CC BY 4.0 · Arq Neuropsiquiatr 2023; 81(09): 825-834
DOI: 10.1055/s-0043-1770351
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Posterior fossa choroidplexus papilloma in the pediatric population: case series and literature review

Papiloma de plexo coroide na fossa posterior na população pediátrica: série de casos e revisão da literatura
1   Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Hospital das Clínicas, Divisão de Neurocirurgia, Ribeirão Preto SP, Brazil.
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1   Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Hospital das Clínicas, Divisão de Neurocirurgia, Ribeirão Preto SP, Brazil.
2   Universidade Federal de São Carlos, Departamento de Medicina, São Carlos SP, Brazil.
,
1   Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Hospital das Clínicas, Divisão de Neurocirurgia, Ribeirão Preto SP, Brazil.
,
1   Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Hospital das Clínicas, Divisão de Neurocirurgia, Ribeirão Preto SP, Brazil.
› Author Affiliations

Abstract

Choroid plexus papillomas (CPPs) are rare benign neoplasms which are particularly uncommon in the posterior fossa in children. We herein present a case series of five patients treated at a tertiary care hospital. A comprehensive literature review was also carried out. The patients treated at the tertiary care hospital were aged between 4 and 16 years. Gross total resection (GTR) was initially achieved in two patients. All patients showed clinical improvement. Moreover, 27 articles published between 1975 and 2021 were selected for the literature review, totaling 46 patients; with the 5 patients previously described, the total sample was composed of 51 cases, With a mean age was 8.2 years. The lesions were located either in the fourth ventricle (65.3%) or the cerebellopontine angle (34.7%). Hydrocephalus was present preoperatively in 66.7% of the patients, and a permanent shunt was required in 31.6% of the cases. The GTR procedure was feasible in 64.5%, and 93.8% showed clinical improvement. For CPPs, GTR is the gold standard treatment and should be attempted whenever feasible, especially because the role of the adjuvant treatment remains controversial. Neuromonitoring is a valuable tool to achieve maximal safe resection. Hydrocephalus is common and must be recognized and promptly treated. Most patients will need a permanent shunt. Though there is still controversy on its efficacy, endoscopic third ventriculostomy is a safe procedure, and was the authors' first choice to treat hydrocephalus.

Resumo

Os papilomas do plexo coroide (PPCs) são neoplasias benignas raras e, na população pediátrica, são particularmente incomuns na fossa posterior. Apresentamos uma série de casos de cinco pacientes atendidos em um hospital terciário. Além disso, foi realizada uma ampla revisão da literatura. Os pacientes atendidos no hospital terciário tinham entre 4 e 16 anos. Ressecção macroscópica total (RMT) foi inicialmente realizada em dois pacientes. Todos os pacientes apresentaram melhora clínica. Além disso, 27 artigos publicados entre 1975 e 2021 foram selecionados para a revisão da literatura, totalizando 46 pacientes. Somados à série de casos atuais, encontramos 51 pacientes, com média de idade de 8,2 anos. As lesões localizavam-se no quarto ventrículo (65,3%) ou no ângulo pontocerebelar (34,7%). Hidrocefalia estava presente no pré-operatório em 66,7% dos pacientes, e derivação ventricular permanente foi necessária em 31,6% dos casos. A RMT foi possível em 64,5%, e 93,8% tiveram melhora clínica. Para os CPPs, a RMT é o tratamento padrão-ouro e deve ser tentado sempre que possível, especialmente porque ainda existem controvérsias quanto ao papel do tratamento adjuvante. A neuromonitorização é uma ferramenta importante para se atingir a máxima ressecção segura. A hidrocefalia é comumente vista nesses pacientes e deve ser identificada e tratada. A maioria dos pacientes irá precisar de uma derivação permanente. Apesar de persistirem controvérsias sobre sua eficácia, a terceiro-ventriculostomia endoscópica foi a primeira escolha para tratar a hidrocefalia na experiência dos autores e é uma opção segura.

