Abstract
Choroid plexus tumors are rare tumors, predominantly found in pediatric patients. Lower grade tumors histologically resemble native choroid plexus while higher grade lesions are progressively more disordered and less differentiated. They are commonly associated with hydrocephalus and usually present with symptoms of that condition. Complete resection is typically curative in the case of low grade lesions. Higher grade lesions may be treated with adjuvant chemotherapy or radiotherapy. The hydrocephalus often resolves but in some cases may persist after resection and may require additional treatment.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Abdulkader MM, Mansour NH, Van Gompel JJ, Bosh GA, Dropcho EJ, Bonnin JM et al (2016) Disseminated choroid plexus papillomas in adults: a case series and review of the literature. J Clin Neurosci 32:148–154
Arasappa R, Danivas V, Venkatasubramanian G (2013) Choroid plexus papilloma presenting as schizophrenia: a case report. J Neuropsychiatry Clin Neurosci 25:E26–E27
Bahar M, Hashem H, Tekautz T, Worley S, Tang A, de Blank P et al (2017) Choroid plexus tumors in adult and pediatric populations: the Cleveland Clinic and University Hospitals experience. J Neuro-Oncol 132:427–432
Berger C, Thiesse P, Lellouch-Tubiana A, Kalifa C, Pierre-Kahn A, Bouffet E (1998) Choroid plexus carcinomas in childhood: clinical features and prognostic factors. Neurosurgery 42:470–475
Beschorner R, Waidelich J, Trautmann K, Psaras T, Schittenhelm J (2013) Notch receptors in human choroid plexus tumors. Histol Histopathol 28:1055–1063
Bettegowda C, Adogwa O, Mehta V, Chaichana KL, Weingart J, Carson BS et al (2012) Treatment of choroid plexus tumors: a 20-year single institutional experience. J Neurosurg Pediatr 10:398–405
Borja MJ, Plaza MJ, Altman N, Saigal G (2013) Conventional and advanced MRI features of pediatric intracranial tumors: supratentorial tumors. AJR Am J Roentgenol 200:W483–W503
Bostrom A, Bostrom JP, von Lehe M, Kandenwein JA, Schramm J, Simon M (2011) Surgical treatment of choroid plexus tumors. Acta Neurochir 153:371–376
Boyd MC, Steinbok P (1987) Choroid plexus tumors: problems in diagnosis and management. J Neurosurg 66:800–805
Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR (2015) Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neuro-Oncol 121:151–157
Chow E, Reardon DA, Shah AB, Jenkins JJ, Langston J, Heideman RL et al (1999) Pediatric choroid plexus neoplasms. Int J Radiat Oncol Biol Phys 44:249–254
Dandy WE (1922) Diagnosis, localization, and removal of tumours of the third ventricle. Bull Johns Hopkins Hosp 33:188–189
Dang L, Fan X, Chaudhry A, Wang M, Gaiano N, Eberhart CG (2006) Notch3 signaling initiates choroid plexus tumor formation. Oncogene 25:487–491
Davis LE, Cushing H (1925) Papillomas of the choroid plexus: with the report of six cases. Arch Neurol Psychiatr 13:681–710
Dhillon RS, Wang YY, McKelvie PA, O’Brien B (2013) Progression of choroid plexus papilloma. J Clin Neurosci 20:1775–1778
Doglietto F, Lauretti L, Tartaglione T, Gessi M, Fernandez E, Maira G (2005) Diffuse craniospinal choroid plexus papilloma with involvement of both cerebellopontine angles. Neurology 65:842
Due-Tonnessen B, Helseth E, Skullerud K, Lundar T (2001) Choroid plexus tumors in children and young adults: report of 16 consecutive cases. Childs Nerv Syst 17:252–256
Eisenberg HM, McComb JG, Lorenzo AV (1974) Cerebrospinal fluid overproduction and hydrocephalus associated with choroid plexus papilloma. J Neurosurg 40:381–385
