Clinical manifestations and diagnosis of central nervous system tumors in children
- Ching Lau, MD, PhD
Ching Lau, MD, PhD
- Chief, Hematology Oncology at Connecticut Children's Medical Center
- Professor at Jackson Laboratory for Genomic Medicine
- Head of Division of Pediatric Hematology-Oncology, University of Connecticut School of Medicine
- Wan-Yee Teo, MBBS, FAAP, MRCPCH (UK), PhD
Wan-Yee Teo, MBBS, FAAP, MRCPCH (UK), PhD
- Assistant Professor
- Duke-NUS Graduate Medical School
- Adjunct Assistant Professor, Department of Pediatrics, Section of Hematology-Oncology
- Baylor College of Medicine
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 . They are the leading cause of death from childhood cancer, surpassing the mortality rate of acute lymphoblastic leukemia .
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 manifestations and initial surgical approach to patients with high-grade gliomas")
●Medulloblastoma (see "Histopathology and molecular pathogenesis of medulloblastoma")
- Linabery AM, Ross JA. Trends in childhood cancer incidence in the U.S. (1992-2004). Cancer 2008; 112:416.
- 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).
- Wilne SH, Dineen RA, Dommett RM, et al. Identifying brain tumours in children and young adults. BMJ 2013; 347:f5844.
- Wilne S, Collier J, Kennedy C, et al. Presentation of childhood CNS tumours: a systematic review and meta-analysis. Lancet Oncol 2007; 8:685.
- 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.
- 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.
- Teo WY, Myseros JS. The gut or the brain?--Gastrointestinal misdiagnoses of infantile brain tumors. Childs Nerv Syst 2014; 30:1449.
- 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.
- Pollack IF. Pediatric brain tumors. Semin Surg Oncol 1999; 16:73.
- 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.
- 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.
- Pillai MG, Unnikrishnan AG, Nair V, et al. Diencephalic cachexia: a rare cause for failure to thrive. J Pediatr 2005; 147:713.
- 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.
- Ullrich NJ. Neurocutaneous Syndromes and Brain Tumors. J Child Neurol 2016; 31:1399.
- 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.
- Isaacs H Jr. I. Perinatal brain tumors: a review of 250 cases. Pediatr Neurol 2002; 27:249.
- Severino M, Schwartz ES, Thurnher MM, et al. Congenital tumors of the central nervous system. Neuroradiology 2010; 52:531.
- 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.
- Wilson PE, Oleszek JL, Clayton GH. Pediatric spinal cord tumors and masses. J Spinal Cord Med 2007; 30 Suppl 1:S15.
- 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.
- Matthews PM, Wylezinska M, Cadoux-Hudson T. Novel approaches to imaging brain tumors. Hematol Oncol Clin North Am 2001; 15:609.
- Houten JK, Babu RP, Miller DC. Thoracic paraganglioma presenting with spinal cord compression and metastases. J Spinal Disord Tech 2002; 15:319.
- Houten JK, Weiner HL. Pediatric intramedullary spinal cord tumors: special considerations. J Neurooncol 2000; 47:225.
- Merchant TE, Kiehna EN, Thompson SJ, et al. Pediatric low-grade and ependymal spinal cord tumors. Pediatr Neurosurg 2000; 32:30.
- 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.
- CLINICAL MANIFESTATIONS
- Age of the patient
- Tumor location
- Common presenting signs and symptoms
- - Headache
- - Nausea and vomiting
- - Ataxia and gait abnormalities
- - Cranial nerve palsies
- - Impaired vision
- - Seizures
- - Papilledema
- - Macrocephaly
- - Developmental delay and behavioral changes
- - Endocrinopathies
- Neurocutaneous syndromes
- Congenital CNS tumors
- Spinal tumors
- - Indications
- - Choice of modality
- MRI imaging
- Positron emission tomography scans
- DIFFERENTIAL DIAGNOSIS
- Diagnostic pitfalls
- SUMMARY AND RECOMMENDATIONS