Calcifying Pseudoneoplasm of the Neuraxis: From Pathogenesis to Diagnostic and Therapeutic Considerations
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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.
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