TY - JOUR
T1 - Cystic dissemination of choroid plexus papilloma
T2 - illustrative cases
AU - Johnson, Gabrielle W.
AU - Mian, Ali Y.
AU - Dahiya, Sonika
AU - Rich, Keith
AU - Chicoine, Michael
AU - Limbrick, David D.
N1 - Publisher Copyright:
© 2022 The authors.
PY - 2022/11
Y1 - 2022/11
N2 - 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.
AB - 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.
KW - choroid plexus papilloma
KW - choroid plexus tumor
KW - cystic dissemination
KW - dissemination
UR - http://www.scopus.com/inward/record.url?scp=85165172434&partnerID=8YFLogxK
U2 - 10.3171/CASE22360
DO - 10.3171/CASE22360
M3 - Review article
C2 - 36377129
AN - SCOPUS:85165172434
SN - 2694-1902
VL - 4
JO - Journal of Neurosurgery: Case Lessons
JF - Journal of Neurosurgery: Case Lessons
IS - 20
M1 - CASE22360
ER -