Choroid Plexus Tumors

  • Christina Snider
  • John H. Suh
  • Erin S. MurphyEmail author


Choroid plexus tumors (CPT) are rare tumors of the neuroepithelial tissue of the brain that are most common in young children. In adults, they arise most frequently in the fourth ventricle, often presenting with hydrocephalus due to overproduction of CSF. There are three histopathological classifications of choroid plexus tumors: choroid plexus papilloma (CPP), atypical choroid plexus papilloma (ACP), and choroid plexus carcinoma (CPC). Histology and extent of surgical resection are prognostic for choroid plexus tumors. Curative treatment for CPP and ACP is typically maximum surgical resection, although recurrence rates are higher in cases of ACP. CPP and ACP have better prognosis than CPC, which often requires multiple modalities for treatment.


Choroid plexus Choroid plexus papilloma Atypical choroid plexus papilloma Choroid plexus carcinoma Radiation therapy Stereotactic radiosurgery 


  1. 1.
    Turkoglu E, Kertmen H, Sanli AM, et al. Clinical outcome of adult choroid plexus tumors: retrospective analysis of a single institute. Acta Neurochir. 2014;156(8):1461–8.Google Scholar
  2. 2.
    Lam S, Lin Y, Cherian J, et al. Choroid plexus tumors in children: a population-based study. Pediatr Neurosurg. 2013;49(6):331–8.Google Scholar
  3. 3.
    McCall T, Binning M, Blumenthal DT, et al. Variations of disseminated choroid plexus papilloma: 2 case reports and a review of the literature. Surg Neurol. 2006;66(1):62–7.Google Scholar
  4. 4.
    World Health Organization. WHO classification of tumours of the central nervous system. Revised 4th ed. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization classification of tumours. Lyon: International Agency for Research on Cancer; 2016.Google Scholar
  5. 5.
    Wolff JEA, Sajedi M, Brant R, et al. Choroid plexus tumours. Br J Cancer. 2002;87(10):1086–91.Google Scholar
  6. 6.
    Aicardi J. Aicardi syndrome. Brain and Development. 2005;27(3):164–71.Google Scholar
  7. 7.
    Morigaki R, Pooh K-H, Shouno K, et al. Choroid plexus papilloma in a girl with hypomelanosis of Ito. J Neurosurg Pediatr. 2012;10(3):182–5.Google Scholar
  8. 8.
    Norman MG, Harrison KJ, Poskitt KJ, et al. Duplication of 9P and hyperplasia of the choroid plexus: a pathologic, radiologic, and molecular cytogenetics study. Pediatr Pathol Lab Med. 1995;15(1):109–20.Google Scholar
  9. 9.
    Sorrell AD, Espenschied CR, Culver JO, et al. TP53 testing and Li-Fraumeni syndrome: current status of clinical applications and future directions. Mol Diagn Ther. 2013;17(1):31–47.Google Scholar
  10. 10.
    Gozali AE, Britt B, Shane L, et al. Choroid plexus tumors; management, outcome, and association with the Li-Fraumeni syndrome: the Children’s Hospital Los Angeles (CHLA) experience, 1991–2010. Pediatr Blood Cancer. 2012;58(6):905–9.Google Scholar
  11. 11.
    Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol (Berl). 2016;131(6):803–20.Google Scholar
  12. 12.
    Cannon DM, Mohindra P, Gondi V, et al. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 2015;121(1):151–7.Google Scholar
  13. 13.
    Irsutti M, Thorn-Kany M, Arrué P, et al. Suprasellar seeding of a benign choroid plexus papilloma of the fourth ventricle with local recurrence. Neuroradiology. 2000;42(9):657–61.Google Scholar
  14. 14.
    Tabori U, Shlien A, Baskin B, et al. TP53 alterations determine clinical subgroups and survival of patients with choroid plexus tumors. J Clin Oncol. 2010;28(12):1995–2001.Google Scholar
  15. 15.
    Thomas C, Sill M, Ruland V, et al. Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups. Neuro Oncol. 2016;18(6):790–6.Google Scholar
  16. 16.
    Ruland V, Hartung S, Kordes U, et al. Choroid plexus carcinomas are characterized by complex chromosomal alterations related to patient age and prognosis. Genes Chromosomes Cancer. 2014;53(5):373–80.Google Scholar
  17. 17.
    Sun MZ, Ivan ME, Clark AJ, et al. Gross total resection improves overall survival in children with choroid plexus carcinoma. J Neurooncol. 2014;116(1):179–85.Google Scholar
