Skip to main content

Advertisement

SpringerLink
Log in

Pediatric pineal region masses: a single-center experience over 25 years

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

Abstract

Purpose

Pineal region tumors (PRT) represent less than 1% of brain neoplasms. The rare and heterogeneous nature of these tumors is reflected in the variety of treatment modalities employed.

Methods

A single-center retrospective review of all pediatric patients with pineal region tumors between November 1996 and June 2021 was performed. Fifty-six cases of pineal tumors were reviewed for age and symptoms upon presentation, diagnostic methods, imaging characteristics, histological classification, treatment modalities, recurrence, and mortality rates.

Results

The average age at diagnosis was 11.3 years. The majority of patients were male (82.1%) and Caucasian (73.2%). The most common presenting symptoms were headache (n = 38, 67.9%) and visual problems (n = 34, 60.7%). Hydrocephalus was present in 49 patients (87.5%). Germinoma (n = 20, 35.7%) and non-germinomatous germ cell tumor (NGGCT) (n = 17, 30.4%) were the most common tumors. Chemotherapy was employed for 54 patients (96.4%), radiation for 49 (87.5%), and surgical resection for 14 (25.0%). The average duration of treatment was 5.9 months. Progression-free survival was 74.4% at 5 years and 72.0% at 10 years. Overall survival was 85.7% at 5 years and 77.1% at 10 years.

Conclusion

Treatment of pineal region tumors must be targeted to each patient based on presentation, subtype, presence of hydrocephalus, and extent of disease. Upfront surgical resection is usually not indicated. As advances in oncological care proceed, treatment modalities may continue to improve in efficacy.

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
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9

Similar content being viewed by others

Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

References

  1. Vuong HG, Ngo TNM, Dunn IF (2021) Incidence, prognostic factors, and survival trend in pineal gland tumors: a population-based analysis. Front Oncol 19(11):780173. https://doi.org/10.3389/fonc.2021.780173

    Article  Google Scholar 

  2. Al-Hussaini M, Sultan I, Abuirmileh N, Jaradat I, Qaddoumi I (2009) Pineal gland tumors: experience from the SEER database. J Neurooncol 94(3):351–358. https://doi.org/10.1007/s11060-009-9881-9

    Article  PubMed  PubMed Central  Google Scholar 

  3. Fedorko S, Zweckberger K, Unterberg AW (2018) Quality of life following surgical treatment of lesions within the pineal region. J Neurosurg 130(1):28–37. https://doi.org/10.3171/2017.7.JNS17260

    Article  PubMed  Google Scholar 

  4. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 131(6):803–820. https://doi.org/10.1007/s00401-016-1545-1

    Article  PubMed  Google Scholar 

  5. Bruno F, Arrigoni F, Maggialetti N, Natella R, Reginelli A, Di Cesare E, Brunese L, Giovagnoni A, Masciocchi C, Splendiani A, Barile A (2019) Neuroimaging in emergency: a review of possible role of pineal gland disease. Gland Surg 8(2):133–140. https://doi.org/10.21037/gs.2019.01.02

  6. Horsburgh A, Massoud TF (2013) The circumventricular organs of the brain: conspicuity on clinical 3T MRI and a review of functional anatomy. Surg Radiol Anat 35(4):343–349. https://doi.org/10.1007/s00276-012-1048-2

    Article  PubMed  Google Scholar 

  7. Mavridis IN, Pyrgelis ES, Agapiou E, Meliou M (2021) Pineal region tumors: pathophysiological mechanisms of presenting symptoms. Am J Transl Res 15;13(6):5758–5766. PMID: 34306324; PMCID: PMC8290707

  8. Schulz M, Afshar-Bakshloo M, Koch A, Capper D, Driever PH, Tietze A, Grün A, Thomale UW (2021) Management of pineal region tumors in a pediatric case series. Neurosurg Rev 44(3):1417–1427. https://doi.org/10.1007/s10143-020-01323-1

    Article  PubMed  Google Scholar 

  9. Carr C, O'Neill BE, Hochhalter CB, Strong MJ, Ware ML (2019) Biomarkers of pineal region tumors: a review. Ochsner J. 19(1):26–31. https://doi.org/10.31486/toj.18.0110