Authors' Contributions

RIP: conceptualization and design of the study, data acquisition, collection and interpretation, literature review, drafting of the article and approval of the final version; MFMB: conceptualization and design of the study, data interpretation, literature review, critical reviews of the article and approval of the final version; MVS, RSO: supervision, conceptualization, and design of the study, data interpretation, literature review, critical review of the article critically and approval of the final version.




Publication History

Received: 01 August 2022

Accepted: 09 February 2023

Article published online:
21 August 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Brassesco MS, Valera ET, Neder L. et al. Polyploidy in atypical grade II choroid plexus papilloma of the posterior fossa. Neuropathology 2009; 29 (03) 293-298
  • 2 Adib SD, Hempel JM, Kandilaris K, Grimm F, Evangelista Zamora R, Tatagiba M. Surgical management of choroid plexus papilloma of the cerebellopontine and cerebellomedullary angle: classification and strategy. Neurosurg Rev 2021; 44 (06) 3387-3397
  • 3 Louis DN, Perry A, Wesseling P. et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro-oncol 2021; 23 (08) 1231-1251
  • 4 Sethi D, Arora R, Garg K, Tanwar P. Choroid plexus papilloma. Asian J Neurosurg 2017; 12 (01) 139-141
  • 5 Bettegowda C, Adogwa O, Mehta V. et al. Treatment of choroid plexus tumors: a 20-year single institutional experience. J Neurosurg Pediatr 2012; 10 (05) 398-405
  • 6 Shi YZ, Chen MZ, Huang W. et al. Atypical choroid plexus papilloma: clinicopathological and neuroradiological features. Acta Radiol 2017; 58 (08) 983-990
  • 7 Menon G, Nair SN, Baldawa SS, Rao RB, Krishnakumar KP, Gopalakrishnan CV. Choroid plexus tumors: an institutional series of 25 patients. Neurol India 2010; 58 (03) 429-435
  • 8 Brassesco MS, Valera ET, Becker AP. et al. Grade II atypical choroid plexus papilloma with normal karyotype. Childs Nerv Syst 2009; 25 (12) 1623-1626
  • 9 Raimondi AJ, Gutierrez FA. Diagnosis and surgical treatment of choroid plexus papillomas. Childs Brain 1975; 1 (2-3): 81-115
  • 10 Hammock MK, Milhorat TH, Breckbill DL. Primary choroid plexus papilloma of the cerebellopontine angle presenting as brain stem tumor in child. Childs Brain 1976; 2 (02) 132-142
  • 11 Laurence KM. The biology of choroid plexus papilloma in infancy and childhood. Acta Neurochir (Wien) 1979; 50 (1-2): 79-90
  • 12 Guidetti B, Spallone A. The surgical treatment of choroid plexus papillomas: the results of 27 years experience. Neurosurg Rev 1981; 4 (03) 129-137
  • 13 Masuzawa T, Shimabukuro H, Yoshimizu N, Sato F. Ultrastructure of disseminated choroid plexus papilloma. Acta Neuropathol 1981; 54 (04) 321-324
  • 14 Zhang W. Choroid plexus papilloma of the cerebellopontine angle, with special reference to vertebral angiographic study. Surg Neurol 1982; 18 (05) 367-371
  • 15 Piguet V, de Tribolet N. Choroid plexus papilloma of the cerebellopontine angle presenting as a subarachnoid hemorrhage: case report. Neurosurgery 1984; 15 (01) 114-116
  • 16 Maria BL, Strauss LC, Wharam MD. Two primary brain tumors in one child. Neurology 1986; 36 (01) 71-73
