Abstract
The aim of this study was to review and describe therapeutic approaches in children with choroid plexus tumor (CPT) based on a nationwide series. The World Health Organization classification subdivides these rare tumors into three histological subtypes corresponding to three grades of malignancy: low grade (grade I) choroid plexus papilloma (CPP), intermediate grade (grade II) atypical choroid plexus papilloma (aCPP) and high grade (grade III) choroid plexus carcinoma (CPC). This retrospective study included 102 French children younger than 18 years, treated from 2000 to 2012: 54 CPP, 26 aCPP and 22 CPC. The 5 year overall survival was 100% in CPP, 96.2% in aCPP and 64.7% in CPC. In patients with localized disease, complete surgical resection was achieved in 48/52 CPP, 20/26 aCPP and 7/14 CPC. In this group, patients with complete surgical resection had better event free survival than patients with partial resection (88.9 vs. 41.6%). 28 patients (1 CPP, 6 aCPP and 22 CPC) had adjuvant chemotherapy. 2 aCPP and 9 CPC had radiotherapy. We underlined the need for a central histological review to accurately analyze clinical data; we reported a much higher overall survival for CPC than in most previous CPT series probably including atypical teratoid rhabdoid tumors. In our series, the 5 years overall survival in CPC (64.7%) was higher than event free survival (25.2%) and could be interpreted as a clue for the efficiency of adjuvant/salvage therapy even if the heterogeneity of applied treatments in this retrospective series does not allow for meaningful statistical comparisons.
Similar content being viewed by others
References
Ogiwara H, Dipatri AJ Jr, Alden TD, Bowman RM, Tomita T (2012) Choroid plexus tumors in pediatric patients. Br J Neurosurg 26:32–37
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (2016) World Health Organization histological classification of tumours of the central nervous system. International Agency for Research on Cancer, Lyon
Safaee M, Oh MC, Bloch O, Sun MZ, Kaur G, Auguste KI, Tihan T, Parsa AT (2013) Choroid plexus papillomas: advances in molecular biology and understanding of tumorigenesis. Neuro Oncol 15:255–267
Gozali AE, Britt B, Shane L, Gonzalez I, Gilles F, McComb JG, Krieger MD, Lavey RS, Shlien A, Villablanca JG, Erdreich-Epstein A, Dhall G, Jubran R, Tabori U, Malkin D, Finlay JL (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
Bettegowda C, Adogwa O, Mehta V, Chaichana KL, Weingart J, Carson BS, Jallo GI, Ahn ES (2012) Treatment of choroid plexus tumors: a 20-year single institutional experience. J Neurosurg Pediatr 10:398–405
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
Greenberg ML (1999) Chemotherapy of choroid plexus carcinoma. Childs Nerv Syst 15:571–577
Lafay-Cousin L, Keene D, Carret AS, Fryer C, Brossard J, Crooks B, Eisenstat D, Johnston D, Larouche V, Silva M, Wilson B, Zelcer S, Bartels U, Bouffet E (2011) Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience. Childs Nerv Syst 27:259–264
Johann PD, Hovestadt V, Thomas C, Jeibmann A, Heß K, Bens S, Oyen F, Hawkins C, Pierson CR, Aldape K, Kim SP, Widing E, Sumerauer D, Hauser P, van Landeghem F, Ryzhova M, Korshunov A, Capper D, Jones DT, Pfister SM, Schneppenheim R, Siebert R, Paulus W, Frühwald MC, Kool M, Hasselblatt M (2016) Cribriform neuroepithelial tumor: Molecular characterization of a SMARCB1-deficient non-rhabdoid tumor with favorable long-term outcome. Brain Pathol. doi:10.1111/bpa.12413