  18. 18.
    Dangouloff-Ros V, Grevent D, Pagès M, et al. Choroid plexus neoplasms: toward a distinction between carcinoma and papilloma using arterial spin-labeling. AJNR Am J Neuroradiol. 2015;36(9):1786–90.Google Scholar
  19. 19.
    Jeibmann A, Wrede B, Peters O, et al. Malignant progression in choroid plexus papillomas. J Neurosurg. 2007;107(3 Suppl):199–202.Google Scholar
  20. 20.
    Wrede B, Liu P, Wolff JEA. Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol. 2007;85(3):345–51.Google Scholar
  21. 21.
    McEvoy AW, Harding BN, Phipps KP, et al. Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre. Pediatr Neurosurg. 2000;32(4):192–9.Google Scholar
  22. 22.
    Fitzpatrick LK, Aronson LJ, Cohen KJ. Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely resected? J Neurooncol. 2002;57(2):123–6.Google Scholar
  23. 23.
    Passariello A, Tufano M, Spennato P, et al. The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas. Childs Nerv Syst. 2015;31(7):1079–88.Google Scholar
  24. 24.
    Slater L-A, Hoffman C, Drake J, et al. Pre-operative embolization of a choroid plexus carcinoma: review of the vascular anatomy. Childs Nerv Syst. 2016;32(3):541–5.Google Scholar
  25. 25.
    Schneider C, Kamaly-Asl I, Ramaswamy V, et al. Neoadjuvant chemotherapy reduces blood loss during the resection of pediatric choroid plexus carcinomas. J Neurosurg Pediatr. 2015;16(2):126–33.Google Scholar
  26. 26.
    Wolff JE, Sajedi M, Coppes MJ, et al. Radiation therapy and survival in choroid plexus carcinoma. Lancet. 1999;353(9170):2126.Google Scholar
  27. 27.
    Mazloom A, Wolff JE, Paulino AC. The impact of radiotherapy fields in the treatment of patients with choroid plexus carcinoma. Int J Radiat Oncol. 2010;78(1):79–84.Google Scholar
  28. 28.
    Zaky W, Dhall G, Khatua S, et al. Choroid plexus carcinoma in children: The Head Start experience. Pediatr Blood Cancer. 2015;62(5):784–9.Google Scholar
  29. 29.
    Samuel TA, Parikh J, Sharma S, et al. Recurrent adult choroid plexus carcinoma treated with high-dose chemotherapy and syngeneic stem cell (bone marrow) transplant. J Neurol Surg A Cent Eur Neurosurg. 2013;74(Suppl 1):e149–54.Google Scholar
  30. 30.
    Mosleh O, Tabori U, Bartels U, et al. Successful treatment of a recurrent choroid plexus carcinoma with surgery followed by high-dose chemotherapy and stem cell rescue. Pediatr Hematol Oncol. 2013;30(5):386–91.Google Scholar
  31. 31.
    Kim I-Y, Niranjan A, Kondziolka D, et al. Gamma knife radiosurgery for treatment resistant choroid plexus papillomas. J Neurooncol. 2008;90(1):105–10.Google Scholar
  32. 32.
    Kamar FG, Kairouz VF, Nasser SM, et al. Atypical choroid plexus papilloma treated with single agent bevacizumab. Rare Tumors [Internet]. 2014;6(1). Available from: Cited 15 Jan 2017.
  33. 33.
    Bahar M, Kordes U, Tekautz T, et al. Radiation therapy for choroid plexus carcinoma patients with Li-Fraumeni syndrome: advantageous or detrimental? Anticancer Res. 2015;35(5):3013–7.Google Scholar
  34. 34.
    Wrede B, Hasselblatt M, Peters O, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol. 2009;95(3):383.Google Scholar
  35. 35.
    Grosu A-L, Oehlke O, Nieder C. Brain tumors. In: Grosu A-L, Nieder C, editors. Target volume definition in radiation oncology [Internet]. Berlin, Heidelberg: Springer; 2015. p. 1–21. Available from: Cited 17 Jan 2017.
  36. 36.
    Soussain C, Ricard D, Fike JR, et al. CNS complications of radiotherapy and chemotherapy. Lancet. 2009;374(9701):1639–51.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Christina Snider
    • 1
  • John H. Suh
    • 2
    • 3
  • Erin S. Murphy
    • 2
    • 3
    Email author
  1. 1.Cleveland Clinic Lerner College of MedicineClevelandUSA
  2. 2.Department of Radiation OncologyTaussig Cancer Institute, Cleveland ClinicClevelandUSA
  3. 3.Rose Ella Burkhardt Brain Tumor and Neuro-oncology CenterCleveland ClinicClevelandUSA

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