Primary Cerebral Angiitis Containing Marked Xanthoma Cells With Massive Intraparenchymal Involvement-Case Report-

Abstract

A 27-year-old woman was referred to our hospital with mild disorientation, bilateral abducens nerve palsy, and mild left hemiparesis. Magnetic resonance (MR) imaging revealed diffuse mass lesions resembling malignant glioma in the right frontal intraparenchymal region, with enhancement of multiple meningeal and intraparenchymal nodules. Partial resection of the frontal lesion was performed. Histological examination revealed that the specimens consisted of brain tissue, with marked perivascular infiltration of histiocytes and sheets of xanthomatous cells. The diagnosis was primary cerebral angiitis containing marked xanthoma cells. Steroid therapy was administered over 1 week. MR imaging showed that the remaining lesions resolved gradually, and had disappeared 2 years after surgery. No neurological symptoms or recurrence of the tumor has been observed during the 6-year period since the operation.

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review

“…The present patient had a single, tumor-like enhancing lesion associated with extensive vasogenic edema on MR imaging. Conversely, PACNS presenting multiple lesions that also mimic aggressive multifocal cerebral neoplasm have been reported (26, 31–34). A retrospective study of 10 tumor-like PACNS cases in France found that the number of patients with single and multiple lesions was similar (4 and 3 patients, respectively) (35).…”

Langerhans Cell Histiocytosis Causing Central Nervous System Vasculitis Leading to Stroke in a Two-year-old Boy: A Case Report

“…Though uncommon in the pediatric population, there are reports in adults of histiocytic disorders leading to CNS vasculitis (Table 1) [9-10]. Vasculitides in children is a rare condition as a whole, occurring in no more than 53 in 100,000 (12401245, 15517648).…”

Mass‐forming primary angiitis of central nervous system with Rosai‐Dorfmann disease‐like massive histiocytosis with emperipolesis

“…A beaded appearance of the cerebral arteries seen on angiography might be a most supportive feature for vasculitis, reflecting variable degrees of stenosis or occlusion, however, these findings are rarely evident in PACNS. The PACNS showing tumor‐like images with mass effect are rare but have been reported, which usually show T2 high, heterogeneously enhancing lesions . Those previously reported mass‐forming PACNS cases were summarized in Table .…”