Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumor-like lesion with unknown pathogenesis. It is likely under-reported due to diagnostic challenges including the nonspecific radiographic features, lack of diagnostic markers, and often asymptomatic nature of the lesions.

We performed detailed examination of 11 CAPNON specimens diagnosed by histopathology, with the help of electron microscopy and immunohistochemistry.

Electron microscopy revealed the presence of fibrillary materials consistent with neurofilaments. In addition to some entrapped axons at the periphery of CAPNONs, we discovered that all specimens stained positive for neurofilament-light (NF-L) within the granular amorphous cores, but not neurofilament-phosphorylated (NF-p). CAPNONs also showed variable infiltration of CD8+ T-cells and a decreased ratio of CD4/CD8+ T-cells, suggesting an immune-mediated process in the pathogenesis of CAPNON.

NF-L and CD4/CD8 immunostains may serve as diagnostic markers for CAPNON and shed light on its pathogenesis.

Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumefactive lesion. CAPNONs can mimic calcified meningiomas at the skull base.

Here, we report two cases of CAPNON and present a systematic review of the literature on skull base CAPNONs, to compare CAPNONs with calcified meningiomas.

Case 1: A 57-year-old man presented with right-sided lower cranial neuropathies and gait ataxia. He underwent a subtotal resection of a right cerebellopontine angle lesion, with significant improvement of his gait ataxia. However, his cranial neuropathies persisted. Pathological examination of the lesion was diagnostic of CAPNON, with the entrapped nerve fibers identified at the periphery of the lesion, correlating with the patient’s cranial neuropathy. Case 2: A 70-year-old man presented with progressive headache, gait difficulty, and cognitive impairment. He underwent a frontotemporal craniotomy for a near-total resection of his right basal frontal CAPNON. He remained neurologically stable 7 years after the initial resection without evidence of disease recurrence. We analyzed 24 reported CAPNONs at the skull base in our systematic review of the literature. Cranial neuropathies were present in 11 (45.8%) patients. Outcomes regarding cranial neuropathies were documented in six patients: two had sacrifice of the nerve function with surgical approaches and four had persistent cranial neuropathies.

While CAPNON can radiologically and grossly mimic calcified meningiomas, they are two distinctly different pathologies. CAPNONs located at the skull base are commonly associated with cranial neuropathies, which may be difficult to reverse despite surgical intervention.