Johann PD, Erkek S, Zapatka M, Kerl K, Buchhalter I, Hovestadt V, Jones DT, Sturm D, Hermann C, Segura Wang M, Korshunov A, Rhyzova M, Gröbner S, Brabetz S, Chavez L, Bens S, Gröschel S, Kratochwil F, Wittmann A, Sieber L, Geörg C, Wolf S, Beck K, Oyen F, Capper D, van Sluis P, Volckmann R, Koster J, Versteeg R, von Deimling A, Milde T, Witt O, Kulozik AE, Ebinger M, Shalaby T, Grotzer M, Sumerauer D, Zamecnik J, Mora J, Jabado N, Taylor MD, Huang A, Aronica E, Bertoni A, Radlwimmer B, Pietsch T, Schüller U, Schneppenheim R, Northcott PA, Korbel JO, Siebert R, Frühwald MC, Lichter P, Eils R, Gajjar A, Hasselblatt M, Pfister SM, Kool M (2016) Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell 29:379–393
Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, Kalifa C, Brunelle F, Sgouros S, Meyer P, Cinalli G, Zerah M, Pierre-Kahn A, Renier D (1998) Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 88:521–528
Lafay-Cousin L, Mabbott DJ, Halliday W, Taylor MD, Tabori U, Kamaly-Asl ID, Kulkarni AV, Bartels U, Greenberg M, Bouffet E (2010) Use of ifosfamide, carboplatin, and etoposide chemotherapy in choroid plexus carcinoma. J Neurosurg Pediatr 5:615–621
Wolff JE, Sajedi M, Brant R, Coppes MJ, Egeler RM (2002) Choroid plexus tumours. Br J Cancer 87:1086–1091
Koh EJ, Wang KC, Phi JH, Lee JY, Choi JW, Park SH, Park KD, Kim IH, Cho BK, Kim SK (2014) Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute. Childs Nerv Syst 30:217–225
Judkins AR, Burger PC, Hamilton RL, Kleinschmidt-DeMasters B, Perry A, Pomeroy SL, Rosenblum MK, Yachnis AT, Zhou H, Rorke LB, Biegel JA (2005) INI1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma. J Neuropathol Exp Neurol 64:391–397
Schittenhelm J, Nagel C, Meyermann R, Beschorner R (2011) Atypical teratoid/rhabdoid tumors may show morphological and immunohistochemical features seen in choroid plexus tumors. Neuropathology 31:461–467
Ruland V, Hartung S, Kordes U, Wolff JE, Paulus W, Hasselblatt M (2014) Choroid plexus carcinomas are characterized by complex chromosomal alterations related to patient age and prognosis. Genes Chromosomes Cancer 53:373–380
Japp AS, Klein-Hitpass L, Denkhaus D, Pietsch T (2016) OTX2 defines a subgroup of atypical teratoid rhabdoid tumors with close relationship to choroid plexus tumors. J Neuropathol Exp Neurol. doi:10.1093/jnen/nlw101
Bahar M, Hashem H, Tekautz T, Worley S, Tang A, de Blank P, Wolff J (2017) Choroid plexus tumors in adult and pediatric populations the Cleveland Clinic and University Hospitals experience. J Neurooncol. doi:10.1007/s11060-017-2384-1
Wrede B, Liu P, Ater J, Wolff JE (2005) Second surgery and the prognosis of choroid plexus carcinoma–results of a meta-analysis of individual cases. Anticancer Res 25:4429–4433
Wrede B, Hasselblatt M, Peters O, Thall PF, Kutluk T, Moghrabi A, Mahajan A, Rutkowski S, Diez B, Wang X, Pietsch T, Kortmann RD, Paulus W, Jeibmann A, Wolff JE (2009) Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol 95:383–392
Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmerman RA, Ryan J, Reaman G, Schut L (1992) Choroid plexus carcinoma of childhood. Cancer 69:580–585
Thomas C, Ruland V, Kordes U, Hartung S, Capper D, Pietsch T, Gerß J, Wolff JE, Paulus W, Hasselblatt M (2015) Pediatric atypical choroid plexus papilloma reconsidered: increased mitotic activity is prognostic only in older children. Acta Neuropathol 129:925–927
