Background: Metastatic renal cell carcinoma (RCC) of the choroid plexus is an exceedingly rare condition, with only 35 reported cases to date. Surgical resection of these tumors poses a unique challenge to neurosurgeons since evidence-based treatment guidelines are yet to be designed. Case Description: The authors describe the case of a 58-year-old woman presenting with progressive neurological deterioration 5 years after a right nephrectomy for a WHO 2016 Stage I RCC. A head, contrast-enhanced, and magnetic resonance revealed signs of obstructive hydrocephalus and a homogeneously contrast-enhancing 5 cm mass located in the trigone of the right lateral ventricle. Furthermore, a search of the literature was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. After screening for duplicates, 35 publications met the eligibility criteria. Finally, 17 manuscripts were included for analysis. Moreover, a detailed description of an illustrative case is provided. The median age at diagnosis for intraventricular metastasis from RCC was 62.9 years, showing a slight female prevalence. The lateral ventricles were reported as the most frequent location with only one patient presenting with obstructive hydrocephalus caused by the obliteration of Monro foramen. Management options included either open craniotomy or radiosurgery. Conclusion: The management of choroid plexus metastasis from RCC is still controversial with various authors proposing different treatment strategies. In this article, in addition to an in-depth case description, a qualitative review of the literature on metastatic RCCs of the choroid plexus using the PRISMA is provided.
