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Imaging of the Brainstem Tumors

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Posterior Fossa Tumors in Children

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Abstract

Brainstem tumors are diverse, with a pathology closely related to their topography. Accordingly a precise evaluation of the lesional anatomy is requested for diagnosis, as well as a detailed structural study. This includes conventional T1, T2 and FLAIR, DWI/ADC for cellular density, T2* imaging for blood, and contrast administration for vascularity and BBB leakage. DTI may be helpful in assessing location and involvement of the longitudinal tracts. The brainstem is divided craniocaudally into midbrain, pons, medulla, and medullo-cervical junction and ventrodorsally into basal and tegmental segments. The most common, and devastating, brainstem tumor is the diffuse infiltrating pontine glioma (DIPG): it develops in the ventral pons, as an infiltrative often anteriorly exophytic mass. Developing nodules with restricted diffusion point to high cellularity often associated with contrast enhancement, necrosis, micro-bleeds, and dilated arterial and venous perforators. Perfusion imaging would show high blood flow and MR spectroscopy high lactate and lipids. By contrast, the tegmental tumors are typically pilocytic astrocytomas, dorsally exophytic into the ventricular lumen. They may be solid, cystic, or both, infiltrate the brainstem (which ependymomas do not do), and usually enhance; still perfusion is low, but lactate may be high. The medullo-cervical tumors may be gangliogliomas. Ventral tumors of the medulla or midbrain may be benign or malignant, diffuse infiltrative tumors being fibrillary astrocytomas. Tectal tumors (actually not really of the brainstem) usually are low-grade gliomas. Other than PNETs and the highly vascular hemangioblastomas, differential includes cavernomas, inflammation, and abscesses.

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References

  1. Epstein F (1985) A staging system for brain stem gliomas. Cancer 56:1804–1806

    Article  CAS  PubMed  Google Scholar 

  2. CBTRUS (2007–2008) Primary brain tumors in the United States. Statistical report 2000–2004

    Google Scholar 

  3. Hayward RM, Patronas M, Baker EH et al (2008) Inter-observer variability in the measurement of diffuse intrinsic pontine gliomas. J Neurooncol 90:57–61

    Article  PubMed Central  PubMed  Google Scholar 

  4. Löbel U, Sedlacik J, Reddick WE et al (2011) Quantitative diffusion-weighted and dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging analysis of T2 hypointense lesion components in pediatric diffuse intrinsic pontine glioma. AJNR Am J Neuroradiol 32(2):315–322

    Article  PubMed Central  PubMed  Google Scholar 

  5. Berger MS, Edwards MSB, LaMasters D et al (1983) Pediatric brain stem tumors: radiographic, pathological and clinical correlations. Neurosurgery 12:298–302

    Article  CAS  PubMed  Google Scholar 

  6. Curless RC, Bowen BC, Pattany PM et al (2002) Magnetic resonance spectroscopy in childhood brainstem tumors. Pediatr Neurol 26:374–378

    Article  PubMed  Google Scholar 

  7. Helton KJ, Phillips NS, Khan RB et al (2006) Diffusion tensor imaging of tract involvement in children with pontine tumors. AJNR Am J Neuroradiol 27:786–793

    CAS  PubMed  Google Scholar 

  8. Schumacher M, Schulte-Mönting J, Stoeter P et al (2007) Magnetic resonance imaging compared with biopsy in the diagnosis of brainstem diseases of childhood: a multicenter review. J Neurosurg 106(2 Suppl Pediatrics):111–119

    PubMed  Google Scholar 

  9. Löbel U, Sedlacik J, Sabin ND et al (2010) Three-dimensional susceptibility-weighted imaging and two-dimensional T2*-weighted gradient-echo imaging of intratumoral hemorrhages in pediatric diffuse intrinsic pontine glioma. Neuroradiology 52:1167–1177

    Article  PubMed Central  PubMed  Google Scholar 

  10. Recinos PF (2007) Brainstem tumors: where are we today? Pediatr Neurosurg 43:192–201

    Article  PubMed  Google Scholar 

  11. Fischbein NJ (1996) Radiological classification of brain stem tumors: correlation of magnetic resonance imaging appearance with clinical outcome. Pediatr Neurosurg 24:9–23

