Advertisement

Supratentorial Primitive Neuroectodermal Tumor

  • Mary Frances McAleerEmail author
Chapter

Abstract

Supratentorial primitive neuroectodermal tumors (SPNET) are rare small round blue cell malignancies with distinct genetic alterations and worse outcomes than the more common infratentorial PNET, medulloblastoma, that most often affects young children less than 5 years of age. Clinical presentation varies with tumor location, and these tumors are often identified by distinct radiographic features including abnormal diffusion restriction on diffusion-weighted magnetic resonance imaging and absence of peritumoral edema, despite the large size of many of these tumors at presentation. Standard therapy for patients with SPNET has been extrapolated from medulloblastoma regimens and combines maximal safe resection and adjuvant radiotherapy and chemotherapy. Outcomes of patients with SPNET have been shown to vary with tumor location (pineal vs. non-pineal), stage, extent of resection, and patient age. This chapter summarizes the data supporting current management strategies, with particular focus on role and delivery of radiation treatment, for pediatric patients with this rare, aggressive brain tumor.

Keywords

Supratentorial primitive neuroectodermal tumor (SPNET) Pineoblastoma Pediatric Radiotherapy Combined modality therapy 

References

  1. Chintagumpala M, Hassall T, Palmer S, Ashley D, Wallace D, Kasow K, Merchant TE, Krasin MJ, Dauser R, Boop F, Krance R, Woo S, Cheuk R, Lau C, Gilbertson R, Gajjar A (2009) A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET. Neuro-Oncology 11(1):33–40. doi: 10.1215/15228517-2008-079 CrossRefPubMedPubMedCentralGoogle Scholar
  2. Cohen BH, Zeltzer PM, Boyett JM, Geyer JR, Allen JC, Finlay JL, McGuire-Cullen P, Milstein JM, Rorke LB, Stanley P, Stehbens JA, Shurin SB, Wisoff J, Stevens KR, Albright AL (1995) Prognostic factors and treatment results for supratentorial primitive neuroectodermaltumors in children using radiation and chemotherapy: a childrens cancer group randomized trial. J Clin Oncol 13:1687–1696CrossRefPubMedGoogle Scholar
  3. Dai AI, Backstrom JW, Burger PC, Duffner PK (2003) Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings. Pediatr Neurol 29(5):430–434. doi: 10.1016/s0887-8994(03)00313-8 CrossRefPubMedGoogle Scholar
  4. Dirks PB, Harris L, Hoffman HJ, Humphreys RP, Drake JM, Rutka JT (1996) Supratentorial primitive neuroectodermal tumors in children. J Neuro-Oncol 29:75–84CrossRefGoogle Scholar
  5. Dubuc AM, Northcott PA, Mack S, Witt H, Pfister S, Taylor MD (2010) The genetics of pediatric brain tumors. Curr Neurol Neurosci Rep 10(3):215–223. doi: 10.1007/s11910-010-0103-9 CrossRefPubMedGoogle Scholar
  6. Duffner PK, Horowitz ME, Krischer JP, Friedman HS, Burger PC, Cohen ME, Sanford RA, Mulhern RK, James HE, Freeman CR, Seidel FG, Kun LE (1993) Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328:1725–1731CrossRefPubMedGoogle Scholar
  7. Dufour C, Kieffer V, Varlet P, Raquin MA, Dhermain F, Puget S, Valteau-Couanet D, Grill J (2014) Tandem high-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuro-ectodermic tumors. Pediatr Blood Cancer 61(8):1398–1402. doi: 10.1002/pbc.25009 CrossRefPubMedGoogle Scholar
  8. Erdem E, Zimmeran RA, Haselgrove JC, Bilaniuk LT, Hunter JV (2001) Diffusion-weighted imaging and fluid attenuated inversion recovery imaging in the evaluation of primitive neuroectodermal tumors. Neuroradiology 43:927–933CrossRefPubMedGoogle Scholar
  9. 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, Rosenblum M, Dunkel I, Miller DC, 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(2):312–318. doi: 10.1002/pbc.21307 CrossRefPubMedGoogle Scholar
  10. Friedrich C, von Bueren AO, von Hoff K, Gerber NU, Ottensmeier H, Deinlein F, Benesch M, Kwiecien R, Pietsch T, Warmuth-Metz M, Faldum A, Kuehl J, Kortmann RD, Rutkowski S (2013) Treatment of young children with CNS-primitive neuroectodermal tumors/pineoblastomas in the prospective multicenter trial HIT 2000 using different chemotherapy regimens and radiotherapy. Neuro-Oncology 15(2):224–234. doi: 10.1093/neuonc/nos292 CrossRefPubMedGoogle Scholar
