Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Clinical manifestations and diagnosis of central nervous system tumors in children

Ching Lau, MD, PhD
Section Editor
David G Poplack, MD
Deputy Editor
Carrie Armsby, MD, MPH


Central nervous system (CNS) tumors include both nonmalignant and malignant tumors of the brain and spinal cord. Primary malignant CNS tumors are the second most common childhood malignancies, after hematologic malignancies, and are the most common pediatric solid organ tumor [1]. They are the leading cause of death from childhood cancer, surpassing the mortality rate of acute lymphoblastic leukemia [2].

A general overview on the clinical manifestations and diagnosis of CNS tumors in children will be reviewed here. The clinical manifestations and diagnosis of the following specific CNS tumors that occur in children are discussed separately.

Low-grade gliomas (see "Classification and pathologic diagnosis of gliomas")

Malignant gliomas (see "Clinical presentation, initial surgical approach, and prognosis of high-grade gliomas")

Medulloblastoma (see "Histopathology and molecular pathogenesis of medulloblastoma")

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Sep 22, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Linabery AM, Ross JA. Trends in childhood cancer incidence in the U.S. (1992-2004). Cancer 2008; 112:416.
  2. Homer MJ, Ries LAG, Krapcho M, et al (Eds). SEER Cancer Statistics Review, 1975-2006 http://seer.cancer.gov/csr/1975_2006/ (Accessed on May 20, 2008).
  3. Wilne SH, Dineen RA, Dommett RM, et al. Identifying brain tumours in children and young adults. BMJ 2013; 347:f5844.
  4. Wilne S, Collier J, Kennedy C, et al. Presentation of childhood CNS tumours: a systematic review and meta-analysis. Lancet Oncol 2007; 8:685.
  5. Wilne SH, Ferris RC, Nathwani A, Kennedy CR. The presenting features of brain tumours: a review of 200 cases. Arch Dis Child 2006; 91:502.
  6. Hayashi N, Kidokoro H, Miyajima Y, et al. How do the clinical features of brain tumours in childhood progress before diagnosis? Brain Dev 2010; 32:636.
  7. Teo WY, Myseros JS. The gut or the brain?--Gastrointestinal misdiagnoses of infantile brain tumors. Childs Nerv Syst 2014; 30:1449.
  8. Fattal-Valevski A, Nissan N, Kramer U, Constantini S. Seizures as the clinical presenting symptom in children with brain tumors. J Child Neurol 2013; 28:292.
  9. Pollack IF. Pediatric brain tumors. Semin Surg Oncol 1999; 16:73.
  10. Taylor M, Couto-Silva AC, Adan L, et al. Hypothalamic-pituitary lesions in pediatric patients: endocrine symptoms often precede neuro-ophthalmic presenting symptoms. J Pediatr 2012; 161:855.
  11. Gropman AL, Packer RJ, Nicholson HS, et al. Treatment of diencephalic syndrome with chemotherapy: growth, tumor response, and long term control. Cancer 1998; 83:166.
  12. Pillai MG, Unnikrishnan AG, Nair V, et al. Diencephalic cachexia: a rare cause for failure to thrive. J Pediatr 2005; 147:713.
  13. Fleischman A, Brue C, Poussaint TY, et al. Diencephalic syndrome: a cause of failure to thrive and a model of partial growth hormone resistance. Pediatrics 2005; 115:e742.
  14. Ullrich NJ. Neurocutaneous Syndromes and Brain Tumors. J Child Neurol 2016; 31:1399.
  15. Lasky JL, Choi EJ, Johnston S, et al. Congenital brain tumors: case series and review of the literature. J Pediatr Hematol Oncol 2008; 30:326.
  16. Isaacs H Jr. I. Perinatal brain tumors: a review of 250 cases. Pediatr Neurol 2002; 27:249.
  17. Severino M, Schwartz ES, Thurnher MM, et al. Congenital tumors of the central nervous system. Neuroradiology 2010; 52:531.
  18. Wilne S, Walker D. Spine and spinal cord tumours in children: a diagnostic and therapeutic challenge to healthcare systems. Arch Dis Child Educ Pract Ed 2010; 95:47.
  19. Wilson PE, Oleszek JL, Clayton GH. Pediatric spinal cord tumors and masses. J Spinal Cord Med 2007; 30 Suppl 1:S15.
  20. Wilne S, Koller K, Collier J, et al. The diagnosis of brain tumours in children: a guideline to assist healthcare professionals in the assessment of children who may have a brain tumour. Arch Dis Child 2010; 95:534.
  21. Matthews PM, Wylezinska M, Cadoux-Hudson T. Novel approaches to imaging brain tumors. Hematol Oncol Clin North Am 2001; 15:609.
  22. Houten JK, Babu RP, Miller DC. Thoracic paraganglioma presenting with spinal cord compression and metastases. J Spinal Disord Tech 2002; 15:319.
  23. Houten JK, Weiner HL. Pediatric intramedullary spinal cord tumors: special considerations. J Neurooncol 2000; 47:225.
  24. Merchant TE, Kiehna EN, Thompson SJ, et al. Pediatric low-grade and ependymal spinal cord tumors. Pediatr Neurosurg 2000; 32:30.
  25. Mehlman CT, Crawford AH, McMath JA. Pediatric vertebral and spinal cord tumors: a retrospective study of musculoskeletal aspects of presentation, treatment, and complications. Orthopedics 1999; 22:49.