  • 17 Lippa C, Abroms IF, Davidson R, DeGirolami U. Congenital choroid plexus papilloma of the fourth ventricle. J Child Neurol 1989; 4 (02) 127-130
  • 18 Shin JH, Lee HK, Jeong AK, Park SH, Choi CG, Suh DC. Choroid plexus papilloma in the posterior cranial fossa: MR, CT, and angiographic findings. Clin Imaging 2001; 25 (03) 154-162
  • 19 Rostasy KM, Sponholz S, Bahn E, Ludwig HC, Hanefeld F. Unusual localization of a choroid plexus papilloma in a 4-year-old female. Pediatr Neurol 2003; 28 (01) 66-68
  • 20 Doglietto F, Lauretti L, Tartaglione T, Gessi M, Fernandez E, Maira G. Diffuse craniospinal choroid plexus papilloma with involvement of both cerebellopontine angles. Neurology 2005; 65 (06) 842
  • 21 Stafrace S, Molloy J. Extraventricular choroid plexus papilloma in a neonate. Pediatr Radiol 2008; 38 (05) 593
  • 22 Larysz D, Blamek S, Larysz P, Pietras K, Mandera M. Posterior fossa brain tissue injury: developmental, neuropsychological, and neurological consequences of brain tumors in children. Acta Neurochir Suppl (Wien) 2010; 106: 271-274
  • 23 Crawford JR, Newbury RO, Levy ML. An unusual posterior fossa tumour in a young child. BMJ Case Rep 2013; 2013: bcr2013010158
  • 24 Ogiwara H, Dipatri Jr AJ, Alden TD, Bowman RM, Tomita T. Choroid plexus tumors in pediatric patients. Br J Neurosurg 2012; 26 (01) 32-37
  • 25 Xiao A, Xu J, He X, You C. Extraventricular choroid plexus papilloma in the brainstem. J Neurosurg Pediatr 2013; 12 (03) 247-250
  • 26 Koh EJ, Wang KC, Phi JH. et al. Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute. Childs Nerv Syst 2014; 30 (02) 217-225
  • 27 Prasad GL, Kumar R, Suri V. Primary cerebellopontine angle choroid plexus papilloma in a child: a rare occurrence. Neurol India 2014; 62 (04) 438-439
  • 28 Prasad GL, Mahapatra AK. Case series of choroid plexus papilloma in children at uncommon locations and review of the literature. Surg Neurol Int 2015; 6: 151
  • 29 Cai C, Stephens BH, Leonard JR, Dahiya S. Posterior fossa tumor with distinct choroid plexus papilloma and ependymoma components. Clin Neuropathol 2015; 34 (03) 132-135
  • 30 Luo W, Liu H, Li J, Yang J, Xu Y. Choroid Plexus Papillomas of the Cerebellopontine Angle. World Neurosurg 2016; 95: 117-125
  • 31 Morshed RA, Lau D, Sun PP, Ostling LR. Spinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report. J Neurosurg Pediatr 2017; 20 (05) 471-479
  • 32 Muñoz Montoya JE, Maldonado Moran MA, Santamaria Rodriguez P, Toro Lopez S, Perez Cataño CS, Luque Suarez JC. Choroid Plexus Papilloma of the Fourth Ventricle: A Pediatric Patient. Asian J Neurosurg 2019; 14 (02) 585-588
  • 33 Trybula SJ, Karras C, Bowman RM, Alden TD, DiPatri AJ, Tomita T. Infratentorial choroid plexus tumors in children. Childs Nerv Syst 2020; 36 (08) 1761-1766
  • 34 Gaddi MJS, Lappay JI, Chan KIP, Pascual JSG, Salonga AEM. Pediatric choroid plexus papilloma arising from the cerebellopontine angle: systematic review with illustrative case. Childs Nerv Syst 2021; 37 (03) 799-807
  • 35 Chen Y, Zhao R, Shi W, Li H. Pediatric atypical choroid plexus papilloma: Clinical features and diagnosis. Clin Neurol Neurosurg 2021; 200: 106345
  • 36 Lu L, Chen H, Weng S, Xu Y. Endoscopic Third Ventriculostomy versus Ventriculoperitoneal Shunt in Patients with Obstructive Hydrocephalus: Meta-Analysis of Randomized Controlled Trials. World Neurosurg 2019; 129: 334-340
