| Summary: | Choroid plexus tumors (CPTs) are uncommon intraventricular tumors of neuroectodermal origin derived from choroid plexus epithelium. They account for only approximately 0.4% - 0.6% of all intracranial tumors. CPTs occur predominantly in the lateral ventricle in children in contrast with adults who have primarily infratentorial lesions. The World Health Organization (WHO) classification of central nervous system tumors divided CPTs in to choroid plexus papilloma (CPP; WHO I), atypical choroid plexus papilloma (APP; WHO II) and choroid plexus carcinoma (CPC; WHO III). CPP is generally a histologically benign, slow-growing tumor; however malignant evolution may occur in 10% - 30% of cases, mainly in the lateral ventricle. There are few studies and reports of pediatric patients with infratentorial CPTs and particularly those with CPT in the cerebellopontine angle (CPA). CPTs arise from the choroid plexus. The choroid plexus of the fourth ventricle consists of two symmetrical parts located in the roof of the ventricle and protruding through the foramina of Luschka and Magendie. PICA supplies most of the choroid plexus in the roof and the median opening of the fourth ventricle, whereas anterior inferior cerebellar artery (AICA) supplies the portion of the plexus in the CPAs and the adjacent part of the lateral recess. Cranial nerve preservation at the CPA during the tumor resection is essential to avoid postoperative complications. In this video, a case of primary extraventricular CPP in the left CPA successfully resected with the retrosigmoid approach is presented.
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