Abstract
Choroid plexus tumors (CPTs), namely, papilloma (CPP) and carcinoma (CPC), often present with hydrocephalus. CPT may lead to hydrocephalus secondary to several mechanisms: CSF overproduction, CSF pathway obstruction, and CSF malabsorption. Tumor locations are more prevalent in the lateral ventricle as opposed to the fourth ventricle. Both tumor subtypes are more prevalent in infantile age groups.
The classical radiological appearance of CPP is of a cauliflower-like lesion, isointense on T1, with brisk and homogeneous enhancement.
Complete surgical resection (gross total resection, GTR) is the cornerstone of treatment for all CPT. Super-selective embolization of the feeding arteries via the anterior or posterior choroidal arteries, using various materials, has been shown to significantly reduce blood loss and improve the surgeon’s resection control and extent of resection.
It has been shown that hydrocephalus may persist even following complete tumor resection; thus careful follow-up and a low threshold for CSF diversion are needed.
CPC is one of the characteristic pediatric tumors of Li-Fraumeni syndrome (LFS), a familial cancer predisposition syndrome characterized by a high frequency of malignant tumors at a young age. TP53 germline mutations are the genetic hallmark of LFS.
CPP is considered a surgical disease, and GTR is the sole treatment needed (except for CSF considerations). However, CPC should be treated with adjuvant treatments, including mainly chemotherapeutic protocols, sometimes added by radiotherapy.
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References
Ait-Ghezali L, Arbabian A, Jeibmann A, Hasselblatt M, Hallaert GG, Van den Broecke C et al (2014) Loss of endoplasmic reticulum calcium pump expression in choroid plexus tumours. Neuropathol Appl Neurobiol 40:726–735
Anei R, Hayashi Y, Hiroshima S, Mitsui N, Orimoto R, Uemori G et al (2011) Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Neurol Med Chir (Tokyo) 51:437–441
Beschorner R, Pantazis G, Jeibmann A, Boy J, Meyermann R, Mittelbronn M et al (2009) Expression of EAAT-1 distinguishes choroid plexus tumors from normal and reactive choroid plexus epithelium. Acta Neuropathol 117:667–675
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
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
Cataltepe O, Liptzin D, Jolley L, Smith TW (2010) Diffuse villous hyperplasia of the choroid plexus and its surgical management. J Neurosurg Pediatr 5:518–522
D’Ambrosio AL, O’Toole JE, Connolly ES Jr, Feldstein NA (2003) Villous hypertrophy versus choroid plexus papilloma: a case report demonstrating a diagnostic role for the proliferation index. Pediatr Neurosurg 39:91–96
Dudley RW, Torok MR, Gallegos D, Liu AK, Handler MH, Hankinson TC (2015) Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neuro-Oncol 121:201–207
Emami-Naeini P, Nejat F, El Khashab M (2008) Cystic choroid plexus papilloma with multiple mural nodules in an infant. Childs Nerv Syst 24:629–631
Fujimura M, Onuma T, Kameyama M, Motohashi O, Kon H, Yamamoto K et al (2004) Hydrocephalus due to cerebrospinal fluid overproduction by bilateral choroid plexus papillomas. Childs Nerv Syst 20:485–488
Giacomazzi J, Selistre SG, Rossi C, Alemar B, Santos-Silva P, Pereira FS et al (2013) Li-Fraumeni and Li-Fraumeni-like syndrome among children diagnosed with pediatric cancer in Southern Brazil. Cancer 119:4341–4349
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
Gupta P, Sodhi KS, Mohindra S, Saxena AK, Das A, Khandelwal N (2013) Choroid plexus papilloma of the third ventricle: a rare infantile brain tumor. J Pediatr Neurosci 8:247–249
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
Hallaert GG, Vanhauwaert DJ, Logghe K, Van den Broecke C, Baert E, Van Roost D et al (2012) Endoscopic coagulation of choroid plexus hyperplasia. J Neurosurg Pediatr 9:169–177
Hartge DR, Axt-Fliedner R, Weichert J (2010) Prenatal diagnosis and successful postnatal therapy of an atypical choroid plexus papilloma-case report and review of literature. J Clin Ultrasound 38:377–383
Japp AS, Gessi M, Messing-Junger M, Denkhaus D, Zur Muhlen A, Wolff JE et al (2015) High-resolution genomic analysis does not qualify atypical plexus papilloma as a separate entity among choroid plexus tumors. J Neuropathol Exp Neurol 74:110–120
Jeibmann A, Hasselblatt M, Gerss J, Wrede B, Egensperger R, Beschorner R et al (2006) Prognostic implications of atypical histologic features in choroid plexus papilloma. J Neuropathol Exp Neurol 65:1069–1073
Jeibmann A, Wrede B, Peters O, Wolff JE, Paulus W, Hasselblatt M (2007) Malignant progression in choroid plexus papillomas. J Neurosurg 107:199–202
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
