Cystic dissemination of choroid plexus papilloma: illustrative cases

Abstract

BACKGROUND Choroid plexus papillomas are benign tumors of the choroid plexus. Although typically focal, they can metastasize. Rarely, patients may present with numerous cystic lesions throughout the craniospinal axis. OBSERVATIONS The authors present three cases of pathologically confirmed fourth ventricular World Health Organization (WHO) grade 1 choroid plexus papillomas presenting with numerous cystic lesions throughout the craniospinal axis. Two cases were treated with only resection of the fourth ventricular mass; one was treated with a partial cyst fenestration. During follow-up, there was only mild interval growth of the cystic lesions over time, and all patients remained asymptomatic from their cystic lesions. The authors summarize five additional cases of cystic dissemination in the published literature and discuss hypotheses for the pathophysiology of this rare presentation. LESSONS Choroid plexus papillomas may present with numerous, widely disseminated cystic lesions within the craniospinal axis. Thus, the authors recommend preoperative and routine imaging of the entire neuroaxis in patients with choroid plexus tumors, regardless of WHO grade. Although the role of adjuvant therapy and cyst fenestration in the treatment of these lesions remains unclear, watchful waiting may be indicated, especially in asymptomatic patients, because the lesions often demonstrate slow, if any, growth over time.

Metastatic choroid plexus papilloma presenting as a sellar mass: A case report and literature review

“…Johnson et al reviewed a series of eight such patients. [6] Six patients had primary solitary lesions (five of which were in the 4 th ventricle), two of which had multiple solid lesions. Cystic lesions were widely distributed throughout subarachnoid spaces, including the spine.…”

“…[ 7 ] The lateral ventricles are the most common location in children, while 4 th ventricular tumors are more common in adults. [ 6 ] Symptoms vary based on location and regional mass effect; however, hydrocephalus is common due to cerebrospinal fluid (CSF) flow obstruction and/or overproduction. Signs of elevated intracranial pressure, including headache, nausea, vomiting, cranial nerve deficits, gait imbalance, and altered mental status are commonly seen.…”

“…Cases of leptomeningeal spread and the formation of numerous cystic lesions in both the brain and spine have been reported. [ 6 , 12 , 13 ]…”

“…Cases of leptomeningeal spread and the formation of numerous cystic lesions in both the brain and spine have been reported. [6,12,13] Here, we present a 57-year-old patient with prior 4 th ventricular CPP with a solid distant metastasis to the sella, treated with supraorbital craniotomy for resection.…”