Ellenbogen RG, Winston KR, Kupsky WJ (1989) Tumors of the choroid plexus in children. Neurosurgery 25:327–335
Gaudio RM, Tacconi L, Rossi ML (1998) Pathology of choroid plexus papillomas: a review. Clin Neurol Neurosurg 100:165–186
Ghatak NR, McWhorter JM (1976) Ultrastructural evidence for CSF production by a choroid plexus papilloma. J Neurosurg 45:409–415
Gopal P, Parker JR, Debski R, Parker JC Jr (2008) Choroid plexus carcinoma. Arch Pathol Lab Med 132:1350–1354
Gorelyshev SK, Matuev KB, Medvedev OA (2013) Migrating choroid plexus papilloma of the lateral ventricle in infant--modern approaches to surgical treatment. Zh Vopr Neirokhir Im N N Burdenko 77:45–49; discussion 49–50
Gozali AE, Britt B, Shane L, Gonzalez I, Gilles F, McComb JG et al (2012) 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 58:905–909
Guidetti B, Spallone A (1981) The surgical treatment of choroid plexus papillomas: the results of 27 years experience. Neurosurg Rev 4:129–137
Gupta N (2003) Choroid plexus tumors in children. Neurosurg Clin N Am 14:621–631
Gyure KA, Morrison AL (2000) Cytokeratin 7 and 20 expression in choroid plexus tumors: utility in differentiating these neoplasms from metastatic carcinomas. Mod Pathol 13:638–643
Haliasos N, Brew S, Robertson F, Hayward R, Thompson D, Chakraborty A (2013a) Pre-operative embolisation of choroid plexus tumours in children. Part II. Observations on the effects on CSF production. Childs Nerv Syst 29:71–76
Haliasos N, Brew S, Robertson F, Hayward R, Thompson D, Chakraborty A (2013b) Preoperative embolisation of choroid plexus tumours in children: Part I-Does the reduction of perioperative blood loss affect the safety of subsequent surgery? Childs Nerv Syst 29:65–70
Hasselblatt M, Bohm C, Tatenhorst L, Dinh V, Newrzella D, Keyvani K et al (2006) Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach. Am J Surg Pathol 30:66–74
Hasselblatt M, Mertsch S, Koos B, Riesmeier B, Stegemann H, Jeibmann A et al (2009) TWIST-1 is overexpressed in neoplastic choroid plexus epithelial cells and promotes proliferation and invasion. Cancer Res 69:2219–2223
Hawkins JC 3rd (1980) Treatment of choroid plexus papillomas in children: a brief analysis of twenty years’ experience. Neurosurgery 6:380–384
Horska A, Ulug AM, Melhem ER, Filippi CG, Burger PC, Edgar MA et al (2001) Proton magnetic resonance spectroscopy of choroid plexus tumors in children. J Magn Reson Imaging 14:78–82
Jagielski J, Zabek M, Wierzba-Bobrowicz T, Lakomiec B, Chrapusta SJ (2001) Disseminating histologically benign multiple papilloma of the choroid plexus: case report. Folia Neuropathol 39:209–213
Jamjoom AA, Sharab MA, Jamjoom AB, Satti MB (2009) Rapid evolution of a choroid plexus papilloma in an infant. Br J Neurosurg 23:324–325
Jeibmann A, Wrede B, Peters O, Wolff JE, Paulus W, Hasselblatt M (2007) Malignant progression in choroid plexus papillomas. J Neurosurg 107:199–202
Jinhu Y, Jianping D, Jun M, Hui S, Yepeng F (2007) Metastasis of a histologically benign choroid plexus papilloma: case report and review of the literature. J Neuro-Oncol 83:47–52
Kahn EA, Luros JT (1952) Hydrocephalus from overproduction of cerebrospinal fluid, and experiences with other parillomas of the choroid plexus. J Neurosurg 9:59–67
Karim A, Fowler M, McLaren B, Cardenas R, Patwardhan R, Nanda A (2006) Concomitant choroid plexus papillomas involving the third and fourth ventricles: a case report and review of the literature. Clin Neurol Neurosurg 108:586–589
Kim TW, Jung TY, Jung S, Kim IY, Moon KS, Jeong EH (2012) Unusual radiologic findings and pathologic growth patterns on choroid plexus papillomas. J Korean Neurosurg Soc 51:272–275
