Skip to main content

Advertisement

SpringerLink
Log in

Results of the treatment of pineal tumors in children: the Lyon experience

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Introduction

Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervous system. Patient survival depends on the histology, the extension of the surgical removal, and the efficacy of the complementary treatment (chemotherapy and cranio-spinal irradiation), as well as the age of the patient.

Materials and methods

In this study, we analyzed 151 pediatric patients treated for pineal tumors from 1997 to 2020 in Lyon, France. All patients were recorded in the French Register of Pineal tumors, which has been centralized and maintained in Lyon since 2010.

Results

Our analysis shows that benign tumors have an overall positive prognosis with total surgical removal. Concerning pineal parenchymal tumors, pinealoblastomas have a poor prognosis , especially in children less than three years old. A new pathological classification system allows for a better stratification of patient risk within different groups of patients with pineal tumors. It is also important to note that the identification of DICER 1 syndrome in families with pinealoblastomas warrant further medical investigation.

Patients with Germ Cell Tumors have more favorable outcomes, with a global survival rate of 87 % and a pure germinoma survival rate of almost 97%. When analyzing the prognosis of pineal gland gliomas, otherwise known as tectal plate gliomas, pilocytic astrocytomas had a promising prognosis. Otherwise, prognosis of other tectal plate gliomas are related to the grade of malignancy and the efficacy of complementary treatment. Lastly, papillary tumors need a complete removal for the best chance of survival, and Atypical teratoid/ rabdoid tumors (AT/TR) still have a bad prognosis, regardless of surgical resection.

Conclusions

Our results show that, with regards to pediatric pineal region tumors, there are still areas in prognostic indicators that need to be improved. Similarly, these pathologies need to be treated via a multidisciplinary approach to improve a patient’s survival rate and their quality of life.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Availability of data and materials

Data and materials are available upon request to the corresponding author.

References

  1. Schulz M, Afshar-Bakshloo M, Koch A et al (2021) Management of pineal region tumors in a pediatric case series. Neurosurg Rev 44:1417–1427. https://doi.org/10.1007/s10143-020-01323-1

    Article  PubMed  Google Scholar 

  2. Pettorini BL, Al-Mahfoud R, Jenkinson MD et al (2013) Surgical pathway and management of pineal region tumours in children. Childs Nerv Syst 29:433–439. https://doi.org/10.1007/s00381-012-1954-y

    Article  PubMed  Google Scholar 

  3. Mottolese C, Szathmari A, Ricci-Franchi AC et al (2015) Supracerebellar infratentorial approach for pineal region tumors: our surgical and technical considerations. Neurochirurgie 61:176–183. https://doi.org/10.1016/j.neuchi.2014.02.004

    Article  CAS  PubMed  Google Scholar 

  4. Mottolese C, Szathmari A, Ricci-Franchi AC et al (2015) The sub-occipital transtentorial approach revisited base on our own experience. Neurochirurgie 61:168–175. https://doi.org/10.1016/j.neuchi.2013.12.005

    Article  CAS  PubMed  Google Scholar 

  5. Mottolese C (2015) Report 2013: tumors of the pineal region. Neurochirurgie 61:60. https://doi.org/10.1016/j.neuchi.2013.03.004

    Article  CAS  PubMed  Google Scholar 

  6. Mottolese C, Beuriat PA, Szathmari A (2015) Pineal tumours: experience of the French National Register and the Lyon School, results and considerations. Neurochirurgie 61:223–235. https://doi.org/10.1016/j.neuchi.2014.02.006

    Article  CAS  PubMed  Google Scholar 

  7. Jaju A, Hwang EI, Kool M et al (2019) MRI features of histologically diagnosed supratentorial primitive neuroectodermal tumors and pineoblastomas in correlation with molecular diagnoses and outcomes: a report from the Children’s Oncology Group ACNS0332 Trial. AJNR Am J Neuroradiol 40:1796–1803. https://doi.org/10.3174/ajnr.A6253

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Parikh KA, Venable GT, Orr BA et al (2017) Pineoblastoma-the experience at St. Jude Children’s Research Hospital Neurosurgery 81:120–128. https://doi.org/10.1093/neuros/nyx005

    Article  PubMed  Google Scholar 

  9. Mynarek M, Pizer B, Dufour C et al (2017) Evaluation of age-dependent treatment strategies for children and young adults with pineoblastoma: analysis of pooled European Society for Paediatric Oncology (SIOP-E) and US Head Start data. Neuro Oncol 19:576–585. https://doi.org/10.1093/neuonc/now234

