Background/Purpose: The aim of this study was to determine the frequency of ML in a large cohort of patients with PCNSV and compare the presenting clinical, laboratory, and imaging features in those with ML to those without.

Methods: We retrospectively studied a cohort of 191 consecutive patients with PCNSV who were seen at the Mayo Clinic, Rochester, MN over a 35- year period (1982-2017). The diagnosis of PCNSV was based on brain/spinal cord biopsy or cerebral angiography. Cerebral biopsy specimens were reviewed by one pathologist (CG) without knowledge of clinical information. Comprehensive information about clinical manifestations at presentation and during the follow-up, laboratory investigations, radiological imaging, results of CNS biopsy or autopsy, type of, duration of, and response to treatment, number of relapses, functional status at last follow-up, and cause of death were recorded. We compared PCNSV patients with tumor-like presentation to those without.

Results: 13/191 (6.8%) patients had tumor-like presentation. In all 13 patients PCNSV diagnosis was established by cerebral biopsy (stereotactic in 10, open-wedge in 3). 4 patients had cerebral angiography, and vasculitis was suggested in one patient. A granulomatous inflammatory histologic pattern was found in 11 biopsies, accompanied by vascular deposits of β-amyloid peptide in 7. In the other 2 biopsies a lymphocytic vasculitis was observed. The 13 patients with tumor-like presentation were compared with the 178 patients without. The patients with ML were more frequently males (77% vs 44%, p = 0.04), were less likely to present with transient ischemic attacks (TIA) (0 vs 27.5%, p = 0.023) and more likely to present with seizures (46% vs 17%, p = 0.022). No significant differences in the CSF findings and ESR levels (normal in 100% and 82% of the patients, respectively) at diagnosis were observed in the two groups. Gadolinium-enhancing lesions were more frequently observed in patients with ML (77% vs 37%, p = 0.007). The frequencies of PCNSV recurrence (38% vs 29%), patients not requiring therapy at last follow-up (15% vs 25%), response to therapy (100% vs 74%), and poor outcomes (modified Rankin disability score ≥ 4) at last followup ( 8% vs 26%) were not significantly different in the two groups. No differences in survival were observed between the 2 groups (p = 0.57). Considering all 191 patients, univariate Cox proportional hazards modeling showed an increased mortality rate in those with increasing age (hazard ratio, HR, 1.4), cerebral infarction on initial MRI (HR 2.95), and angiographic large vessel involvement (HR 3.2), while mortality rate was lower in those with gadolinium–enhancing lesions on MRI (HR 0.3).

Conclusion: Tumor-like presentation represents a small subgroup of patients with PCNSV, and are often associated with vascular deposits of β-amyloid at biopsy, have seizures as presenting manifestation and gadolinium-enhancing cerebral lesions on MRI. As in PCNSV without ML, treatment response and prognosis was favorable in most patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/primary-central-nervous-system-vasculitis-with-tumor-like-presentation/