Department of Neurosurgery, University of Toronto, Toronto, Ontario, Canada.
Division of Neurosurgery, Department of Surgery, McMaster University, Hamilton, Ontario, Canada.
World Neurosurg. 2021 Apr;148:165-176. doi: 10.1016/j.wneu.2021.01.076. Epub 2021 Jan 26.
Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumefactive lesion that can develop anywhere within the neuraxis. The incidence is likely underreported, given its nonspecific imaging features and because CAPNON has often been overwhelmed by the presence of comorbid disease. CAPNON is typically diagnosed by the histopathological examination findings. However, the histopathological diagnosis is often challenging owing to the existence of similar calcifying pathological entities. Although the pathogenesis of CAPNON has remained elusive, emerging evidence supports a reactive proliferative and immune-mediated process involving the aggregation of neurofilament light chain protein and the infiltration of immune cells. The management of CAPNON is largely dependent on the symptoms, which are mainly related to the location and associated mass effects. Maximal surgical resection will result in excellent patient outcomes with rare recurrence, especially in patients presenting with epilepsy. The discovery of neurofilament light chain protein within CAPNON suggests that neurofilament might be implicated in the pathogenesis of CAPNON, serve as an immunohistochemical marker to improve the diagnostic accuracy of CAPNON, and hold therapeutic potential for the treatment of CAPNON.
中枢神经系统钙化假性肿瘤（CAPNON）是一种罕见的肿块样病变，可发生在中枢神经系统的任何部位。由于其影像学特征不具有特异性，且 CAPNON 常因合并疾病的存在而被掩盖，因此其发病率可能被低估。CAPNON 通常通过组织病理学检查结果来诊断。然而，由于存在类似的钙化性病变实体，组织病理学诊断通常具有挑战性。尽管 CAPNON 的发病机制仍不清楚，但新出现的证据支持涉及神经丝轻链蛋白聚集和免疫细胞浸润的反应性增殖和免疫介导过程。CAPNON 的治疗在很大程度上取决于症状，这些症状主要与病变位置和相关的占位效应有关。最大限度的手术切除将带来极好的患者预后，复发罕见，特别是在癫痫患者中。CAPNON 中神经丝轻链蛋白的发现表明神经丝可能与 CAPNON 的发病机制有关，可作为免疫组织化学标志物，提高 CAPNON 的诊断准确性，并为 CAPNON 的治疗提供治疗潜力。