Knierim DS (1990) Choroid plexus tumors in infants. Pediatr Neurosurg 16:276–280
Koh EJ, Wang KC, Phi JH, Lee JY, Choi JW, Park SH et al (2014) Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute. Childs Nerv Syst 30:217–225
Koos B, Paulsson J, Jarvius M, Sanchez BC, Wrede B, Mertsch S et al (2009) Platelet-derived growth factor receptor expression and activation in choroid plexus tumors. Am J Pathol 175:1631–1637
Krieger MD, Panigrahy A, McComb JG, Nelson MD, Liu X, Gonzalez-Gomez I et al (2005) Differentiation of choroid plexus tumors by advanced magnetic resonance spectroscopy. Neurosurg Focus 18:E4
Krishnan S, Brown PD, Scheithauer BW, Ebersold MJ, Hammack JE, Buckner JC (2004) Choroid plexus papillomas: a single institutional experience. J Neuro-Oncol 68:49–55
Laarakker AS, Nakhla J, Kobets A, Abbott R (2017) Incidental choroid plexus papilloma in a child: a difficult decision. Surg Neurol Int 8:86
Lafay-Cousin L, Keene D, Carret AS, Fryer C, Brossard J, Crooks B et al (2011) Choroid plexus tumors in children less than 36 months: the Canadian pediatric brain tumor consortium (CPBTC) experience. Childs Nerv Syst 27:259–264
Lam S, Lin Y, Cherian J, Qadri U, Harris DA, Melkonian S et al (2013) Choroid plexus tumors in children: a population-based study. Pediatr Neurosurg 49:331–338
Lechanoine F, Zemmoura I, Velut S (2017) Treating cerebrospinal fluid rhinorrhea without dura repair: a case report of posterior fossa choroid plexus papilloma and review of the literature. World Neurosurg 108:990 e991–990 e999
Lena G, Genitori L, Molina J, Legatte JR, Choux M (1990) Choroid plexus tumours in children. Review of 24 cases. Acta Neurochir 106:68–72
Li L, Grausam KB, Wang J, Lun MP, Ohli J, Lidov HG et al (2016) Sonic Hedgehog promotes proliferation of Notch-dependent monociliated choroid plexus tumour cells. Nat Cell Biol 18:418–430
Longatti P, Basaldella L, Orvieto E, Dei Tos A, Martinuzzi A (2006) Aquaporin(s) expression in choroid plexus tumours. Pediatr Neurosurg 42:228–233
Losi-Guembarovski R, Kuasne H, Guembarovski AL, Rainho CA, Colus IM (2007) DNA methylation patterns of the CDH1, RARB, and SFN genes in choroid plexus tumors. Cancer Genet Cytogenet 179:140–145
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A et al (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK et al (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820
Mallick S, Benson R, Melgandi W, Rath GK (2017) Effect of surgery, adjuvant therapy, and other prognostic factors on choroid plexus carcinoma: a systematic review and individual patient data analysis. Int J Radiat Oncol Biol Phys 99:1199–1206
Masson CB (1934) Complete removal of two tumors of the third ventricle with recovery. Arch Surg 28:527–537
Mazloom A, Wolff JE, Paulino AC (2010) The impact of radiotherapy fields in the treatment of patients with choroid plexus carcinoma. Int J Radiat Oncol Biol Phys 78:79–84
McGirr SJ, Ebersold MJ, Scheithauer BW, Quast LM, Shaw EG (1988) Choroid plexus papillomas: long-term follow-up results in a surgically treated series. J Neurosurg 69:843–849
Menon G, Nair SN, Baldawa SS, Rao RB, Krishnakumar KP, Gopalakrishnan CV (2010) Choroid plexus tumors: an institutional series of 25 patients. Neurol India 58:429–435
Merino DM, Shlien A, Villani A, Pienkowska M, Mack S, Ramaswamy V et al (2015) Molecular characterization of choroid plexus tumors reveals novel clinically relevant subgroups. Clin Cancer Res 21:184–192
Morshed RA, Lau D, Sun PP, Ostling LR (2017) Spinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report. J Neurosurg Pediatr 20:471–479
