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This group of tumors occurs in the tissues that cover the brain and spinal cord. They occur in the cerebrum, the part of the brain that controls thought, movement and sensation. Learn about dysembryoplastic neuroepithelial tumors in children and find information on how we support and care for children with DNT before, during, and after treatment.
The Brain Tumor Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center cares for children with many different types of common and rare brain and spinal tumors, including astrocytomas, medulloblastomas, ependymoma, glioblastomas, and primitive neuroectodermal tumors (PNET).
Your child will receive care from some of the world’s most experienced pediatric brain tumor doctors and internationally recognized pediatric subspecialists.
Our team works closely together to develop a care plan that offers your child the highest possible quality of life after treatment, and takes the needs of your child and your family into account.
Children treated at the Brain Tumor Center have access to some of the most advanced diagnostics and therapies, including:
Thanks to refined surgical techniques and improved chemotherapy and radiation therapy, the majority of children with brain and spinal cord tumors are now long-term survivors. However, they may face physical, social, and intellectual challenges that require specialized care.
Learn more about our Brain Tumor Center.
If your child has been experiencing seizures that don’t respond to medication, a possible cause may be a dysembryoplastic neuroepithelial tumor (DNT). This is a rare, benign type of tumor that occurs in the tissues that cover the brain and spinal cord. The outlook for a child with DNT is generally good. As you read further below, you will find general information about dysembryoplastic neuroepithelial tumors (DNT).
Your child will be seen through Dana-Farber/Boston Children's Cancer and Blood Disorders Center, an integrated pediatric oncology program through Dana-Farber Cancer Institute and Boston Children's Hospital that provides — in one specialized program — all the services of both a leading cancer center and a pediatric hospital.
Our pediatric neuro-oncology, neurosurgical and neurology specialists at Dana-Farber/Boston Children's offer:
Through the Stop and Shop Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, your child will be are able to meet with his entire care team at the same follow-up visit.
A DNT usually begins in children and teenagers who are 20 years old or younger.
While each child may experience symptoms differently, and symptoms may vary depending on the size and exact location of the tumor, the most common symptom for DNT is the presence of seizures that are difficult to control with anti-seizure medication. In fact, it’s not uncommon for children to have their first seizure before age 10 and then continue to experience them for several years before the diagnosis is made. DNT tumors are found in the cerebrum, which is the part of the brain that controls thought, movement and sensation, so your child may experience other symptoms that relate to those functions.
These tumors consist of different types of abnormal cells including:
If your child’s doctor suspects that he may have a dysembryoplastic neuroepithelial tumor, she may order diagnostic tests including:
At Dana-Farber/Boston Children's, we know how difficult a diagnosis of a brain tumor can be, both for your child and for your whole family. That’s why our physicians are focused on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs.
The most common treatment for DNT is to remove the tumor surgically. Because it is a benign tumor, and prognosis is good even if not the entire tumor is not removed, radiation and chemotherapy are not used.
The outlook for dysembryoplastic neuroepithelial tumors (DNT) is uniformly good, regardless of how much of the tumor is removed.
Treatment for brain tumors in children has progressed tremendously in the last decade:
Our program offers unique access to a range of clinical trials in which your child can receive the newest brain tumor treatments. Through this research, our physicians work to improve current therapeutic approaches and outcomes for many hard-to-treat pediatric brain tumors. Dana-Farber/Boston Children's oversees New England’s most active pediatric oncology clinical research program; it provides access to unique clinical trials for patients with newly diagnosed, relapsed or refractory brain tumors. We are:
Rapidly translating scientific discoveries to the bedside is a major focus of the program. Members of our brain tumor team:
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.
Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are also important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of proliferative activity are increasingly used in tumor diagnosis and classification.
Primary central nervous system tumors are a diverse group of diseases that together constitute
the most common solid tumor in childhood. The Central Brain Tumor Registry of the United States (CBTRUS) estimates that approximately 4,300 U.S. children are diagnosed each year.
Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.
Ostrom QT, Gittleman H, Farah P, et al.: CBTRUS statistical report: Primary brain and central nervous system tumors diagnosed in the United States in 2006-2010. Neuro Oncol 15 (Suppl 2): ii1-56, 2013.
