A 5-year-old child presenting with tumor-like primary angiitis of the central nervous system

Xiuwei Zhuo; Weixing Feng; Ji Zhou; Weihua Zhang; Shuai Gong; Fang Fang; Jiuwei Li

doi:10.1002/ped4.12322

A 5-year-old child presenting with tumor-like primary angiitis of the central nervous system

Pediatr Investig. 2022 Apr 20;6(2):140-143. doi: 10.1002/ped4.12322. eCollection 2022 Jun.

Authors

Xiuwei Zhuo¹, Weixing Feng¹, Ji Zhou¹, Weihua Zhang¹, Shuai Gong¹, Fang Fang¹, Jiuwei Li¹

Affiliation

¹ Department of Neurology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China.

Abstract

Introduction: Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the CNS. A small proportion of the lesions may present as a tumor-like mass, which is rarely seen in children.

Case presentation: A 5-year-old girl was admitted to our hospital because of an intermittent headache. Brain imaging suggested a space-occupying lesion in the right cerebral hemisphere. The final diagnosis was PACNS with a lymphocytic pattern by stereotactic brain biopsy. Her condition improved after immunotherapy.

Conclusion: Pediatricians should consider the possibility of PACNS when encountering intracranial tumor-like lesions. Early diagnosis of tumor-like PACNS and prompt immunotherapy could improve the long-term prognosis and avoid surgery.

Keywords: Central nervous system; Children; Primary angiitis; Tumor.

Publication types

Case Reports