Nagib MG, O’Fallon MT (2000) Lateral ventricle choroid plexus papilloma in childhood: management and complications. Surg Neurol 54:366–372
Niikawa S, Ito T, Murakawa T, Hirayama H, Ando T, Sakai N et al (1993) Recurrence of choroid plexus papilloma with malignant transformation--case report and lectin histochemistry study. Neurol Med Chir (Tokyo) 33:32–35
Nupponen NN, Paulsson J, Jeibmann A, Wrede B, Tanner M, Wolff JE et al (2008) Platelet-derived growth factor receptor expression and amplification in choroid plexus carcinomas. Mod Pathol 21:265–270
Ogiwara H, Dipatri AJ Jr, Alden TD, Bowman RM, Tomita T (2012) Choroid plexus tumors in pediatric patients. Br J Neurosurg 26:32–37
Ostrom QT, Gittleman H, Liao P, Rouse C, Chen Y, Dowling J et al (2014) CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro-Oncology 16(Suppl 4):iv1–i63
Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmerman RA et al (1992) Choroid plexus carcinoma of childhood. Cancer 69:580–585
Pandey S, Sharma V, Singh K, Ghosh A, Gupta PK (2016) Uncommon presentation of choroid plexus papilloma in an infant. J Pediatr Neurosci 11:61–63
Paul L, Madan M, Rammling M, Chigurupati S, Chan SL, Pattisapu JV (2011) Expression of aquaporin 1 and 4 in a congenital hydrocephalus rat model. Neurosurgery 68:462–473
Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, Kalifa C, Brunelle F, Sgouros S et al (1998) Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 88:521–528
Peyre M, Bah A, Kalamarides M (2012) Multifocal choroid plexus papillomas: case report. Acta Neurochir 154:295–299
Pianetti Filho G, Fonseca LF, da Silva MC (2002) Choroid plexus papilloma and Aicardi syndrome: case report. Arq Neuropsiquiatr 60:1008–1010
Picht T, Jansons J, van Baalen A, Harder A, Pietilae TA (2006) Infant with unusually large choroid plexus papilloma undergoing emergency surgery. Case report with special emphasis on the surgical strategy. Pediatr Neurosurg 42:116–121
Prendergast N, Goldstein JD, Beier AD (2018) Choroid plexus adenoma in a child: expanding the clinical and pathological spectrum. J Neurosurg Pediatr 21:428–433
Safaee M, Oh MC, Bloch O, Sun MZ, Kaur G, Auguste KI et al (2013a) Choroid plexus papillomas: advances in molecular biology and understanding of tumorigenesis. Neuro-Oncology 15:255–267
Safaee M, Clark AJ, Bloch O, Oh MC, Singh A, Auguste KI et al (2013b) Surgical outcomes in choroid plexus papillomas: an institutional experience. J Neuro-Oncol 113:117–125
Salunke P, Sahoo SK, Madhivanan K, Radotra BD (2014) A typical radiological presentation in a case of choroid plexus carcinoma. Surg Neurol Int 5:63
Sampath S, Nitin G, Yasha TC, Chandramouli BA, Devi BI, Kovoor JM (2008) Does choroid plexus tumour differ with age? Br J Neurosurg 22:373–388
Scala M, Morana G, Milanaccio C, Pavanello M, Nozza P, Garre ML (2017) Atypical choroid plexus papilloma: spontaneous resolution of diffuse leptomeningeal contrast enhancement after primary tumor removal in 2 pediatric cases. J Neurosurg Pediatr 20:284–288
Scholsem M, Scholtes F, Robe PA, Bianchi E, Kroonen J, Deprez M (2012) Multifocal choroid plexus papilloma: a case report. Clin Neuropathol 31:430–434
Serifoglu I, Oz II, Yazgan O, Caglar E, Guneyli S, Sunar Erdem CZ (2016) Spinal seeding of choroid plexus papilloma. Spine J 16:e421–e422
Shi YZ, Chen MZ, Huang W, Guo LL, Chen X, Kong D et al (2017a) Atypical choroid plexus papilloma: clinicopathological and neuroradiological features. Acta Radiol 58:983–990
Shi Y, Li X, Chen X, Xu Y, Bo G, Zhou H et al (2017b) Imaging findings of extraventricular choroid plexus papillomas: a study of 10 cases. Oncol Lett 13:1479–1485