The classification of childhood central nervous system (CNS) tumors is based on histology and location. Tumors are classically categorized as infratentorial, supratentorial, parasellar, or spinal.
Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of proliferative activity are increasingly used in tumor diagnosis and classification and will likely affect classification and nomenclature in the future.
Primary CNS spinal cord tumors comprise approximately 1% to 2% of all childhood CNS tumors. The classification of spinal cord tumors is based on histopathologic characteristics of the tumor and does not differ from that of primary brain tumors.
Infratentorial (posterior fossa) tumors include the following:
Supratentorial tumors include the following:
Parasellar tumors include the following:
Spinal cord tumors include the following:
Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.
Important concepts that should be understood by those treating and caring for a
child who has a brain tumor or spinal cord tumor include the following:
(Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information about possible long-term or late effects.)
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.
Presently, there is no uniformly accepted staging system for most childhood brain tumors. These tumors are classified and treated based on their histology and location within the brain (see Table below). However, with advances in molecular data, it is conceivable that genomic factors will refine classification approaches for certain groups of tumors, such as medulloblastomas  and low-grade gliomas.
Related PDQ Treatment Summary
Astrocytomas and Other Tumors of Glial Origin
– Low-Grade Astrocytomas
Diffuse fibrillary astrocytoma
Childhood Astrocytomas Treatment
Subependymal giant cell astrocytoma
– High-Grade Astrocytomas
Giant cell glioblastoma
Brain Stem Glioma
Diffuse intrinsic pontine glioma
Childhood Brain Stem Glioma Treatment
Focal or low-grade brain stem glioma
CNS Embryonal Tumors
Childhood CNS Embryonal Tumors Treatment
Medulloblastoma with extensive nodularity
– CNS Primitive Neuroectodermal Tumors (PNETs)
– Pineal Parenchymal Tumors
– CNS Atypical Teratoid/Rhabdoid Tumor
Childhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment
CNS Germ Cell Tumors
Childhood CNS Germ Cell Tumors Treatment
Teratoma with malignant transformation
– Non-Germinomatous Germ Cell Tumors
Mixed germ cell tumor
Yolk sac tumor
Childhood Craniopharyngioma Treatment
Subependymoma (WHO Grade I)
Childhood Ependymoma Treatment
Myxopapillary ependymoma (WHO Grade I)
Ependymoma (WHO Grade II)
Anaplastic ependymoma (WHO Grade III)
Tumors of the Choroid Plexus
CNS = central nervous system; WHO = World Health Organization.
Recurrence is not uncommon in both low-grade and malignant childhood brain tumors and may occur many years after initial treatment. Disease may occur at the primary tumor site or, especially in malignant tumors, at noncontiguous central nervous system (CNS) sites. Systemic relapse is rare but may occur. At time of recurrence, a complete evaluation for extent of relapse is indicated for all malignant tumors and, at times, for lower-grade lesions. Biopsy or surgical re-resection may be necessary for confirmation of relapse; other entities, such as secondary tumor and treatment-related brain necrosis, may be clinically indistinguishable from tumor recurrence. The determination of the need for surgical intervention must be individualized based on the initial tumor type, the length of time between initial treatment and the reappearance of the lesion, and the clinical picture.
Early-phase therapeutic trials may be available for selected patients via Children's Oncology Group phase I institutions, the Pediatric Brain Tumor Consortium, or other entities.
Northcott PA, Shih DJ, Peacock J, et al.: Subgroup-specific structural variation across 1,000 medulloblastoma genomes. Nature 488 (7409): 49-56, 2012.
Kool M, Korshunov A, Remke M, et al.: Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol 123 (4): 473-84, 2012.
Jones DT, Kocialkowski S, Liu L, et al.: Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas. Cancer Res 68 (21): 8673-7, 2008.
Pfister S, Janzarik WG, Remke M, et al.: BRAF gene duplication constitutes a mechanism of MAPK pathway activation in low-grade astrocytomas. J Clin Invest 118 (5): 1739-49, 2008.