    Article  CAS  PubMed  Google Scholar 

  10. Jouvet A, Fauchon F, Liberski P et al (2003) Papillary tumor of the pineal region. Am J Surg Pathol 27:505–512

    Article  PubMed  Google Scholar 

  11. Dallier F, Di Roio C (2015) Sitting position for pineal surgery: some anaesthetic considerations. Neurochirurgie 61:164–167. https://doi.org/10.1016/j.neuchi.2014.10.110

    Article  CAS  PubMed  Google Scholar 

  12. Lindroos A-C, Niiya T, Randell T et al (2010) Sitting position for removal of pineal region lesions: the Helsinki experience. World Neurosurg 74:505–513. https://doi.org/10.1016/j.wneu.2010.09.026

    Article  PubMed  Google Scholar 

  13. Hernesniemi J, Romani R, Albayrak BS et al (2008) Microsurgical management of pineal region lesions: personal experience with 119 patients. Surg Neurol 70:576–583. https://doi.org/10.1016/j.surneu.2008.07.019

    Article  PubMed  Google Scholar 

  14. Tanrıkulu B, Özek MM (2020) Management of mature pineal region teratomas in pediatric age group. Childs Nerv Syst 36:153–163. https://doi.org/10.1007/s00381-019-04204-1

    Article  PubMed  Google Scholar 

  15. Kinoshita Y, Yamasaki F, Tominaga A et al (2017) Pitfalls of neuroendoscopic biopsy of intraventricular germ cell tumors. World Neurosurg 106:430–434. https://doi.org/10.1016/j.wneu.2017.07.013

    Article  PubMed  Google Scholar 

  16. Tanikawa M, Yamada H, Sakata T et al (2019) Exclusive endoscopic occipital transtentorial approach for pineal region tumors. World neurosurgery. https://doi.org/10.1016/j.wneu.2019.08.038

    Article  PubMed  Google Scholar 

  17. Li BK, Vasiljevic A, Dufour C et al (2020) Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study. Acta Neuropathol 139:223–241. https://doi.org/10.1007/s00401-019-02111-y

    Article  CAS  PubMed  Google Scholar 

  18. Deshmukh VR, Smith KA, Rekate HL et al (2004) Diagnosis and management of pineocytomas. Neurosurgery 55:349–355; discussion 355–357

  19. Kumar N, Srinivasa GY, Madan R, Salunke P (2018) Role of radiotherapy in residual pineal parenchymal tumors. Clin Neurol Neurosurg 166:91–98. https://doi.org/10.1016/j.clineuro.2018.01.027

    Article  PubMed  Google Scholar 

  20. Tian Y, Liu R, Qin J et al (2018) Retrospective analysis of the clinical characteristics, therapeutic aspects, and prognostic factors of 18 cases of childhood pineoblastoma. World Neurosurg 116:e162–e168. https://doi.org/10.1016/j.wneu.2018.04.135

    Article  PubMed  Google Scholar 

  21. Görgün Ö, Koç B, Kebudi R et al (2021) Clinical characteristics, late effects and outcomes in pineoblastomas in children: a single center experience. Turk J Pediatr 63:955–961. https://doi.org/10.24953/turkjped.2021.06.002

  22. Vasiljevic A, Szathmari A, Champier J et al (2015) Histopathology of pineal germ cell tumors. Neurochirurgie 61:130–137. https://doi.org/10.1016/j.neuchi.2013.06.006

    Article  CAS  PubMed  Google Scholar 

  23. de Kock L, Sabbaghian N, Druker H et al (2014) Germ-line and somatic DICER1 mutations in pineoblastoma. Acta Neuropathol 128:583–595. https://doi.org/10.1007/s00401-014-1318-7

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Gilheeney SW, Saad A, Chi S et al (2008) Outcome of pediatric pineoblastoma after surgery, radiation and chemotherapy. J Neurooncol 89:89–95. https://doi.org/10.1007/s11060-008-9589-2

    Article  PubMed  Google Scholar 

  25. Tomita T (1998) Neurosurgical perspectives in pediatric neurooncology. Childs Nerv Syst 14:94–96. https://doi.org/10.1007/s003810050185

    Article  CAS  PubMed  Google Scholar 

  26. Huo X-L, Wang B, Zhang G-J et al (2020) Adverse factors of treatment response and overall survival in pediatric and adult patients with pineoblastoma. Cancer Manag Res 12:7343–7351. https://doi.org/10.2147/CMAR.S258476