Thomas C, Sill M, Ruland V, Witten A, Hartung S, Kordes U, Jeibmann A, Beschorner R, Keyvani K, Bergmann M, Mittelbronn M, Pietsch T, Felsberg J, Monoranu CM, Varlet P, Hauser P, Olar A, Grundy RG, Wolff JE, Korshunov A, Jones DT, Bewerunge-Hudler M, Hovestadt V, von Deimling A, Pfister SM, Paulus W, Capper D, Hasselblatt M (2016) Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups. Neuro Oncol 18:790–796
Fitzpatrick LK, Aronson LJ, Cohen KJ (2002) Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely resected? J Neurooncol 57:123–126
Duffner PK, Kun LE, Burger PC, Horowitz ME, Cohen ME, Sanford RA, Krischer JP, Mulhern RK, James HE, Rekate HL et al (1995) Postoperative chemotherapy and delayed radiation in infants and very young children with choroid plexus carcinomas. The Pediatric Oncology Group. Pediatr Neurosurg 22:189–196
Sun MZ, Ivan ME, Clark AJ, Oh MC, Delance AR, Oh T, Safaee M, Kaur G, Bloch O, Molinaro A, Gupta N, Parsa AT (2014) Gross total resection improves overall survival in children with choroid plexus carcinoma. J Neurooncol 116:179–185
Wrede B, Liu P, Wolff JE (2007) Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol 85:345–351
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
Razzaq AA, Cohen AR (1997) Neoadjuvant chemotherapy for hypervascular malignant brain tumors of childhood. Pediatr Neurosurg 27:296–303
Haliasos N, Brew S, Robertson F, Hayward R, Thompson D, Chakraborty A (2013) 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
Haliasos N, Brew S, Robertson F, Hayward R, Thompson D, Chakraborty A (2013) Pre-operative embolisation of choroid plexus tumours in children. Part II. Observations on the effects on CSF production. Childs Nerv Syst 29:71–76
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
Addo NK, Kamaly-Asl ID, Josan VA, Kelsey AM, Estlin EJ (2011) Preoperative vincristine for an inoperable choroid plexus papilloma: a case discussion and review of the literature. J Neurosurg Pediatr 8:149–153
Sun MZ, Oh MC, Ivan ME, Kaur G, Safaee M, Kim JM (2014) Current management of choroid plexus carcinomas. Neurosurg Rev 37:179–192
Wolff JE, Sajedi M, Coppes MJ, Anderson RA, Egeler RM (1999) Radiation therapy and survival in choroid plexus carcinoma. Lancet 353:2126
Mosleh O, Tabori U, Bartels U, Huang A, Schechter T, Bouffet E (2013) Successful treatment of a recurrent choroid plexus carcinoma with surgery followed by high-dose chemotherapy and stem cell rescue. Pediatr Hematol Oncol 30:386–391
Zaky W, Dhall G, Khatua S, Brown RJ, Ginn KF, Gardner SL, Yildiz VO, Yankelevich M, Finlay JL (2015) Choroid plexus carcinoma in children: the head start experience. Pediatr Blood Cancer 62:784–789
Acknowledgements
This study was supported by the association <<111 des Arts>>. We thank SFCE participating centers and the pediatric oncologists, pathologists and clinical research assistants for their contribution to this study. We thank Anita from Oscar’s Angels association, for her help in this study. We thank the Groupe d’Etude de Neuropathologie Oncologique Pédiatrique (GENOP) for their histopathological review.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
All authors declare that they have no conflicts of interest.
Electronic supplementary material
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Siegfried, A., Morin, S., Munzer, C. et al. A French retrospective study on clinical outcome in 102 choroid plexus tumors in children. J Neurooncol 135, 151–160 (2017). https://doi.org/10.1007/s11060-017-2561-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11060-017-2561-2