Singh P, Khan A, Scott G, Jasper M, Singh E (2017) Lesson of the month 2: a choroid plexus papilloma manifesting as anorexia nervosa in an adult. Clin Med (Lond) 17:183–185
Spallone A, Pastore FS, Giuffre R, Guidetti B (1990) Choroid plexus papillomas in infancy and childhood. Childs Nerv Syst 6:71–74
St Clair SK, Humphreys RP, Pillay PK, Hoffman HJ, Blaser SI, Becker LE (1991) Current management of choroid plexus carcinoma in children. Pediatr Neurosurg 17:225–233
Sun MZ, Oh MC, Ivan ME, Kaur G, Safaee M, Kim JM et al (2014a) Current management of choroid plexus carcinomas. Neurosurg Rev 37:179–192;. discussion 192
Sun MZ, Ivan ME, Clark AJ, Oh MC, Delance AR, Oh T et al (2014b) Gross total resection improves overall survival in children with choroid plexus carcinoma. J Neuro-Oncol 116:179–185
Tabori U, Shlien A, Baskin B, Levitt S, Ray P, Alon N et al (2010) TP53 alterations determine clinical subgroups and survival of patients with choroid plexus tumors. J Clin Oncol 28:1995–2001
Tacconi L, Delfini R, Cantore G (1996) Choroid plexus papillomas: consideration of a surgical series of 33 cases. Acta Neurochir 138:802–810
Taggard DA, Menezes AH (2000) Three choroid plexus papillomas in a patient with Aicardi syndrome. A case report. Pediatr Neurosurg 33:219–223
Taylor MB, Jackson RW, Hughes DG, Wright NB (2001) Magnetic resonance imaging in the diagnosis and management of choroid plexus carcinoma in children. Pediatr Radiol 31:624–630
Thomas C, Ruland V, Kordes U, Hartung S, Capper D, Pietsch T et al (2015) Pediatric atypical choroid plexus papilloma reconsidered: increased mitotic activity is prognostic only in older children. Acta Neuropathol 129:925–927
Tong Y, Merino D, Nimmervoll B, Gupta K, Wang YD, Finkelstein D et al (2015) Cross-species genomics identifies TAF12, NFYC, and RAD54L as choroid plexus carcinoma oncogenes. Cancer Cell 27:712–727
Trifiletti RR, Incorpora G, Polizzi A, Cocuzza MD, Bolan EA, Parano E (1995) Aicardi syndrome with multiple tumors: a case report with literature review. Brain Dev 17:283–285
Unger MBE (1906) Zur kenntnis der primaren epithelgeschwulste der adergeflechte des dehirns. Arch Klin Chir 81:61–82
Wind JJ, Bell RS, Bank WO, Myseros JS (2010) Treatment of third ventricular choroid plexus papilloma in an infant with embolization alone. J Neurosurg Pediatr 6:579–582
Wolff JE, Sajedi M, Coppes MJ, Anderson RA, Egeler RM (1999) Radiation therapy and survival in choroid plexus carcinoma. Lancet 353:2126
Wolff JE, Sajedi M, Brant R, Coppes MJ, Egeler RM (2002) Choroid plexus tumours. Br J Cancer 87:1086–1091
Wrede B, Hasselblatt M, Peters O, Thall PF, Kutluk T, Moghrabi A et al (2009) Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neuro-Oncol 95:383–392
Zachary G, George J, Jaishri B, Peter B, Stephanie T (2014) Management of disseminated choroid plexus papilloma: a case study. Pediatr Blood Cancer 61:562–563
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 The American Physiological Society
About this chapter
Cite this chapter
Fulkerson, D.H., Leibold, A., Priemer, D., Balsara, K. (2020). Choroid Plexus Tumors. In: Praetorius, J., Blazer-Yost, B., Damkier, H. (eds) Role of the Choroid Plexus in Health and Disease. Physiology in Health and Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-0716-0536-3_10
Download citation
DOI: https://doi.org/10.1007/978-1-0716-0536-3_10
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-0716-0535-6
Online ISBN: 978-1-0716-0536-3
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)