Choroid Plexus Tumor
Overview
Having a tumor in the brain is always a very serious matter, and a choroid plexus tumor is no exception. Choroid plexus tumors arise in the choroid plexus, tissue located in the spaces of the brain called ventricles.
While all brain tumors are life-threatening, most children and adolescents who have been diagnosed with one survive into adulthood. Many of them face physical, psychological, social and intellectual challenges related to their treatment, and require ongoing care to help with school and with skills they will use throughout adulthood.
As you read on, you’ll find detailed information about choroid plexus tumors.
- The choroid plexus makes cerebrospinal fluid (CSF), the liquid that surrounds the brain and spinal cord.
- Choroid plexus tumors are rare, representing only 3 percent of brain tumors in children
- They’re seen more often in younger children – between 10 and 20 percent of brain tumors that occur within the first year of life are choroid plexus tumors
- Girls and boys are equally affected
- The vast majority of choroid plexus tumors are either choroid plexus papillomas (CPP) or choroid plexus carcinomas (CPC). CPP are often easier to treat.
How Dana-Farber/Boston Children's Cancer and Blood Disorders Center approaches choroid plexus tumors
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.
After treatment, your child will receive expert follow-up care through the Stop and Shop Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, where he will be are able to meet with his neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologist at the same follow-up visit.
- Our pediatric brain tumor survivorship clinic is held weekly.
- In addition to meeting with your pediatric neuro-oncologists, neurologist and neurosurgeon, your child may also see one of our endocrinologists or alternative/complementary therapy specialists.
- School liaisons and psychosocial personnel from the pediatric brain tumor team are also available.
- If your child needs rehabilitation, he may also meet with speech, physical and occupational therapists during and after treatments.
In-depth
We understand that you may have a lot of questions when your child is diagnosed with a choroid plexus tumor:
- What exactly is it?
- What are potential complications in my child’s case?
- What are the treatments?
- What are possible side effects from treatment?
- How will it affect my child long term?
We’ve tried to provide some answers to those questions here, and when you meet with our experts, we can explain your child’s condition and treatment options fully.
How are choroid plexus tumors classified?
An important part of diagnosing a brain tumor involves staging and classifying the disease, which will help your child’s doctor determine treatment options and prognosis. Staging is the process of determining whether the tumor has spread and if so, how far.
There are three main types of choroid plexus tumors: papillomas, atypical papillomas and carcinomas:
Choroid plexus papillomas (CPP) and atypical papillomas (APP)
- grow more slowly ("benign")
- are by far more common, accounting for 80 to 90 percent of choroid plexus tumors
- only rarely spread to other parts of the brain and spinal cord
Choroid plexus carcinomas (CPC)
- grow more aggressively (“malignant”)
- are more likely to spread (metastasize)
- are less common, accounting for 10 to 20 percent of all choroid plexus tumors.
In rare cases, a choroid plexus tumor may appear to have characteristics of both CPP and CPC.
Can a choroid plexus papilloma turn into a choroid plexus carcinoma?
Generally speaking, these are two different kinds of tumors, and usually a CPP stays a CPP. Tumors have a wide spectrum in terms of how harmful they are, and sometimes a CPP may develop some unusual features, but usually not enough to fulfill the criteria for a CPC. APP may occasionally behave like a CPP.
What causes choroid plexus tumors?
It’s important to understand that these tumors most often have no known cause. There’s nothing that you could have done or avoided doing that would have prevented them from developing. There is one rare genetic disease called Li-Fraumeni Syndrome that can be associated with CPP. If your child has a CPP, his doctors will talk to you about genetic testing for this disease.
What are the symptoms of a choroid plexus tumor?
Symptoms vary depending on size and location of tumor. A choroid plexus tumor can block the normal flow of cerebrospinal fluid, causing increased pressure on the brain (hydrocephalus) and enlargement of the skull. It can also cause symptoms including:
- headaches (generally upon awakening in the morning)\
- nausea and vomiting (often worse in the morning and improving throughout the day)
- lethargy and irritability
- problems feeding or walking
- enlarged fontanels (the soft "spot" that occurs before the bones in the head become solid) in infants
Keep in mind that these symptoms may resemble other, more common conditions or medical problems. If you don’t have a diagnosis and are concerned, always consult your child's physician.
Questions to ask your child’s doctor
After your child is diagnosed with a brain tumor, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.
Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.
If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too.
Some of the questions you may want to ask include:
- Has my child’s brain tumor spread?
- Can the tumor be treated with surgery?
- How long will my child need to be in the hospital?
- What are the possible short and long-term complications of treatment? How will they be addressed?
- What is the likelihood of cure?
- What services are available to help my child and my family cope?
FAQ
Q: Will my child be OK?
A: A child’s long-term health after treatment for a choroid plexus tumor varies significantly depending on the whether the tumor has spread and whether it can be completely removed through surgery. Choroid plexus papillomas are generally more likely to be successfully treated than choroid plexus carcinomas. Your doctor will discuss treatment options with you and your family including clinical trials and supportive care.
Q: What is the expected outcome (prognosis) for children after treatment for choroid plexus tumors?
A: Choroid plexus papillomas and atypical choroid plexus papillomas are usually treated by surgical removal of the tumor. Choroid plexus carcinomas are often harder to treat. It’s important to remove the tumor, but treatment also requires additional therapies such as chemotherapy and/or radiation.
Q: What services are available to help my child and my family cope?
