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W HILE PEDIATRIC CENTRAL nervous system (CNS) tumors a re relatively rare — with about 3,000 new cases each year in the U.S. 1 — they present a unique challenge. The impli- cations of treatment must be weighed carefully as significant side effects can linger into adulthood. The good news is advancements in treatments are offering patient families more options. There is no known cause for most pediatric CNS tumors, which include a variety of types (medulloblastomas, astrocytomas and others), but the working theory revolves around an underlying genetic origin in many cases. Standard treatment often involves surgical resec- tion, although some tumors may be in locations that are not amenable to resection and only a biopsy is possible. Following surgery, chemotherapy, and in some cases radiation therapy, are advised. These options, however, carry potential side effects, which may impact speech, cognition and emotional development in this young patient population. Over the last 10 years, we have begun to better understand the molecular and genomic signatures of these tumors and are developing more targeted treatment approaches. In particular, by delving into how the cell regulates itself, we may be able to use different agents to target specific pathways to turn off certain cell functions, such as shutting down or blocking the pathways that allow cell growth or division. Perhaps, the biggest change occur- ring in neuro-oncology at present is risk stratification. By identifying patients at low, standard and high risk for recur- rence, it is possible to adjust treatments accordingly. If recurrence is less likely, the patient likely needs less therapy, reducing the chance of long-term impli- cations. For example, radiation near the pituitary gland can cause endocrine problems, while chemotherapy can cause complications relating to memory and higher executive functioning. When dealing with pediatric patients, it's also important to provide survivorship services in addition to care. During treatment, children miss school and social activities, and the journey back to normal life after treatment can be challenging. A broad spectrum of care that includes school liaisons and psy- chologists can aid the transition. These professionals can help school administra- tors understand the unique needs of children with pediatric CNS tumors. Beyond the school and social compo- nents, patient families should also be educated about the short- and long-term health issues survivors may experience, such as childhood obesity or heart dis- ease, which may result from inactivity related to care. Overall, the treatment advances taking place are encouraging for physicians and families. Collaboration between pediatric hospitals can also offer patients a broader range of treatment options close to home, making compliance easier for patient families. As we learn more about the nature of this disease, care approaches can continue to be adjusted so children can not only survive CNS tumors, but also go on to lead healthy lives. Sara h R u sh , M D, i s a p e di at r ic neuro-oncolog ist in Akron Children's Hospital's Showers Family Center for Childhood Cancer and Blood Disorders. She is Director of Akron Children's Neuro- Oncolog y and New Agent and Targeted Therapy prog rams. Akron Children's has joined with Cincinnati Children's Hospital Medical Center's Advanced Cancer Therapies Network to offer cancer patients easier access to Phase I and Phase 2 clinical trials. Clinical trials cur- rently available include those sponsored by the Children's Oncolog y Group (COG), as well as the Pediatric Brain Tumor Consortium, National Cancer Institute Pediat r ic Pha se 1 Con sor t ium, Ne w Approaches to Neuroblastoma Therapy Consortium, Collaborative Ependymoma Research Network, Neurof ibromatosis Clinical Trials Consortium, and other leading p ediat r ic clinical re search consortia. This network brings together clinical research centers to improve the treatment of pediatric cancer and other tumor disorders. 1. Mueller S., Chang S., "Pediatric bra in t u mors: c u r rent t reat ment st rateg ies a nd f uture t herapeutic approaches," Neurotherapeutics: The Journal of the American Society for Experimenta l NeuroTherapeutics, 2009 Jul; 6(3)570-86. ■ Innovations in Treating Pediatric CNS Tumors By Sarah Rush, MD Offer Promise for Young Patients ++++++++++++++++++++++++++++++++ + +++ + +++ ++++++++++++++++++++++++++++++++ SPECIAL SECTION: CANCER 2 4 | Cleveland/Akron/Canton MD NEWS ■ M D N E W S . CO M