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Supratentorial Primitive Neuroectodermal Tumors

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Tumors of the Central Nervous System, Volume 4

Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 4))

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Abstract

Supratentorial primitive neuroectodermal tumor (sPNET) only represents 2.5–3% of childhood brain tumors. It has a worse prognosis than its posterior fossa counterpart and a different molecular signature has been demonstrated. The mainstay of therapy is gross total resection followed by adjuvant therapy that is age dependant, in older children this involves craniospinal radiation and chemotherapy, in the infant different approaches to minimize radiation have been attempted. Prognosis is variable between 5 and 30% 5 year survival, dependent on the child’s age, the presence of dissemination at diagnosis and the treatment options available.

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References

  • Abeloff M (2008) Cancers of the CNS. Abeloff’s clinical oncology, 4th edn, Ch 70. Elsevier, Churchill Livingstone

    Google Scholar 

  • Albright AL, Wisoff JH, Zeltzer P, Boyett J, Rorke LB, Stanley P, Geyer JR, Milstein JM (1995) Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. A neurosurgical perspective from the Children’s Cancer Group. Pediatr Neurosurg 22:1–7

    Article  PubMed  CAS  Google Scholar 

  • Arsic D, Cameron V, Ellmers L, Quan QB, Keenan J, Beasley S (2004) Adriamycin disruption of the Shh-Gli pathway is associated with abnormalities of foregut development. J Pediatr Surg 39:1747–1753

    Article  PubMed  Google Scholar 

  • Bajaj A, Bajaj SK, Bajaj N (2007) Bilateral retinoblastoma in early infancy: a case report. Kathmandu Univ Med J 5:550–551

    CAS  Google Scholar 

  • Burnett ME, White EC, Sih S, von Haken MS, Cogen PH (1997) Chromosome arm 17p deletion analysis reveals molecular genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumors of the central nervous system. Cancer Genet Cytogenet 97:25–31

    Article  PubMed  CAS  Google Scholar 

  • Dai AI, Backstrom JW, Burger PC, Duffner PK (2003) Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings. Pediatr Neurol 29:430–434

    Article  PubMed  Google Scholar 

  • Dirks PB, Harris L, Hoffman HJ, Humphreys RP, Drake JM, Rutka JT (1996) Supratentorial primitive neuroectodermal tumors in children. J Neurooncol 29:75–84

    Article  PubMed  CAS  Google Scholar 

  • Dunkel IJ, Jubran RF, Gururangan S, Chantada GL, Finlay JL, Goldman S, Khakoo Y, Obrein JM, Orjuela M, Rodriguez-Galindo C, Souweidane MM, Abramson DH (2010) Trilateral retinoblastoma: potentially curable with intensive chemotherapy. Pediatr Blood Cancer 54:384–387

    Article  PubMed  Google Scholar 

  • Durkin ME, Yuan BZ, Zhou X, Zimonjic DB, Lowy DR, Thorgeirsson SS, Popescu NC (2007) DLC-1:a Rho GTPase-activating protein and tumour suppressor. J Cell Mol Med 11:1185–1207

    Article  PubMed  CAS  Google Scholar 

  • Fangusaro J, Finlay J, Sposto R, Ji L, Saly M, Zacharoulis S, Asgharzadeh S, Abromowitch M, Olshefski R, Halpern S, Dubowy R, Comito M, Diez B, Kellie S, Hukin J, Rosenbulm M, Dunkel I, Miller D, Allen J, Gardner S (2008) Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and II experience. Pediatr Blood Cancer 50:312–318

    Article  PubMed  Google Scholar 

  • Farrell CJ, Plotkin SR (2007) Genetic causes of brain tumors: neurofibromatosis, tuberous sclerosis, von Hippel-Lindau, and other syndromes. Neurol Clin 25:925–946

    Article  PubMed  Google Scholar 

  • Girschick HJ, Klein R, Scheurlen WG, Kuhl J (2001) Cytogenetic and histopathologic studies of congenital supratentorial primitive neuroectodermal tumors: a case report. Pathol Oncol Res 7:67–71

    Article  PubMed  CAS  Google Scholar 

  • Jakacki RI (1999) Pineal and nonpineal supratentorial primitive neuroectodermal tumors. Childs Nerv Syst 15:586–591

    Article  PubMed  CAS  Google Scholar 

  • Johnston DL, Keene DL, Lafay-Cousin L, Steinbok P, Sung L, Carret AS, Crooks B, Strother D, Wilson B, Odame I, Eisenstat D, Mpofu C, Zelcer S, Huang A, Bouffet E (2008) Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol 86:101–108

    Article  PubMed  Google Scholar 

  • Kivelam T (1999) Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. J ClinOncol 17:1829–1837

    Google Scholar 

  • Klisch J, Husstedt H, Hennings S, von Velthoven V, Pagenstecher A, Schumacher M (2000) Supratentorial primitive neuroectodermal tumours: diffusion-weighted MRI. Neuroradiology 42:393–398