    Article  CAS  PubMed  Google Scholar 

  12. Epstein FJ, Farmer JP (1993) Brain-stem glioma growth patterns. J Neurosurg 78:408–412

    Article  CAS  PubMed  Google Scholar 

  13. ten Donkelaar HJ, Lammens M, Cruysberg JRM, Cremers CWJR (2006) Development and developmental disorders of the brain stem. In: ten Donkelaar HL, Lammens M, Hori A (eds) Clinical neuroembryology. Springer, Berlin Heidelberg pp 269–308

    Chapter  Google Scholar 

  14. Keynes R, Lumsden A (1990) Segmentation and the origin of regional diversity in the vertebrate central nervous system. Neuron 2:1–9

    Article  Google Scholar 

  15. ten Donkelaar HJ, Lammens M, Cruysberg JRM et al (2006) Development and developmental disorders of the forebrain. In: ten Donkelaar HL, Lammens M, Hori A (eds) Clinical neuroembryology. Springer, Berlin Heidelberg pp 345–428

    Chapter  Google Scholar 

  16. Puelles L, Rubenstein LR (2003) Forebrain gene expression domains and the evolving prosomeric model. Trends Neurosc 26:469–476

    Article  CAS  Google Scholar 

  17. Vescovi AL, Galli R, Reynolds BA (2006) Brain tumour stem cells. Nat Rev Cancer 6:425–436

    Article  CAS  PubMed  Google Scholar 

  18. Gilbertson RJ, Gutmann DH (2007) Tumorigenesis in the brain: location, location, location. Cancer Res 67:5579–5582

    Article  CAS  PubMed  Google Scholar 

  19. Dirks PB (2008) Brain tumour stem cells: the undercurrents of human brain cancer and their relationship to neural stem cells. Phil Trans R Soc B 363:139–152

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  20. Sehgal V, Delproposto Z, Haacke EM et al (2005) Clinical applications of neuroimaging with susceptibility-weighted imaging. J Magn Reson Imaging 22:439–450

    Article  PubMed  Google Scholar 

  21. Sehgal V, Delproposto Z, Haddar D et al (2006) Susceptibility-weighted imaging to visualize blood products and improve tumor contrast in the study of brain masses. J Magn Reson Imaging 24:41–51

    Article  PubMed  Google Scholar 

  22. Rauscher A, Sedlacik J, Barth M et al (2005) Noninvasive assessment of vascular architecture and function during modulated blood oxygenation using susceptibility-weighted magnetic resonance imaging. Magn Reson Med 54:87–95

    Article  PubMed  Google Scholar 

  23. Chen X, Weigel D, Ganslandt O et al (2007) Diffusion tensor imaging and white matter tractography in patients with brainstem lesions. Acta Neurochir (Wien) 149:1117–1131

    Article  CAS  Google Scholar 

  24. Helton KJ, Weeks JK, Pillips NS et al (2008) Diffusion tensor imaging of brainstem tumors: axonal degeneration of motor and sensory tracts. J Neurosurg Pediatrics 1:270–276

    Article  Google Scholar 

  25. Giussani C, Poliakov A, Ferri RT et al (2010) DTI fiber tracking to differentiate demyelinating diseases from diffuse brain stem glioma. NeuroImage 52:217–223

    Article  PubMed  Google Scholar 

  26. Krieger MD, Blüml S, McComb JG (2003) Magnetic resonance spectroscopy of atypical diffuse pontine masses. Neurosurg Focus 15(1):E5

    PubMed  Google Scholar 

  27. Panigrahy A, Nerson MD Jr, Finlay JL et al (2008) Metabolism of diffuse intrinsic brainstem gliomas in children. Neuro-Oncology 10:32–44

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  28. Laprie A, Pirzkall A, Haas-Kogan DA et al (2005) Longitudinal multivoxel MR spectroscopy study of pediatric diffuse brainstem glioma treated with radiotherapy. Int J Radiat Oncol Biol Phys 62:20–31