  11. Geyer JR, Zeltzer PM, Boyett JM, Rorke LB, Stanley P, Albright AL, Wisoff JH, Milstein JM, Allen JC, Finlay JL, Ayers GD, Shurin SB, Stevens KR, Bleyer WA (1994) Survival of infants with primitive neuroectodermal tumors or malignant ependymomas of the CNS treated with eight drugs in 1 day: a report from the childrens cancer group. J Clin Oncol 12:1607–1615CrossRefPubMedGoogle Scholar
  12. 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 pediatric pineoblastoma after surgery, radiation and chemotherapy. J Neuro-Oncol 89(1):89–95. doi: 10.1007/s11060-008-9589-2 CrossRefGoogle Scholar
  13. Hong TS, Mehta MP, Boyett JM, Donahue B, Rorke LB, Zeltzer PM (2005) Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG-921: a phase III combined modality study. Pediatr Blood Cancer 45(5):676–682. doi: 10.1002/pbc.20184 CrossRefPubMedGoogle Scholar
  14. Jakacki RI (2005) Treatment strategies for high-risk medulloblastoma and supratentorial primitive neuroectodermal tumors. J Neurosurg (Pediatrics 1) 102:44–52CrossRefGoogle Scholar
  15. Jakacki RI, Zeltzer PM, Boyett JM, Albright AL, Allen JC, Geyer JR, Rorke LB, Stanley P, Stevens KR, Wisoff J, McGuire-Cullen PL, Milstein JM, Packer RJ, Finlay JL (1995) Survival and prognostic factors following radiation and/or chemotherapy for primitive neuroectodermal tumors of the pineal region in infants and children: a report of the childrens cancer group. J Clin Oncol 13:1377–1383CrossRefPubMedGoogle Scholar
  16. Jimenez RB, Sethi R, Depauw N, Pulsifer MB, Adams J, McBride SM, Ebb D, Fullerton BC, Tarbell NJ, Yock TI, Macdonald SM (2013) Proton radiation therapy for pediatric medulloblastoma and supratentorial primitive neuroectodermal tumors: outcomes for very young children treated with upfront chemotherapy. Int J Radiat Oncol Biol Phys 87(1):120–126. doi: 10.1016/j.ijrobp.2013.05.017 CrossRefPubMedGoogle Scholar
  17. Johnston DL, Keene DL, Lafay-Cousin L, Steinbok P, Sung L, Carret AS, Crooks B, Strother D, Wilson B, Odame I, Eisenstat DD, Mpofu C, Zelcer S, Huang A, Bouffet E (2008) Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neuro-Oncol 86(1):101–108. doi: 10.1007/s11060-007-9440-1 CrossRefGoogle Scholar
  18. Kuhl J, Muller HL, Berthold F, Kortmann R-D, Deinlein F, Maab E, Graf N, Gnekow A, Scheurlen W, Gobel U, Wolff JEA, Bamberg M, Kaatsch P, Kleihues P, Rating D, Sorensen N, Wiestler OD (1998) Preradiation chemotherapy of children and young adults with malignant brain tumors: results of the German pilot trial HIT '88/'89. Klin Padiatr 210:227–233CrossRefPubMedGoogle Scholar
  19. MacDonald TJ, Rood BR, Santi MR, Vezina G, Bingaman K, Cogen PH, Packer RJ (2003) Advances in the diagnosis, molecular genetics, and treatment of pediatric embryonal CNS tumors. Oncologist 8:174–186CrossRefPubMedGoogle Scholar
  20. Marec-Berard P, Jouvet A, Thiesse P, Kalifa C, Doz F, Frappaz D (2002) Supratentorial embryonal tumors in children under 5 years of age: an SFOP study of treatment with postoperative chemotherapy alone. Med Pediatr Oncol 38(2):83–90. doi: 10.1002/mpo.1277 CrossRefPubMedGoogle Scholar
  21. Mason WP, Grovas A, Halpern S, Dunkel IJ, Garvin J, Heller G, Rosenblum M, Gardner S, Lyden D, Sands S, Puccetti D, Lindsley K, Merchant TE, O’Malley B, Bayer L, McElwain Petriccione M, Allen J, Finlay JL (1998) Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16:210–221CrossRefPubMedGoogle Scholar
  22. Massimino M, Gandola L, Spreafico F, Luksch R, Collini P, Giangaspero F, Simonetti F, Casanova M, Cefalo G, Pignoli E, Ferrari A, Terenziani M, Podda M, Meazza C, Polastri D, Poggi G, Ravagnani F, Fossati-Bellani F (2006) Supratentorial primitive neuroectodermal tumors (S-PNET) in children: a prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy. Int J Radiat Oncol Biol Phys 64(4):1031–1037. doi: 10.1016/j.ijrobp.2005.09.026 CrossRefPubMedGoogle Scholar
