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Outcome and prognostic features in paediatric pineoblastomas: analysis of cases from the Surveillance, Epidemiology, and End Results registry (1990–2007)

  • Original Article - Pediatric Neurosurgery
  • Published:
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Abstract

Background

Paediatric pineoblastomas are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports.

Methods

Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated clinical and pathological factors associated with outcome in paediatric pineoblastomas. Paediatric patients (< 16 years old) with pineoblastomas diagnosed between 1990 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient’s age at diagnosis, gender, race, tumour spread and size, surgical resection and the use of adjuvant radiotherapy.

Results

Seventy-eight patients were identified from the database. Twelve patients were excluded as 11 had no surgery and one patient was excluded as the surgical status was unknown. Analysis of the remaining 66 patients revealed a median age at diagnosis of 5.5 years. Three patients underwent biopsy. Seventeen patients underwent full and partial resection, respectively. A further 46 patients underwent surgery the nature of which was not recorded. Thirty-nine patients (59.1%) received adjuvant radiotherapy. Eight patients (12.1%) had both surgery (full or partial resection) and radiotherapy. The median overall survival was 40.5 months. Univariate analysis demonstrated that older age at diagnosis was the only positive predictor of overall survival.

Conclusion

This study represents the largest analysis of paediatric pineoblastomas to date. The only clinically relevant prognostic factor was older age at diagnosis. The role of surgery and adjuvant radiotherapy on overall survival remains to be defined.

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Abbreviations

CCLG:

Children’s Cancer and Leuakaemia Group

CI:

Confidence interval

CSF:

Cerebrospinal fluid

HR:

Hazard ratio

ICD:

International classification of diseases

MGMT:

O6-methylguanin-DNA-methyltransferase

NCI:

National Cancer Institute

OS:

Overall survival

PBL:

Pineoblastoma

SEER:

Surveillance, Epidemiology, and End Results

sPNET:

Supratentorial primitive neuroectodermal tumour

UK:

United Kingdom

US:

United States

WHO:

World Health Organisation

References

  1. Cuccia V, Rodríguez F, Palma F, Zuccaro G (2006) Pinealoblastomas in children. Childs Nerv Syst 22(6):577–585

    Article  Google Scholar 

  2. Lee J, Wakabayashi T, Yoshida J (2005) Management and survival of pineoblastoma: an analysis of 34 adults from the brain tumour registry of Japan. Neurol Med Chir (Tokyo) 45(3):132–141

    Article  Google Scholar 

  3. Gilheeney SW, Saad A, Chi S, Turner C, Ullrich NJ, Goumnerova L, Scott RM, Marcus K, Lehman L, De Girolami U, Kieran MW (2008) Outcome of paediatric pineoblastoma after surgery, radiation and chemotherapy. J Neuro-Oncol 89(1):89–95

    Article  Google Scholar 

  4. Chang SM, Lillis-Hearne PK, Larson DA, Wara WM, Bollen AW, Prados MD (1995) Pineoblastoma in adults. Neurosurgery 37(3):383–390

    Article  CAS  PubMed  Google Scholar 

  5. Reddy AT, Janss AJ, Phillips PC, Weiss HL, Packer RJ (2000) Outcome for children with supratentorial primitive neuroectodermal tumours treated with surgery, radiation, and chemotherapy. Cancer 88(9):2189–2193

    Article  CAS  PubMed  Google Scholar 

  6. Borit A, Blackwood W, Mair WG (1980) The separation of pineocytoma from pineoblastoma. Cancer 45(6):1408–1418

    Article  CAS  PubMed  Google Scholar 

  7. Sreekantaiah C, Jockin H, Breecher M, Sandberg AA (1989) Interstitial deletion of chromosome 11q in a pineoblastoma. Cancer Genet Cytogenet 39:125–131

    Article  CAS  PubMed  Google Scholar 

  8. Brown AE, Leibundgut K, Niggli FK, Betts DR (2006) Cytogenetics of pineoblastoma: four new cases and a literature review. Cancer Genet Cytogenet 170(2):175–179

    Article  CAS  PubMed  Google Scholar 

  9. Roberts P, Chumas PD, Picton S, Bridges L, Livingstone JH, Sheridan E (2001). A review of the cytogenetics of 58 pediatric brain tumors. Cancer Genet Cytogenet 131(1):1–12. Review

  10. Hart MN, Earle KM (1973) Primitive neuroectodermal tumours of the brain in children. Cancer 32(4):890–897

    Article  CAS  PubMed  Google Scholar 

  11. DeBoer R, Batjer H, Marymont M, Goldman S, Walker M, Gottardi-Littell N, Raizer J (2009) Response of an adult patient with pineoblastoma to vorinostat and retinoic acid. J Neuro-Oncol 95(2):289–292