    Article  PubMed  CAS  Google Scholar 

  • Kuhn SA, Hanisch UK, Ebmeier K, Beetz C, Brodhun M, Reichart R, Ewald C, Deufel T, Kalff R (2007) A paediatric supratentorial primitive neuroectodermal tumour associated with malignant astrocytic transformation and a clonal origin of both components. Neurosurg Rev 30:143–149

    Article  PubMed  Google Scholar 

  • Li MH, Bouffet E, Hawkins CE, Squire JA, Huang A (2005) Molecular genetics of supratentorial primitive neuroectodermal tumors and pineoblastoma. Neurosurg Focus 19:E3

    PubMed  Google Scholar 

  • Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109

    Article  PubMed  Google Scholar 

  • McBride SM, Daganzo SM, Banerjee A, Gupta N, Lamborn KR, Prados MD, Berger MS, Wara WM, Haas-Kogan DA (2008) Radiation is an important component of multimodality therapy for pediatric non-pineal supratentorial primitive neuroectodermal tumors. Int J Radiat Oncol 72:1319–1323

    Article  Google Scholar 

  • Mueller S, Chang S (2009) Pediatric brain tumors: current treatment strategies and future therapeutic approaches. Neurotherapeutics 6:570–586

    Article  PubMed  CAS  Google Scholar 

  • Pang J, Chang Q, Chung YF, Teo JG, Poon WS, Zhou LF, Kong X, Ng HK (2005) Epigenetic inactivation of DLC-1 in supratentorial primtive neuroectodermal tumor. Human Pathol 36:36–43

    Article  CAS  Google Scholar 

  • Paulino AC (1999) Trilateral retinoblastoma: is the location of the intracranial tumor important? Cancer 86:135–141

    Article  PubMed  CAS  Google Scholar 

  • Pelengaris S, Khan M, Evan G (2002) c-MYC: more than just a matter of life and death. Nat Rev Cancer 2:764–776

    Article  PubMed  CAS  Google Scholar 

  • Pizer BL, Weston CL, Robinson KJ, Ellison DW, Ironside J, Saran F, Lashford LS, Tait D, Lucraft H, Walker DA (2006) Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study. Eur J Cancer 42:1120–1128

    Article  PubMed  Google Scholar 

  • Plum F, Posner JB (1980) Structural causes of stupor and coma. The diagnosis of stupor and coma, 3rd edn, Ch 3: 93. F.A. Davis Company, Philadelphia

    Google Scholar 

  • Siffert J, Allen JC (1997) Medulloblastoma. J Neuro-Oncol 24(68):181–209

    Google Scholar 

  • Strother D, Ashley D, Kellie SJ, Patel A, Jones-Wallace D, Thompson S, Heideman R, Benaim E, Krance R, Bowman L, Gajjar A (2001) Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study. J Clin Oncol 19:2696–2704

    PubMed  CAS  Google Scholar 

  • Sung KW, Yoo KH, Cho EJ, Koo HH, Lim do H, Shin HJ, Ahn SD, Ra YS, Choi ES, Ghim TT (2007) High-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk or relapsed medulloblastoma or supratentorial primitive neuroectodermal tumor. Pediatr Blood Cancer 48:408–415

    Article  PubMed  Google Scholar 

  • Timmermann B, Kortmann RD, Kuhl J, Meisner C, Dieckmann K, Pietsch T, Bamberg M (2002) Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol 20:842–849

    Article  PubMed  Google Scholar 

  • Timmermann B, Kortmann RD, Kuhl J, Rutkowski S, Meisner C, Pietsch T, Deinlein F, Urban C, Warmuth-Metz M, Bamberg M (2006) Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials. J Clin Oncol 24:1554–1560

    Article  PubMed  Google Scholar 

  • Yang HJ, Nam DH, Wang KC, Kim YM, Chi JG, Cho BK (1999) Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors. Childs Nerv Syst 15:377–383

    Article  PubMed  CAS  Google Scholar 

  • Zurawel RH, Allen C, Chiappa S, Cato W, Biegel J, Cogen P, de Sauvage F, Raffel C (2000) Analysis of PTCH/SMO/SHH pathway genes in medulloblastoma. Genes Chromosome Cancer 27:44–51

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Neurology Division ACB, K3-145, British Columbia Children’s Hospital, Vancouver, BC, V6H3V4, Canada

    Mohit Singh & Juliette Hukin

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  1. Mohit Singh
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  2. Juliette Hukin
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Correspondence to Juliette Hukin .

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  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

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Singh, M., Hukin, J. (2012). Supratentorial Primitive Neuroectodermal Tumors. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 4. Tumors of the Central Nervous System, vol 4. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-1706-0_2

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  • DOI: https://doi.org/10.1007/978-94-007-1706-0_2

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  • Print ISBN: 978-94-007-1705-3

  • Online ISBN: 978-94-007-1706-0

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