    Article  PubMed  PubMed Central  Google Scholar 

  27. Abdelbaki MS, Abu-Arja MH, Davidson TB et al (2020) Pineoblastoma in children less than six years of age: the Head Start I, II, and III experience. Pediatr Blood Cancer 67:e28252. https://doi.org/10.1002/pbc.28252

    Article  PubMed  PubMed Central  Google Scholar 

  28. Regis J, Bouillot P, Rouby-Volot F et al (1996) Pineal region tumors and the role of stereotactic biopsy: review of the mortality, morbidity, and diagnostic rates in 370 cases. Neurosurgery 39:907–912; discussion 912–914

  29. Faure-Conter C (2015) Tumoral markers in tumors of the pineal region. Neurochirurgie 61:143–145. https://doi.org/10.1016/j.neuchi.2013.12.006

    Article  CAS  PubMed  Google Scholar 

  30. Takami H, Graffeo CS, Perry A et al (2021) The Third Eye Sees Double: cohort study of clinical presentation, histology, surgical approaches, and ophthalmic outcomes in pineal region germ cell tumors. World Neurosurg 150:e482–e490. https://doi.org/10.1016/j.wneu.2021.03.030

    Article  PubMed  Google Scholar 

  31. Somji M, Badhiwala J, McLellan A, Kulkarni AV (2016) Diagnostic yield, morbidity, and mortality of intraventricular neuroendoscopic biopsy: systematic review and meta-analysis. World Neurosurg 85:315-324.e2. https://doi.org/10.1016/j.wneu.2015.09.011

    Article  PubMed  Google Scholar 

  32. Matsutani M, Sano K, Takakura K et al (1997) Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86:446–455. https://doi.org/10.3171/jns.1997.86.3.0446

    Article  CAS  PubMed  Google Scholar 

  33. Lapras C, Patet JD, Mottolese C, Lapras C (1987) Direct surgery for pineal tumors: occipital-transtentorial approach. Prog Exp Tumor Res 30:268–280

    Article  CAS  PubMed  Google Scholar 

  34. Calaminus G, Frappaz D, Kortmann RD et al (2017) Outcome of patients with intracranial non-germinomatous germ cell tumors-lessons from the SIOP-CNS-GCT-96 trial. Neuro Oncol 19:1661–1672. https://doi.org/10.1093/neuonc/nox122

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Khatua S, Dhall G, O’Neil S et al (2010) Treatment of primary CNS germinomatous germ cell tumors with chemotherapy prior to reduced dose whole ventricular and local boost irradiation. Pediatr Blood Cancer 55:42–46. https://doi.org/10.1002/pbc.22468

    Article  PubMed  Google Scholar 

  36. Noudel R, Vinchon M, Dhellemmes P et al (2008) Intracranial teratomas in children: the role and timing of surgical removal. J Neurosurg Pediatr 2:331–338. https://doi.org/10.3171/PED.2008.2.11.331

    Article  PubMed  Google Scholar 

  37. Ostrom QT, de Blank PM, Kruchko C et al (2015) Alex’s lemonade stand foundation infant and childhood primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Neuro Oncol 16(Suppl 10):x1–x36. https://doi.org/10.1093/neuonc/nou327

    Article  PubMed  Google Scholar 

  38. Jouvet A, Fèvre-Montange M, Besançon R et al (1994) Structural and ultrastructural characteristics of human pineal gland, and pineal parenchymal tumors. Acta Neuropathol 88:334–348

    Article  CAS  PubMed  Google Scholar 

  39. Mathkour M, Hanna J, Ibrahim N et al (2021) Papillary tumor of the pineal region in pediatric populations: an additional case and systematic review of a rare tumor entity. Clin Neurol Neurosurg 201:106404. https://doi.org/10.1016/j.clineuro.2020.106404

    Article  PubMed  Google Scholar 

  40. Fèvre-Montange M, Hasselblatt M, Figarella-Branger D et al (2006) Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases. J Neuropathol Exp Neurol 65:1004–1011. https://doi.org/10.1097/01.jnen.0000240462.80263.13

    Article  PubMed  Google Scholar 

  41. Poulgrain K, Gurgo R, Winter C et al (2011) Papillary tumour of the pineal region. J Clin Neurosci 18:1007–1017. https://doi.org/10.1016/j.jocn.2010.12.027

    Article  PubMed  Google Scholar 

  42. Berhouma M, Ni H, Delabar V et al (2015) Update on the management of pineal cysts: case series and a review of the literature. Neurochirurgie 61:201–207. https://doi.org/10.1016/j.neuchi.2013.08.010