    Article  Google Scholar 

  12. Nozza P, Casciana ML, Rossi A, Cama A, Milanaccio C, Raso A, Ravegnani M, Morreale G, Garrè ML (2010) Post-chemotherapy maturation of a pineoblastoma. Acta Neuropathol 119(5):651–653

    Article  PubMed  Google Scholar 

  13. Selvanathan SK, Hammouche S, Smethurst W, Salminen HJ, Jenkinson MD (2012) Outcome and prognostic features in adult pineoblastomas: analysis of cases from the SEER database. Acta Neurochir 154(5):863–869

    Article  PubMed  Google Scholar 

  14. Lutterbach J, Fauchon F, Schild SE, Chang SM, Pagenstecher A, Volk B, Ostertag C, Momm F, Jouvet A (2002) Malignant pineal parenchymal tumours in adult patients: patterns of care and prognostic factors. Neurosurgery 51(1):44–55

    Article  PubMed  Google Scholar 

  15. Kurisaka M, Arisawa M, Mori T, Sakamoto T, Seike M, Mori K, Okada T, Wakiguchi H, Kurashige T (1998) Combination chemotherapy (cisplatin, vinblastin) and low-dose irradiation in the treatment of pineal parenchymal cell tumours. Childs Nerv Syst 14(10):564–569

    Article  CAS  PubMed  Google Scholar 

  16. Matsumoto K, Higashi H, Tomita S, Ohmoto T (1995) Pineal region tumours treated with interstitial brachytherapy with low activity sources (192-iridium). Acta Neurochir 136(1–2):21–28

    Article  CAS  PubMed  Google Scholar 

  17. Nakamura M, Saeki N, Iwadate Y, Sunami K, Osato K, Yamaura A (2000) Neuroradiological characteristics of pineocytoma and pineoblastoma. Neuroradiology 42(7):509–514

    Article  CAS  PubMed  Google Scholar 

  18. Tomita T, McLone DG, Yasue M (1998) Cerebral primitive neuroectodermal tumours in childhood. J Neuro-Oncol 6(3):233–243

    Article  Google Scholar 

  19. Schild SE, Scheithauer BW, Haddock MG, Wong WW, Lyons MK, Marks LB, Norman MG, Burger PC (1996) Histologically confirmed pineal tumours and other germ cell tumours of the brain. Cancer 78(12):2564–2571

    Article  CAS  PubMed  Google Scholar 

  20. Banerjee AK, Kak VK (1974) Pineoblastoma with spontaneous intra and extracranial metastasis. J Pathol 114(1):9–12

    Article  CAS  PubMed  Google Scholar 

  21. Fauchon F, Jouvet A, Paquis P, Saint-Pierre G, Mottolese C, Ben Hassel M, Chauveinc L, Sichez JP, Philippon J, Schlienger M, Bouffet E (2000) Parenchymal pineal tumours: a clinicopathological study of 76 cases. Int J Radiat Oncol Biol Phys 46(4):959–968

    Article  CAS  Google Scholar 

  22. Gururangan S, McLaughlin C, Quinn J, Rich J, Reardon D, Halperin EC, Herndon J 2nd, Fuchs H, George T, Provenzale J, Watral M, McLendon RE, Friedman A, Friedman HS, Kurtzberg J, Vredenbergh J, Martin PL (2003) High-dose chemotherapy with autologous stem cell rescue in children and adults with newly diagnosed pineoblastomas. J Clin Oncol 21(11):2187–2191

    Article  CAS  PubMed  Google Scholar 

  23. Hinkes BG, Von Hoff K, Deinlein F, Warmuth-Metz M, Soerensen N, Timmermann B, Mittler U, Urban C, Bode U, Pietsch T, Schlegel PG, Kortmann RD, Kuehl J, Rutkowski S (2007) Childhood pineoblastoma: experiences from the prospective multicentre trials HIT-SKK87, HIT-SKK92 and HIT91. J Neuro-Oncol 81(2):217–223

    Article  CAS  Google Scholar 

  24. Chan AW, Tarbell NJ, Black PM, Louis DN, Frosch MP, Ancukiewicz M, Chapman P, Loeffler JS (2000) Adult medulloblastoma: prognostic factors and patterns of relapse. Neurosurgery 47(3):623–631

    CAS  PubMed  Google Scholar 

  25. Charafe-Jauffret E, Lehmann G, Fauchon F, Michiels JF, Paquis P, Maraninchi D, Hassoun J (2001) Vertebral metastases from pineoblastoma. Arch Pathol Lab Med 125(7):939–943

