Selection of an appropriate therapy can only occur if the correct diagnosis is made and the stage of the disease is accurately determined.
Children with primary brain or spinal cord tumors represent a major therapy challenge that, for optimal results, requires the coordinated efforts of pediatric specialists in fields such as neurosurgery, neuropathology, radiation oncology, pediatric oncology, neuro-oncology, neurology, rehabilitation, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.[2,3] For example, radiation therapy of pediatric brain tumors is technically demanding and should be performed in centers that have experience in this area.
For most childhood brain and spinal cord tumors, the optimal treatment regimen has not been determined. Children who have brain and spinal cord tumors should be considered for enrollment in a clinical trial when an appropriate study is available. Such clinical trials are being carried out by institutions and cooperative groups. Survival of childhood cancer has advanced as a result of clinical trials that have attempted to improve upon the best accepted therapy available. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and then comparing the results with those previously obtained from existing therapy. Information about ongoing clinical trials is available from the NCI Web site.
While more than 70% of children diagnosed with brain tumors will survive for more than 5 years after diagnosis, survival rates are wide-ranging depending on tumor type and stage. Long-term sequelae related to the initial presence of the tumor and subsequent treatment are common.[4,5,6] Debilitating effects on growth and neurologic development have frequently been observed after radiation therapy, especially in younger children. Secondary tumors have increasingly been diagnosed in long-term survivors. For this reason, the role of chemotherapy in allowing a delay or reduction in the administration of radiation therapy is under study, and preliminary results suggest that chemotherapy can be used to delay, limit, and sometimes obviate, the need for radiation therapy in children with benign and malignant lesions.[8,9,10] Long-term management of these patients is complex and requires a multidisciplinary approach.
(Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information about possible long-term or late effects.)
Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics.
Fisher JL, Schwartzbaum JA, Wrensch M, et al.: Epidemiology of brain tumors. Neurol Clin 25 (4): 867-90, vii, 2007.
Blaney SM, Haas-Kogan D, Young Poussaint T, et al.: Gliomas, ependymomas, and other nonembryonal tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia, Pa: Lippincott Williams and Wilkins, 2011, pp 717-771.
Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.
Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010.
Reimers TS, Mortensen EL, Nysom K, et al.: Health-related quality of life in long-term survivors of childhood brain tumors. Pediatr Blood Cancer 53 (6): 1086-91, 2009.
Iuvone L, Peruzzi L, Colosimo C, et al.: Pretreatment neuropsychological deficits in children with brain tumors. Neuro Oncol 13 (5): 517-24, 2011.
Armstrong GT: Long-term survivors of childhood central nervous system malignancies: the experience of the Childhood Cancer Survivor Study. Eur J Paediatr Neurol 14 (4): 298-303, 2010.
Duffner PK, Horowitz ME, Krischer JP, et al.: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328 (24): 1725-31, 1993.
Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11 (5): 850-6, 1993.
Mason WP, Grovas A, Halpern S, et al.: Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16 (1): 210-21, 1998.
Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.
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