  10. Faure-Conter C (2015) Tumoral markers in tumors of the pineal region. Neurochirurgie 61(2–3):143–5. https://doi.org/10.1016/j.neuchi.2013.12.006

  11. Blakeley JO, Grossman SA (2006) Management of pineal region tumors. Curr Treat Options Oncol 7(6):505–516. https://doi.org/10.1007/s11864-006-0025-6

    Article  PubMed  Google Scholar 

  12. Herrada-Pineda T, Revilla-Pacheco F, Manrique-Guzman S (2015) Endoscopic approach for the treatment of pineal region tumors. J Neurol Surg A Cent Eur Neurosurg 76(1):8–12. https://doi.org/10.1055/s-0032-1330958

    Article  PubMed  Google Scholar 

  13. Huang X, Zhang R, Mao Y, Zhou LF, Zhang C (2016) Recent advances in molecular biology and treatment strategies for intracranial germ cell tumors. World J Pediatr 12(3):275–282. https://doi.org/10.1007/s12519-016-0021-2

    Article  PubMed  Google Scholar 

  14. Murray MJ, Bartels U, Nishikawa R, Fangusaro J, Matsutani M, Nicholson JC (2015) Consensus on the management of intracranial germ-cell tumours. Lancet Oncol 16(9):e470–e477. https://doi.org/10.1016/s1470-2045(15)00244-2

    Article  PubMed  Google Scholar 

  15. Nakamura H, Takami H, Yanagisawa T, Kumabe T, Fujimaki T, Arakawa Y, Karasawa K, Terashima K, Yokoo H, Fukuoka K, Sonoda Y, Sakurada K, Mineharu Y, Soejima T, Fujii M, Shinojima N, Hara J, Yamasaki K, Fujimura J, Yamasaki F, Takahashi M, Suzuki T, Sato I, Nishikawa R, Sugiyama K (2022) The Japan Society for Neuro-Oncology guideline on the diagnosis and treatment of central nervous system germ cell tumors. Neuro Oncol 24(4):503–515. https://doi.org/10.1093/neuonc/noab242

    Article  PubMed  Google Scholar 

  16. Frappaz D, Conter CF, Szathmari A, Valsijevic A, Mottolese C (2015) The management of pineal tumors as a model for a multidisciplinary approach in neuro-oncology. Neurochirurgie 61(2–3):208–211. https://doi.org/10.1016/j.neuchi.2014.03.003

    Article  CAS  PubMed  Google Scholar 

  17. Matsutani M (2008) Treatment of intracranial germ cell tumors: the second phase II study of Japanese GCT study group. Neuro Oncol 10(3):420–421

    Google Scholar 

  18. Bouffet E, Baranzelli MC, Patte C, Portas M, Edan C, Chastagner P, Mechinaud-Lacroix F, Kalifa C (1999) Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience. Societe Francaise d'Oncologie Pediatrique. Br J Cancer 79(7–8):1199–1204. https://doi.org/10.1038/sj.bjc.6690192

  19. Fetcko K, Dey M (2018) Primary central nervous system germ cell tumors: a review and update. Med Res Arch 6(3):1719. https://doi.org/10.18103/mra.v6i3.1719

  20. Goldman S, Bouffet E, Fisher PG, Allen JC, Robertson PL, Chuba PJ, Donahue B, Kretschmar CS, Zhou T, Buxton AB, Pollack IF (2015) Phase II trial assessing the ability of neoadjuvant chemotherapy with or without second-look surgery to eliminate measurable disease for nongerminomatous germ cell tumors: a children’s oncology group study. J Clin Oncol 33(22):2464–2471. https://doi.org/10.1200/JCO.2014.59.5132

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Fangusaro J, Wu S, MacDonald S, Murphy E, Shaw D, Bartels U, Khatua S, Souweidane M, Lu HM, Morris D, Panigrahy A, Onar-Thomas A, Fouladi M, Gajjar A, Dhall G (2019) Phase II trial of response-based radiation therapy for patients with localized CNS nongerminomatous germ cell tumors: a children’s oncology group study. J Clin Oncol 37(34):3283–3290. https://doi.org/10.1200/JCO.19.00701