There is no uniformly accepted staging system for childhood primary spinal cord tumors. These tumors are classified and treated based on their location within the spinal cord and histology. Refer to the following PDQ summaries for more information on the staging and treatment of newly diagnosed and recurrent childhood spinal cord tumors:
This information is provided by the National Cancer Institute.
This information was last updated on August 12, 2014.
Many children with cancer receive treatment in the outpatient setting, which allows them to stay in school and continue to develop intellectually and socially. However, returning to school can be an emotional experience; our Back to School Program is designed to ease your child's transition back to the classroom.
Concierge Services is your one-stop place to learn about Dana-Farber programs, services and resources, as well as information on getting around Boston, finding lodging or restaurants, and activities in the area.
The Expressive Arts Therapy program, sponsored by the Leonard P. Zakim Center for Integrative Therapies, provides adult patients, family members, and caregivers with a variety of options to support well-being during cancer treatment. From live music meditation to painting technique workshops, the program offers a range of creative outlets to suit every interest.
Dana-Farber and Children's Hospital, including parking facilities, are fully accessible to people with disabilities. There are wheelchairs at the main entrance, and security staff can provide personal assistance. We also have many educational materials available in large print and audiotape formats.
The Ethics Consultation Service is available for patients and families who may be facing difficult decisions and choices regarding care. Our goal is to bring together patients, families and health care providers to talk about ethical concerns and help everyone involved arrive at a resolution that is right for all.
Find practical tips and suggestions for individuals caring for a family member or friend with cancer, including creating a caregiving plan, finding community resources, and looking after your own well-being.
Friends' Place provides personal consultations to help cancer patients of all ages cope with changes in physical appearance that result from cancer treatment. Our experienced, compassionate team provides fittings for compression garments or breast prostheses, helps with wigs and other head coverings, and offers make-up and skincare advice.
The Friends' Corner Gift Shop, located on the first floor of the Yawkey Center for Cancer Care, offers a wide selection of unique gifts and everyday items for patients, families and staff.
Every year, thousands of patients with cancer from around the world come to Dana-Farber for their care. We provide a wide array of logistical and other services for individuals who live outside the United States.
Dana-Farber provides interpreting services for patients whose first language is not English. Interpreters may be requested for any activity, including registration, booking appointments, attending treatments and exams, support groups, and meetings with doctors and other members of your health care team.
Just for Teens provides programs and activities for teens and young adults with cancer at the Jimmy Fund Clinic and Children's Hospital Boston. We offer activities and events both inside and out of the hospital so that you have creative ways to pass the time and can meet other teens who are going through similar experiences.
Our nutritionists are registered dietitians who can assist you in planning an optimal diet during any stage of your cancer journey, cope with any side effects you may experience, and answer your questions about the latest findings on cancer and nutrition.
The Eleanor and Maxwell Blum Patient and Family Resource Center and its satellite resource rooms are staffed by health care professionals and provide computer stations, books, brochures, videos, and CDs to help you find information and support on a variety of issues about cancer treatment and care.
Patients websites help friends and family members stay up-to-date on their loved ones' condition and write messages of support and encouragement.
The Dana-Farber pharmacy fills prescriptions for all pediatric and adult patients. Our pharmacists are an extension of the patient care team and work closely with your physicians to provide seamless, convenient, safe care.
More than 1,200 Dana-Farber patients and their families have enjoyed free trips to baseball games, theater shows, museums, and other attractions this year through the Recreational Resources program.
The School Liaison Program is for pediatric patients who are diagnosed with or have completed a treatment that involves the central nervous system. We provide consultation about the cognitive late effects of treatment to help parents understand and advocate for their child's learning needs.
Through all stages of cancer treatment and survivorship, our Spiritual Care staff is available 24 hours a day to provide emotional and spiritual support for adults and pediatric patients and family members.
Integrative therapies, also known as complementary therapies, range from acupuncture and massage to nutritional guidance and music therapy. Patients treated at the Zakim Center credit its services with easing nausea, improving circulation, and reducing pain, stress, and anxiety associated with cancer treatment.
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