    Article  CAS  PubMed  Google Scholar 

  43. Chaussemy D, Cebulla H, Coca A et al (2015) Interest and limits of endoscopic approaches for pineal region tumours. Neurochirurgie 61:160–163. https://doi.org/10.1016/j.neuchi.2015.03.001

    Article  CAS  PubMed  Google Scholar 

  44. Kodera T, Bozinov O, Sürücü O et al (2011) Neurosurgical venous considerations for tumors of the pineal region resected using the infratentorial supracerebellar approach. J Clin Neurosci 18:1481–1485. https://doi.org/10.1016/j.jocn.2011.02.035

    Article  PubMed  Google Scholar 

  45. Lazar ML, Clark K (1974) Direct surgical management of masses in the region of the vein of Galen. Surg Neurol 2:17–21

    CAS  PubMed  Google Scholar 

  46. Stern J, Ross D (1993) Stereotactic management of benign pineal region cysts: report of two cases. Neurosurgery. https://doi.org/10.1097/00006123-199302000-00024

    Article  PubMed  Google Scholar 

  47. Mottolese C, Szathamari A, Beuriat PA et al (2015) Neuroendoscopy and pineal tumors: a review of the literature and our considerations regarding its utility. Neurochirurgie 61:155–159. https://doi.org/10.1016/j.neuchi.2013.12.008

    Article  CAS  PubMed  Google Scholar 

  48. Turtz AR, Hughes WB, Goldman HW (1995) Endoscopic treatment of a symptomatic pineal cyst: technical case report. Neurosurgery 37:1013–1014; discussion 1014–1015. https://doi.org/10.1227/00006123-199511000-00025

  49. Cardia A, Caroli M, Pluderi M et al (2006) Endoscope-assisted infratentorial-supracerebellar approach to the third ventricle: an anatomical study. J Neurosurg 104:409–414. https://doi.org/10.3171/ped.2006.104.6.409

    Article  PubMed  Google Scholar 

  50. Oi S, Kamio M, Joki T, Abe T (2001) Neuroendoscopic anatomy and surgery in pineal region tumors: role of neuroendoscopic procedure in the “minimally-invasive preferential” management. J Neurooncol 54:277–286

    Article  CAS  PubMed  Google Scholar 

  51. Claustrat B, Brun J, Chazot G (2005) The basic physiology and pathophysiology of melatonin. Sleep Med Rev 9:11–24. https://doi.org/10.1016/j.smrv.2004.08.001

    Article  PubMed  Google Scholar 

  52. Zaazoue MA, Goumnerova LC (2016) Pineal region tumors: a simplified management scheme. Childs Nerv Syst 32:2041–2045. https://doi.org/10.1007/s00381-016-3157-4

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, 32 Avenue du Doyen Jean Lépine, 69500, Hospices Civils de LyonBron, France

    Alexandru Szathmari, Pierre-Aurélien Beuriat, Federico Di Rocco & Carmine Mottolese

  2. Université Claude Bernard, Lyon 1, 69100, Villeurbanne, France

    Pierre-Aurélien Beuriat & Federico Di Rocco

  3. Department of Pathology and Neuropathology, GHE, Hospices Civils de Lyon, 69500, Bron, France

    Alexandre Vasiljevic

  4. Department of Pediatric Hematology and Oncology, Institut d’hématologie et d’oncologie Pédiatrique, 69008, Lyon, France

    Pierre Leblond & Cécile Faure-Conter

  5. Department of Radiotherapy, Centre Léon Bérard, 69008, Lyon, France

    Line Claude

Authors
  1. Alexandru Szathmari
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Pierre-Aurélien Beuriat
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Alexandre Vasiljevic
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Pierre Leblond
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Cécile Faure-Conter
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Line Claude
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Federico Di Rocco
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Carmine Mottolese
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

AS and PAB collected the data, designed and performed the research, analyzed and interpreted the data, wrote the paper, and performed the statistical analysis. CM designed and performed the research, analyzed and interpreted the data, wrote the paper, and supervised the study. All others authors performed research and revised the manuscript.

Corresponding author

Correspondence to Carmine Mottolese.

Ethics declarations

Ethics approval and consent to participate

Study was approved by the local ethic committee. No patients opposed to participate in the study.

Competing interests

The authors declare no competing interests.

Consent for publication

All authors consent for publication of the manuscript.

Conflict of interest

All authors report no disclosures relevant to this manuscript.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Szathmari, A., Beuriat, PA., Vasiljevic, A. et al. Results of the treatment of pineal tumors in children: the Lyon experience. Childs Nerv Syst 39, 2317–2327 (2023). https://doi.org/10.1007/s00381-022-05649-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-022-05649-7

Keywords

Search

Navigation