瘤样原发性中枢神经系统血管炎的诊治分析（附6例报道并文献复习）

目的 探讨瘤样原发性中枢神经系统血管炎（TLP-PACNS）的诊断、治疗及预后，以提高对该病的认识。方法 回顾性分析2011年3月～2021年2月手术治疗的6例TLP-PACNS的临床资料，并对相关文献进行复习。结果 术前头颅MRI显示单发局限性病灶3例，考虑胶质瘤；多发病灶3例，考虑转移瘤。6例均行开颅病灶切除术，术后病理证实为PACNS。2例术后给予激素治疗后明显好转；1例多发病灶术后激素治疗后无变化，加用环磷酰胺后好转；3例单发病灶术后自然缓解；随访3个月～2年无复发。结论 TLP-PACNS是一种非常少见的疾病，临床表现和影像学表现缺乏特异性，术前易误诊，病理检查是诊断金标准，激素治疗是一种有效的方法。

Diagnosis and treatment of tumor-like presentation of primary angiitis of the central nervous system (report of 6 cases and literature review)

Objective To investigate the diagnosis, treatment, and prognosis of tumor-like presentation (TLP) primary angiitis of the central nervous system (PACNS) to improve the understanding of this disease. Methods The clinical data of 6 patients with TLP-PACNS who underwent surgery from March 2011 to February 2021 were retrospectively analyzed, and the relevant literatures were reviewed. Results Preoperative MRI showed single localized lesion in 3 patients who were misdiagnosed with glioma, and multiple lesions in 3 patients who were misdiagnosed with metastatic tumors. All patients received craniotomy and the postoperative pathological results showed PACNS in all patients. Two patients were treated with hormone therapy after the operation. One patient with multiple lesions showed no change after hormone therapy, but improved after cyclophosphamide. Three patients with single lesion were naturally relieved after the operation. There was no recurrence during the follow-up (range, 3 months~2years). Conclusions TLP-PACNS, lacking specific clinical and imaging manifestations, is a very rare disease and is easily misdiagnosed preoperatively. Pathological examination is the gold standard of diagnosis, and hormone therapy is an effective method for TLP-PACNS.