    Article  PubMed  Google Scholar 

  29. Warmuth C, Günther M, Zimmer C (2003) Quantification of blood flow in brain tumors: comparison of arterial spin labeling and dynamic susceptibility-weighted contrast-enhanced MR imaging. Radiology 228:523–532

    Article  PubMed  Google Scholar 

  30. Grand S, Lefournier V, Krainik A et al (2007) Imagerie de perfusion: principes et applications cliniques. J Radiol 88:444–471

    Article  CAS  PubMed  Google Scholar 

  31. Alvarez-Linera J (2010) Magnetic resonance techniques for the brainstem. Semin Ultrasound CT MRI 31:230–245

    Article  CAS  Google Scholar 

  32. Komotar RJ, Zacharia BE, Sughrue ME et al (2008) Magnetic resonance imaging characteristics of pilomyxoid astrocytomas. Neurol Res 30:945–951

    Article  PubMed  Google Scholar 

  33. Lee IH, Kim JH, Suh YL et al (2010) Imaging characteristics of pilomyxoid astrocytoma in comparison with pilocytic astrocytomas. Eur J Radiol 79:311–316

    Google Scholar 

  34. Jallo GI, Biser-Rohrbaugh A, Freed D (2004) Brainstem gliomas. Childs Nerv Syst 20:143–153

    Article  PubMed  Google Scholar 

  35. Hoffman HJ, Becker L, Craven MA (1980) A clinically and pathologically distinct group of benign brainstem gliomas. Neurosurgery 7:243–248

    Article  CAS  PubMed  Google Scholar 

  36. Farmer JP, Montes JL, Freeman CR et al (2001) Brainstem gliomas. Pediatr Neurosurg 34:206–214

    Article  CAS  PubMed  Google Scholar 

  37. Kestle J, Townsend JJ, Brockmeyer DL, Walker ML (2004) Juvenile pilocytic astrocytoma of the brainstem in children. J Neurosurg (Pediatrics 2) 101:1–6

    Article  Google Scholar 

  38. Epstein F, Wisoff J (1987) Intraaxial tumors of the cervicomedullary junction. J Neurosurg 67:483–487

    Article  CAS  PubMed  Google Scholar 

  39. Poussaint TY, Yousuf N, Barnes PD et al (1999) Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up. Pediatr Radiol 29:662–668

    Article  Google Scholar 

  40. Abbott R, Shiminski-Maher T, Wisoff JH, Epstein FJ (1991) Intrinsic tumors of the medulla: surgical complications. Pediatr Neurosurg 17:239–244

    Article  PubMed  Google Scholar 

  41. Sandri A, Sardi N, Genitori L et al (2006) Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome. Childs Nerv Syst 22:1127–1135

    Article  CAS  PubMed  Google Scholar 

  42. Ternier J, Wray A, Puget S et al (2006) Tectal plate lesions in children. J Neurosurg 104(6 Suppl Pediatrics):369–373

    PubMed  Google Scholar 

  43. Konno H, Yamamoto T, Iwasaki Y et al (1985) A case of quadrigeminal hamartoma. Acat Neuropathol (Berl) 68:155–159

    Article  CAS  Google Scholar 

  44. Robertson PL, Murazko KM, Brunberg JA et al (1995) Pediatric midbrain tumors: a benign subgroup of brainstem gliomas. Pediatr Neurosurg 22:65–73

    Google Scholar 

  45. Dağlioğlu E, Çataltepe O, Akalan N (2003) Tectal gliomas in children: the implications for natural history and management strategy. Pediatr Neurosurg 38:223–231

    Article  PubMed  Google Scholar 

  46. Vandertop WP, Hoffman HJ, Drake JM et al (1992) Focal midbrain tumors in children. Neurosurgery 31:186–194

    Article  CAS  PubMed  Google Scholar 

  47. Tomita T, Cortes RF (2002) Astrocytomas of the cerebral peduncle in children: surgical experience in seven patients. Childs Nerv Syst 18:225–230

