pain in the V1 and V2 dermatomes and previous workup and imaging studies. The patient had undergone treatment of a presumed right-side cerebellopontine angle meningioma as determined by the magnetic resonance imaging characteristics (no biopsy). The patient subsequently underwent right-sided retrosigmoid craniotomy and gross total resection of the tumor. The postoperative period was uneventful with resolution of the trigeminal neuralgia. Histopathologic examination revealed spindle cell neoplasm with histopathologic and immunohistochemical features consistent with leiomyoma. The tumor cells were positive for smooth muscle actin and desmin and were negative for S100, SOX-10, epithelial membrane antigen, glial fibrillary acidic protein, progesterone receptor, CD31, CD34, and E-cadherin.
Primary intracranial leiomyomas are rare tumors associated with EBV infection that occur in immunocompromised patients. These lesions should be considered in the differential diagnosis for patients with known immunocompromised status (e.g., human immunodeficiency virus), and tissue biopsy should be considered.
原发性颅内平滑肌瘤是一种罕见的平滑肌肿瘤，常与 Epstein-Barr 病毒（EBV）相关，全球报道的病例不足 30 例。这些肿瘤通常发生在免疫功能低下的患者中，尤其是人类免疫缺陷病毒感染者。本报告描述了一例发生于桥小脑角的 EBV 相关平滑肌瘤病例。患者表现为三叉神经痛，据我们所知，这是首例报道的该肿瘤类型的解剖部位和表现。
一名 41 岁男性患者出现右侧面部 V1 和 V2 皮区疼痛，此前曾进行过相关检查和影像学研究。根据磁共振成像特征（未行活检），患者被诊断为右侧桥小脑角脑膜瘤，并接受了治疗。随后，患者接受了右侧乙状窦后入路开颅肿瘤全切除手术。术后恢复顺利，三叉神经痛得到缓解。组织病理学检查显示梭形细胞肿瘤，具有平滑肌瘤的组织病理学和免疫组织化学特征。肿瘤细胞阳性表达平滑肌肌动蛋白和结蛋白，而 S100、SOX-10、上皮膜抗原、胶质纤维酸性蛋白、孕激素受体、CD31、CD34 和 E-钙黏蛋白均为阴性。
原发性颅内平滑肌瘤是一种罕见的与 EBV 感染相关的肿瘤，发生于免疫功能低下的患者中。对于已知免疫功能低下的患者（如人类免疫缺陷病毒感染者），应考虑将这些病变纳入鉴别诊断，应考虑进行组织活检。