  23. 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 Biol Phys 72(5):1319–1323. doi: 10.1016/j.ijrobp.2008.03.033 CrossRefPubMedGoogle Scholar
  24. Miller S, Rogers HA, Lyon P, Rand V, Adamowicz-Brice M, Clifford SC, Hayden JT, Dyer S, Pfister S, Korshunov A, Brundler MA, Lowe J, Coyle B, Grundy RG (2011) Genome-wide molecular characterization of central nervous system primitive neuroectodermal tumor and pineoblastoma. Neuro-Oncology 13(8):866–879. doi: 10.1093/neuonc/nor070 CrossRefPubMedPubMedCentralGoogle Scholar
  25. Pizer BL, Weston CL, Robinson KJ, Ellison DW, Ironside J, Saran F, Lashford LS, Tait D, Lucraft H, Walker DA, Bailey CC, Taylor RE (2006) Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study. Eur J Cancer 42(8):1120–1128. doi: 10.1016/j.ejca.2006.01.039 CrossRefPubMedGoogle Scholar
  26. Pomeroy SL, Tamayo P, Gaasenbeek M, Sturla SL, Angelo M, McLaughlin ME, Kim JYH, Goumnerova LC, Black PM, Lau C, Allen JC, Zagzag D, Olson JM, Curran T, Wetmore C, Biegel JA, Poggio T, Mukherjee S, Rifkin R, Califano A, Stolovitzky G, Louis DN, Mesirov JP, Lander ES, Golub TR (2002) Prediction of central nervous system embryonal tumor outcome based on gene expression. Nature 415:436–442CrossRefPubMedGoogle Scholar
  27. Reddy AT, Janss AJ, Phillips PC, Weiss HL, Packer RJ (2000) Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy. Cancer 88:2189–2193CrossRefPubMedGoogle Scholar
  28. Rickert CH, Paulus W (2001) Epidemiology of central nervous tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst 17:503–511CrossRefPubMedGoogle Scholar
  29. Russo C, Pellarin M, Tingby O, Bollen AW, Lamborn KR, Mohapatra G, Collins VP, Feuerstein BG (1999) Comparative genomix hybridization in patients with supratentorial and infratentorial primitive neuroectodermal tumors. Cancer 86(2):331–339CrossRefPubMedGoogle Scholar
  30. South M, Chiu JK, Teh BS, Bloch C, Schroeder TM, Paulino AC (2008) Supine craniospinal irradiation using intrafractional junction shifts and field-in-field dose shaping: early experience at Methodist hospital. Int J Radiat Oncol Biol Phys 71(2):477–483. doi: 10.1016/j.ijrobp.2007.10.029 CrossRefPubMedGoogle Scholar
  31. 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 neuroectodaermal tumor after craniospinal radiotherapy: results of a collaborative study. J Clin Oncol 19:2696–2704CrossRefPubMedGoogle Scholar
  32. Strother DR, Lafay-Cousin L, Boyett JM, Burger P, Aronin P, Constine L, Duffner P, Kocak M, Kun LE, Horowitz ME, Gajjar A (2014) Benefit from prolonged dose-intensive chemotherapy for infants with malignant brain tumors is restricted to patients with ependymoma: a report of the Pediatric Oncology Group randomized controlled trial 9233/34. Neuro-Oncology 16(3):457–465. doi: 10.1093/neuonc/not163 CrossRefPubMedGoogle Scholar
  33. Tate M, Sughrue ME, Rutkowski MJ, Kane AJ, Aranda D, McClinton L, McClinton L, Barani IJ, Parsa AT (2012) The long-term postsurgical prognosis of patients with pineoblastoma. Cancer 118(1):173–179. doi: 10.1002/cncr.26300 CrossRefPubMedGoogle Scholar
  34. Terterov S, Krieger MD, Bowen I, McComb JG (2010) Evaluation of intracranial cerebrospinal fluid cytology in staging pediatric medulloblastomas, supratentorial primitive neuroectodermal tumors, and ependymomas. J Neurosurg Pediatr 6(2):131–136. doi: 10.3171/2010.5.PEDS09333 CrossRefPubMedGoogle Scholar
  35. Timmermann B, Kortmann R-D, 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 20:842–849CrossRefPubMedGoogle Scholar
  36. Varan A, Yazici N, Akalan N, Yalcin B, Akyuz C, Kutluk T, Buyukpamukcu M (2012) Primitive neuroectodermal tumors of the central nervous system associated with genetic and metabolic defects. J Neurosurg Sci 56(1):49–53PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Radiation OncologyThe University of Texas MD Anderson Cancer CenterHoustonUSA

Personalised recommendations