    CAS  PubMed  Google Scholar 

  26. Frank F, Gaist G, Piazza G, Ricci RF, Sturiale C, Galassi E (1985) Stereotaxic biopsy and radioactive implantation for interstitial therapy of tumours of the pineal region. Surg Neurol 23(3):275–280

    Article  CAS  PubMed  Google Scholar 

  27. Patil AA, Good R, Bashir R, Etemadrezaie H (1995) Non-resective treatment of pineoblastoma: a case report. Surg Neurol 44(4):386–393

    Article  CAS  PubMed  Google Scholar 

  28. Tarbell NJ, Friedman H, Polkinghorn WR, Yock T, Zhou T, Chen Z, Burger P, Barnes P, Kun L (2013) High-risk medulloblastoma: a pediatric oncology group randomized trial of chemotherapy before or after radiation therapy (POG 9031). J Clin Oncol 31(23):2936–2941

    Article  PubMed  PubMed Central  Google Scholar 

  29. Barlas O, Bayindir C, Imer M, Ayan I, Darendeliler E (2000) Nonresective management of pineoblastoma. Minim Invasive Neurosurg 43(3):163–170

    Article  CAS  PubMed  Google Scholar 

  30. Schild SE, Scheithauer BW, Schomberg PJ, Hook CC, Kelly PJ, Frick L, Robinow JS, Buskirk SJ (1993) Pineal parenchymal tumours. Clinical, pathologic, and therapeutic aspects. Cancer 72(3):870–880

    Article  CAS  PubMed  Google Scholar 

  31. Cohen BH, Zeltzer PM, Boyett JM, Geyer JR, Allen JC, Finlay JL, McGuire-Cullen P, Milstein JM, Rorke LB, Stanley P et al (1995) Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial. J Clin Oncol 13(7):1687–1696

    Article  CAS  PubMed  Google Scholar 

  32. Prados MD, Warnick RE, Wara WM, Larson DA, Lamborn K, Wilson CB (1995) Medulloblastoma in adults. Int J Radiat Oncol Biol Phys 32(4):1145–1152

    Article  CAS  PubMed  Google Scholar 

  33. Chin HW, Maruyama Y (1982) Prognostic factors in medulloblastoma. Am J Clin Oncol 5(4):359–369

    Article  CAS  PubMed  Google Scholar 

  34. Cumberlin RL, Luk KH, Wara WM, Sheline GE, Wilson CB (1979) Medulloblastoma: treatment results and effect on normal tissues. Cancer 43(3):1014–1020

    Article  CAS  PubMed  Google Scholar 

  35. Jakacki RI, Zeltzer PM, Boyett JM, Albright AL, Allen JC, Geyer JR, Rorke LB, Stanley P, Stevens KR, Wisoff J et al (1995) Survival and prognostic factors following radiation and/or chemotherapy for primitive neuroectodermal tumours of the pineal region in infants and children: a report of the Childrens Cancer Group. J Clin Oncol 13(6):1377–1383

    Article  CAS  PubMed  Google Scholar 

  36. Silverman CL, Simpson JR (1982) Cerebellar medulloblastoma: the importance of posterior fossa dose to survival and patterns of failure. Int J Radiat Oncol Biol Phys 8(11):1869–1876

    Article  CAS  PubMed  Google Scholar 

  37. Gorlia T, Van Den Bent MJ, Hegi ME, Mirimanoff RO, Weller M, Cairncross JG, Eisenhauer E, Belanger K, Brandes AA, Allgeier A, Lacombe D, Stupp R (2008) Nomograms for predicting survival of patients with newly diagnosed glioblastoma: prognostic factor analysis of EORTC and NCIC trial 26981–22981/CE 3. Lancet Oncol 9(1):29–38

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, Leeds General Infirmary, Leeds, LS1 3EX, UK

    Senthil K. Selvanathan, Oliver Richards, Saira Alli, Atul K. Tyagi & Paul D. Chumas

  2. Department of Paediatric Oncology, Leeds General Infirmary, Leeds, LS1 3EX, UK

    Martin Elliott

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  1. Senthil K. Selvanathan
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  2. Oliver Richards
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Correspondence to Oliver Richards.

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All authors certify that they have no affiliations with or involvement in any organisation or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest and expert testimony or patent-licencing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

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Selvanathan, S.K., Richards, O., Alli, S. et al. Outcome and prognostic features in paediatric pineoblastomas: analysis of cases from the Surveillance, Epidemiology, and End Results registry (1990–2007). Acta Neurochir 161, 1799–1807 (2019). https://doi.org/10.1007/s00701-019-03909-1

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