    Article  PubMed  Google Scholar 

  48. Sun B, Wang CC, Wang G (1999) MRI characteristics of midbrain tumors. Neuroradiology 41:158–162

    Article  CAS  PubMed  Google Scholar 

  49. Sanford RA, Bebin J, Smith WR (1982) Pencil glioma of the aqueduct of Sylvius. J Neurosurg 57:690–696

    Article  CAS  PubMed  Google Scholar 

  50. Boydston WR, Sanford RA, Muhlbauer MS et al (1991–1992) Glioma of the tectum and periaqueductal region of the mesencephalon. Pediatr Neurosurg 17:234–238

    Google Scholar 

  51. Marcorelles P, Fallet-Bianco C, Oury JF et al (2005) Fetal aqueductal glioneuronal hamartoma : a clinicopathological and physiopathological study of three cases. Clin Neuropathol 24:155–162

    CAS  PubMed  Google Scholar 

  52. Broniscer A, Laningham FH, Kocak M et al (2006) Intratumoral hemorrhage among children with newly diagnosed, diffuse brainstem glioma. Cancer 106:1364–1371

    Article  PubMed  Google Scholar 

  53. Grand SD, Kremer S, Tropres IM et al (2007) Perfusion-sensitive MRI of pilocytic astrocytomas: initial results. Neuroradiology 49:545–550

    Article  PubMed  Google Scholar 

  54. Kumar VA, Knopp EA, Zagzag D (2010) Magnetic resonance dynamic susceptibility-weighted contrast-enhanced perfusion imaging in the diagnosis ofof posterior fossa hemangioblastomas and pilocytic astrocytomas: initial results. J Comput Assist Tomogr 34:825–829

    Article  PubMed  Google Scholar 

  55. Milanaccio C, Nozza P, Ravegnani M et al (2005) Cervico-medullary desmoplastic infantile ganglioglioma: an unusual case with diffuse leptomeningeal dissemination at diagnosis. Pediatr Blood Cancer 45:986–990

    Article  PubMed  Google Scholar 

  56. Covington DB, Rosenblum MK, Brathwaite CD, Sandberg DI (2009) Angiocentric glioma-like tumor of the midbrain. Pediatr Neurosurg 45:429–433

    Article  PubMed  Google Scholar 

  57. Bilaniuk LT, Molloy PT, Zimmerman RA et al (1997) Neurofibromatosis type 1: brain stem tumors. Neuroradiology 39:642–653

    Article  CAS  PubMed  Google Scholar 

  58. Rosser T, Packer RJ (2002) Intracranial neoplasms in patients with neurofibromatosis 1. J Child Neurol 17:630–637

    Article  PubMed  Google Scholar 

  59. Ulrich NJ, Raja AI, Irons MB et al (2007) Brainstem lesions in neurofibromatosis type 1. Neurosurgery 61:762–767

    Article  Google Scholar 

  60. Terazaki M, Uchikado H, Takeuchi Y, Shigemori M (2005) Minimally invasive management of ependymoma of the aqueduct of Sylvius: therapeutic considerations and management. Minim Invasive Neurosurg 48:322–324

    Article  Google Scholar 

  61. Keene DL, Hsu E, Ventureyra E et al (1998) An unusual behavior of brainstem ependymoma. J Child Neurol 13:86–88

    Article  CAS  PubMed  Google Scholar 

  62. U-King-Im JM, Taylor MD, Raybaud C (2010) Posterior fossa ependymomas: new radiological classification with surgical correlation. Childs Nerv Syst 26:1765–1772

    Article  PubMed  Google Scholar 

  63. Zagzag D, Miller DC, Knopp E et al (2000) Primitive neuroectodermal tumors in the brainstem: investigation of seven cases. Pediatrics 106:1045–1053

    Article  CAS  PubMed  Google Scholar 

  64. Gavriel H, Shuper A, Kornreich L et al (2000) Diffuse intrinsic brainstem disease with neurologic deterioration : not what it seemed. Med Ped Oncol 34:213–214