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Al-Mahfoudh R, Zakaria R, Irvine E, Pizer B, Mallucci CL (2014) The management of bifocal intracranial germinoma in children. Childs Nerv Syst 30(4):625–630. https://doi.org/10.1007/s00381-013-2287-1

    Article  CAS  PubMed  Google Scholar 

  23. Suresh TN, Mahadevan A, Santosh V, Shankar SK (2004) Subarachnoid spread of germinoma mimicking tuberculous meningitis. Neurol India 52(2):251–3. PMID: 15269485. Available from: https://www.neurologyindia.com/text.asp?2004/52/2/251/11057

  24. Michaiel G, Strother D, Gottardo N, Bartels U, Coltin H, Hukin J, Wilson B, Zelcer S, Hansford JR, Hassall T, AbdelBaki MS, Cole KA, Hoffman L, Smiley NP, Smith A, Vinitsky A, Vitanza NA, Wright A, Yeo KK, Chow LML, Vanan MI, Dhall G, Bouffet E, Lafay-Cousin L (2020) Intracranial growing teratoma syndrome (iGTS): an international case series and review of the literature. J Neurooncol 147(3):721–730. https://doi.org/10.1007/s11060-020-03486-9

    Article  CAS  PubMed  Google Scholar 

  25. Shepard MJ, Haider AS, Prabhu SS, Sawaya R, DeMonte F, McCutcheon IE, Weinberg JS, Ferguson SD, Suki D, Fuller GN, Lang FF (2022) Long term outcomes following surgery for pineal region tumors. J Neurooncol 156(3):491–498. https://doi.org/10.1007/s11060-021-03919-z

    Article  PubMed  Google Scholar 

  26. Kang JK, Jeun SS, Hong YK, Park CK, Son BC, Lee IW, Kim MC (1998) Experience with pineal region tumors. Childs Nerv Syst 14(1–2):63–8. https://doi.org/10.1007/s003810050177

  27. Huo XL, Wang B, Zhang GJ, Ma JP, Wang L, Zhang LW, Xu XY, Li XJ, Li H, Li D, Zhang JT, Wu Z (2020) Adverse factors of treatment response and overall survival in pediatric and adult patients with pineoblastoma. Cancer Manag Res 18(12):7343–7351. https://doi.org/10.2147/CMAR.S258476

    Article  Google Scholar 

  28. Wilkening GN, Madden JR (2012) Memory disorders in children with central nervous system germ cell tumors. J Pediatr Oncol Nurs 29(3):161–70. https://doi.org/10.1177/1043454212439938

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA

    Noor Malik, Derek C. Samples, Megan M. Finneran, Sarah Graber, Todd C. Hankinson & Michael H. Handler

  2. Department of Neurological Surgery, Carle BroMenn Medical Center, Normal, IL, USA

    Megan M. Finneran

  3. Department of Pediatrics, Center for Cancer and Blood Disorders, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA

    Kathleen Dorris, Gregory Norris & Nicholas K. Foreman

Authors
  1. Noor Malik
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Derek C. Samples
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Megan M. Finneran
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sarah Graber
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Kathleen Dorris
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Gregory Norris
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Nicholas K. Foreman
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Todd C. Hankinson
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Michael H. Handler
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Conceptualization: Michael H. Handler; methodology: Michael H. Handler, Noor Malik, Derek C. Samples, Nicholas K. Foreman, Kathleen Dorris, and Todd C. Hankinson; formal analysis and investigation: Sarah Graber, Noor Malik, Derek C. Samples, Megan Finneran, and Gregory Norris; writing—original draft preparation: Noor Malik; writing—review and editing: Michael H. Handler, Derek C. Samples, Sarah Graber, Gregory Norris, and Nicholas K. Foreman; supervision: Michael H. Handler.

Corresponding author

Correspondence to Michael H. Handler.

Ethics declarations

Competing interests

The authors declare no competing interests.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher's Note

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Malik, N., Samples, D.C., Finneran, M.M. et al. Pediatric pineal region masses: a single-center experience over 25 years. Childs Nerv Syst 39, 2307–2316 (2023). https://doi.org/10.1007/s00381-022-05593-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-022-05593-6

Keywords

Search

Navigation