Kumar R, Singh S (2005) Childhood choroid plexus papillomas: operative complications. Childs Nerv Syst 21:138–143
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
Lang SS, Beslow LA, Gabel B, Judkins AR, Fisher MJ, Sutton LN et al (2012) Surgical treatment of brain tumors in infants younger than six months of age and review of the literature. World Neurosurg 78:137–144
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
Mirone G, Ruggiero C, Spennato P, Aliberti F, Trischitta V, Cinalli G (2015) Cortical gluing and Ringer lactate solution inflation to avoid cortical mantle collapse and subdural fluid collections in pediatric neurosurgery: safety and feasibility. Childs Nerv Syst 31:945–951
Mishra A, Ojha BK, Chandra A, Singh SK, Chandra N, Srivastava C (2014) Choroid plexus papilloma of posterior third ventricle: a case report and review of literature. Asian J Neurosurg 9:238
Nimjee SM, Powers CJ, McLendon RE, Grant GA, Fuchs HE (2010) Single-stage bilateral choroid plexectomy for choroid plexus papilloma in a patient presenting with high cerebrospinal fluid output. J Neurosurg Pediatr 5:342–345
Otten ML, Riina HA, Gobin YP, Souweidane MM (2006) Preoperative embolization in the treatment of choroid plexus papilloma in an infant. Case Report. J Neurosurg 104:419–421
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
Phi JH, Shin CH, Wang KC, Park SH, Kim SK (2011) Catastrophic electrolyte imbalance caused by excessive production and overdrainage of cerebrospinal fluid in an infant with choroid plexus papilloma. Childs Nerv Syst 27:1153–1156
Prasad GL, Kumar R, Suri V (2014) Primary cerebellopontine angle choroid plexus papilloma in a child: a rare occurrence. Neurol India 62:438–439
Pratheesh R, Moorthy RK, Singh R, Rajshekhar V (2009) Choroid plexus papilloma presenting as a non-contrast-enhancing fourth ventricular mass in a child. Neurol India 57:486–488
Safaee M, Oh MC, Sughrue ME, Delance AR, Bloch O, Sun M et al (2013) The relative patient benefit of gross total resection in adult choroid plexus papillomas. J Clin Neurosci 20:808–812
Serowka K, Chiu Y, Gonzalez I, Gilles F, McComb G, Krieger M et al (2010) Central nervous system (CNS) tumors in the first six months of life: the Children’s Hospital Los Angeles experience, 1979–2005. Pediatr Hematol Oncol 27:90–102
Singh L, Kelkar S, Agrawal N (2008) Use of an inflatable balloon on a catheter to prevent subdural fluid collections after intraventricular tumor surgery: technical note. J Neurosurg 108:1028–1032
Sun MZ, Ivan ME, Clark AJ, Oh MC, Delance AR, Oh T et al (2014a) Gross total resection improves overall survival in children with choroid plexus carcinoma. J Neuro-Oncol 116:179–185
Sun MZ, Oh MC, Ivan ME, Kaur G, Safaee M, Kim JM et al (2014b) Current management of choroid plexus carcinomas. Neurosurg Rev 37:179–192. discussion 192
Takahashi M, Yamamoto J, Aoyama Y, Soejima Y, Akiba D, Nishizawa S (2009) Efficacy of multi-staged surgery and adjuvant chemotherapy for successful treatment of atypical choroid plexus papilloma in an infant: case report. Neurol Med Chir (Tokyo) 49:484–487
Tamburrini G, Caldarelli M, Di Rocco F, Massimi L, D’Angelo L, Fasano T et al (2006) The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia. Childs Nerv Syst 22:605–608
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
Trivelato FP, Manzato LB, Rezende MT, Barroso PM, Faleiro RM, Ulhoa AC (2012) Preoperative embolization of choroid plexus papilloma with Onyx via the anterior choroidal artery: technical note. Childs Nerv Syst 28:1955–1958
Turkoglu E, Kertmen H, Sanli AM, Onder E, Gunaydin A, Gurses L et al (2014) Clinical outcome of adult choroid plexus tumors: retrospective analysis of a single institute. Acta Neurochir 156:1461–1468. discussion 1467–1468
Wang HH, Luo CB, Guo WY, Wu HM, Lirng JF, Wong TT et al (2013) Preoperative embolization of hypervascular pediatric brain tumors: evaluation of technical safety and outcome. Childs Nerv Syst 29:2043–2049
Warren DT, Hendson G, Cochrane DD (2009) Bilateral choroid plexus hyperplasia: a case report and management strategies. Childs Nerv Syst 25:1617–1622
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
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
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Roth, J., Constantini, S. (2019). Hydrocephalus Secondary to Choroid Plexus Tumors and Choroid Plexus Hyperplasia. In: Cinalli, G., Özek, M., Sainte-Rose, C. (eds) Pediatric Hydrocephalus. Springer, Cham. https://doi.org/10.1007/978-3-319-27250-4_57
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DOI: https://doi.org/10.1007/978-3-319-27250-4_57
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