    Article  CAS  Google Scholar 

  65. Guzmán-de-Villoria JA, Fernández-Garcia P, Ferreiro-Argüelles C (2010) Differential diagnosis of T2 hyperintense brainstem lesions. Part 1. Focal lesions. Semin Ultrasound CT MRI 31:246–259

    Article  Google Scholar 

  66. Guzmán-de-Villoria JA, Fernández-Garcia P, Ferreiro-Argüelles C (2010) Differential diagnosis of T2 hyperintense brainstem lesions. Part 2. Diffuse lesions. Semin Ultrasound CT MRI 31:260–274

    Article  Google Scholar 

  67. Giliberto G, Lanzino DJ, Diehn FE et al (2010) Brainstem cavernous malformations : anatomical, clinical and surgical considerations. Neurosurg Focus 29:E9

    Article  PubMed  Google Scholar 

  68. Lebre RM, de Lonlay P, Valayannopoulos V et al (2010) Mitochondrial ND5 mutations mimicking brainstem tectal glioma. Neurology 75:93

    Article  PubMed  Google Scholar 

  69. van Poppel K, Broniscer A, Patay Z, Morris EB (2009) Alexander disease: an important mimicker of focal brainstem glioma. Pediatr Blood Cancer 53:1355–1356

    Article  PubMed Central  PubMed  Google Scholar 

  70. Wagner S, Benesh M, Berthold F et al (2006) Secondary dissemination in children with high-grade malignant gliomas and diffuse pontine gliomas. Brit J Cancer 95:991–997

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  71. Singh S, Bhutani R, Jalali R (2007) Leptomeninges as a site of relapse of locally controlled, diffuse pontine glioma with review of the literature. Childs Nerv Syst 23:117–121

    Article  PubMed  Google Scholar 

  72. Sethi R, Allen J, Donahue B et al (2010) Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma. J Neurooncol 102:121–127

    Google Scholar 

  73. Kornreich L, Schwarz M, Kamarzyn B et al (2005) Role of MRI in the management of children with diffuse pontine tumors: a study of 15 patients and review of the literature. Pediatr Radiol 35:872–879

    Article  PubMed  Google Scholar 

  74. Prabhu S, Ng S, Vajapeyam S et al (2011) DTI assessment of the brainstem white matter tracts in pediatric BSG before and after therapy. Childs Nerv Syst 27:11–18

    Article  PubMed Central  PubMed  Google Scholar 

  75. Bobek-Billiewicz B, Stasil-Pres G, Majchrzak H, Zarudzki L (2010) Differentiation between brain tumor recurrence and radiation necrosis using perfusion, diffusion-weighted imaging and MR spectroscopy. Folia Neuropathol 48:81–92

    Google Scholar 

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Authors and Affiliations

  1. Division Head of Neuroradiology, Hospital for Sick Children and University of Toronto, 555 University Avenue, Toronto, ON, M5G1X8, Canada

    Charles Raybaud M.D.

  2. Department of Medical Imaging, King Abdulaziz Medical City, 9515, Jeddah, 28273, Saudi Arabia

    Abeer Almehdar M.D.

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  1. Charles Raybaud M.D.
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Correspondence to Charles Raybaud M.D. .

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Editors and Affiliations

  1. Neurosurgery, Acıbadem University, Istanbul, Istanbul, Turkey

    M. Memet Özek

  2. Pediatric Neurosurgery, Santobono Children's Hospital, Naples, Napoli, Italy

    Giuseppe Cinalli

  3. Dept. Neurosurgery, Royal Childrens Hospital, Melbourne, Victoria, Australia

    Wirginia Maixner

  4. Pediatric Neurosurgery, Hopital Necker-Enfants Malades, Paris, Paris, France

    Christian Sainte-Rose

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Raybaud, C., Almehdar, A. (2015). Imaging of the Brainstem Tumors. In: Özek, M., Cinalli, G., Maixner, W., Sainte-Rose, C. (eds) Posterior Fossa Tumors in Children. Springer, Cham. https://doi.org/10.1007/978-3-319-11274-9_31

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  • DOI: https://doi.org/10.1007/978-3-319-11274-9_31

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-11273-2

  • Online ISBN: 978-3-319-11274-9

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