  • 37 Uche EO, Okorie C, Iloabachie I, Amuta DS, Uche NJ. Endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) in non-communicating hydrocephalus (NCH): comparison of outcome profiles in Nigerian children. Childs Nerv Syst 2018; 34 (09) 1683-1689
  • 38 Dewan MC, Lim J, Shannon CN, Wellons III JC. The durability of endoscopic third ventriculostomy and ventriculoperitoneal shunts in children with hydrocephalus following posterior fossa tumor resection: a systematic review and time-to-failure analysis. J Neurosurg Pediatr 2017; 19 (05) 578-584
  • 39 Gholampour S, Bahmani M, Shariati A. Comparing the Efficiency of Two Treatment Methods of Hydrocephalus: Shunt Implantation and Endoscopic Third Ventriculostomy. Basic Clin Neurosci 2019; 10 (03) 185-198
  • 40 Ferguson SD, Levine NB, Suki D. et al. The surgical treatment of tumors of the fourth ventricle: a single-institution experience. J Neurosurg 2018; 128 (02) 339-351
  • 41 Wolff JE, Sajedi M, Brant R, Coppes MJ, Egeler RM. Choroid plexus tumours. Br J Cancer 2002; 87 (10) 1086-1091
  • 42 Toescu SM, Samarth G, Layard Horsfall H. et al. Fourth ventricle tumors in children: complications and influence of surgical approach. J Neurosurg Pediatr 2020; 27 (01) 52-61 cited 26Jan2022
  • 43 Tomasello F, Conti A, Cardali S, La Torre D, Angileri FF. Telovelar Approach to Fourth Ventricle Tumors: Highlights and Limitations. World Neurosurg 2015; 83 (06) 1141-1147
  • 44 Palmer C, Kalsi P, Scoones D, Bradey N, Strachan R. Recurrence of fourth ventricular choroid plexus papilloma with late occurrence of a spinal drop metastasis. Br J Neurosurg 2010; 24 (04) 482-484
  • 45 Safaee M, Oh MC, Bloch O. et al. Choroid plexus papillomas: advances in molecular biology and understanding of tumorigenesis. Neuro-oncol 2013; 15 (03) 255-267
  • 46 Browne-Farmer C, Hazrati LN, Mamatjan Y. et al. Paediatric atypical choroid plexus papilloma: is adjuvant therapy necessary?. J Neurooncol 2021; 155 (01) 63-70
  • 47 Hosmann A, Hinker F, Dorfer C. et al. Management of choroid plexus tumors-an institutional experience. Acta Neurochir (Wien) 2019; 161 (04) 745-754
  • 48 Kim IY, Niranjan A, Kondziolka D, Flickinger JC, Lunsford LD. Gamma knife radiosurgery for treatment resistant choroid plexus papillomas. J Neurooncol 2008; 90 (01) 105-110
  • 49 Colò F, Larrouquere L, Rivoirard R. et al. Bevacizumab in progressive disseminated atypical choroid plexus papilloma in adults. Neuro-oncol 2020; 22 (07) 1046-1047
  • 50 Dubey A, Sung WS, Shaya M. et al. Complications of posterior cranial fossa surgery–an institutional experience of 500 patients. Surg Neurol 2009; 72 (04) 369-375
  • 51 Sáenz A, Badaloni E, Grijalba M, Villalonga JF, Argañaraz R, Mantese B. Risk factors for surgical site infection in pediatric posterior fossa tumors. Childs Nerv Syst 2021; 37 (10) 3049-3056
  • 52 Ghali MGZ. Telovelar surgical approach. Neurosurg Rev 2021; 44 (01) 61-76
  • 53 Santamarta D, Blázquez JA, Maillo A, Muñoz A, Caballero M, Morales F. [Analysis of cerebrospinal fluid related complications (hydrocephalus, fistula, pseudomeningocele and infection) following surgery for posterior fossa tumors]. Neurocirugia (Astur) 2003; 14 (02) 117-126