Hydrocephalus secondary to choroid plexus tumors and choroid plexus hyperplasia

Abstract

Choroid plexus tumors (CPTs), namely, papilloma (CPP) and carcinoma (CPC), often present with hydrocephalus. CPT may lead to hydrocephalus secondary to several mechanisms: CSF overproduction, CSF pathway obstruction, and CSF malabsorption. Tumor locations are more prevalent in the lateral ventricle as opposed to the fourth ventricle. Both tumor subtypes are more prevalent in infantile age groups. The classical radiological appearance of CPP is of a cauliflower-like lesion, isointense on T1, with brisk and homogeneous enhancement. Complete surgical resection (gross total resection, GTR) is the cornerstone of treatment for all CPT. Super-selective embolization of the feeding arteries via the anterior or posterior choroidal arteries, using various materials, has been shown to significantly reduce blood loss and improve the surgeon's resection control and extent of resection. It has been shown that hydrocephalus may persist even following complete tumor resection; thus careful follow-up and a low threshold for CSF diversion are needed. CPC is one of the characteristic pediatric tumors of Li-Fraumeni syndrome (LFS), a familial cancer predisposition syndrome characterized by a high frequency of malignant tumors at a young age. TP53 germline mutations are the genetic hallmark of LFS. CPP is considered a surgical disease, and GTR is the sole treatment needed (except for CSF considerations). However, CPC should be treated with adjuvant treatments, including mainly chemotherapeutic protocols, sometimes added by radiotherapy.