A: We offer a variety of services to help you, your child and your family get through this difficult time.
Q: Where will my child be treated?
A: Children treated on an outpatient basis through Dana-Farber/Boston Children's are cared for at the Jimmy Fund Clinic on the third floor of Dana-Farber Cancer Institute. If your child needs to be admitted to the hospital, she will stay at Boston Children's Hospital on the ninth floor of the Berthiaume Building.
Q: What kind of supportive or palliative care is available for my child?
A: When appropriate, our Pediatric Advanced Care Team (PACT) offers supportive treatments intended to optimize the quality of life and promote healing and comfort for children with life-threatening illness. PACT can also provide psychosocial support and help arrange end-of-life care when necessary.
Tests
The first step in treating your child is forming an accurate and complete diagnosis, so your child’s physician may order a number of different tests to best diagnose the tumor. In addition to a physical exam, a medical history and neurological exam (which tests reflexes, muscle strength, eye and mouth movement, coordination and alertness), your child’s doctor may request tests including:
- computerized tomography scan (also called a CT or CAT scan) — a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images of the brain. CT scans are more detailed than general x-rays. If a choroid plexus tumor is suspected, your child may have a CT scan of the brain
- magnetic resonance imaging (MRI) — a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of the brain and spine.
- biopsy — in many cases, a tissue sample from the tumor will be taken through a needle during a simple surgical procedure to confirm the diagnosis
- lumbar puncture (spinal tap) — to remove a small sample of cerebrospinal fluid (CSF), a special needle is placed into the lower back, into the spinal canal, the area around the spinal cord. CSF is the fluid that bathes the brain and spinal cord. The sample is sent for testing to determine if any tumor cells have started to spread. For children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.
After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.
Treatment and care
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. We’ll work with you to create a care plan that’s best for your child.
Your child’s physician will determine a specific course of treatment based on several factors, including:
- your child's age, overall health and medical history
- type, location, and size of the tumor
- extent of the disease
- your child's tolerance for specific medications, procedures or therapies
- how your child's doctors expects the disease to progress
There are a number of treatments we may recommend. Some of them help to treat the tumor while others are intended to address complications of the disease or side effects of the treatment. These treatments include:
Surgery
Between 85 and 100 percent of children with choroid plexus papilloma (CPP) are treated successfully with complete removal of the tumor. For choroid plexus tumors, chemotherapy before surgery may help shrink the tumor, making it easier to remove. For patients with choroid plexus carcinoma (CPC), it’s important to remove as much of the tumor as possible, but additional treatment, usually radiation and chemotherapy, is needed.
Chemotherapy
Chemotherapy are drug treatments that work by interfering with the cancer cell's ability to grow or reproduce. Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
How is chemotherapy given?
Your child may receive chemotherapy:
- orally, as a pill to swallow
- intramuscularly, as an injection into the muscle or fat tissue
- intravenously, directly to the bloodstream (intravenously, or “IV”)
- intrathecally, directly into the spinal fluid with a needle (intrathecally)
Does chemotherapy cause side effects?
While chemotherapy can be quite effective in treating certain cancers, the drugs do not differentiate normal healthy cells from cancer cells. Because of this, your child may experience adverse side effects during treatment. Being able to anticipate these side effects can help you, your child and your care team prepare for, and, in some cases, prevent these symptoms from occurring.
What are common side effects and how are side effects managed?
Common side effects to chemotherapy given for treatment of choroid plexus carcinoma include fatigue, headache, diarrhea and constipation, weakened immune system and need for transfusions. These side effects can usually be effectively managed with standard medical approaches.
Our Pediatric Brain Tumor Program also has access to specialists who deliver complementary or alternative medicines. These treatments, which may help control pain and side effects of therapy include the following.
- acupuncture/acupressure
- therapeutic touch
- massage
- herbs
- dietary recommendations
Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.
Radiation
Our doctors may also use precisely targeted and dosed radiation to kill cancer cells left behind after your child’s surgery. This treatment is important to control the local growth of tumor.
We usually don’t use radiation therapy unless your child’s tumor has regrown after chemotherapy. Due to the potential side effects of radiation, including effects on learning and hormone function, it is best avoided if your child is young (especially under age 10).
What is the long-term outlook for a child with a choroid plexus tumor?
Today, the majority of children and adolescents diagnosed with pediatric brain tumors will survive into adulthood. However, many of them will face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.
What about progressive or recurrent disease?
There are many standard and experimental treatment options for children with progressive or recurrent choroid plexus tumors.
Dana-Farber Cancer Institute is one of nine institutes in the nation belonging to the Pediatric Oncology Experimental Therapeutic Investigators Consortium. The consortium is dedicated to the development of new and innovative treatments for children with newly diagnosed as well as progressive or recurrent choroid plexus tumors and other brain tumors. We are also home to the world’s largest pediatric low-grade astrocytoma research program and the Department of Defense Neurofibromatosis Clinical Trial Consortium.
Long-term follow-up
To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children's established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic.
Today, more than 1,000 pediatric brain tumor survivors of all ages are followed by the Outcomes Clinic, a multi-disciplinary program designed to address long-term health and social issues for families and survivors of childhood brain tumors. Some of the post-treatment services provided by the Outcomes Clinic include:
- MRI scans to monitor for tumor recurrences
- intellectual function evaluation
- endocrine evaluation and treatment
- neurologic assessment
- psychosocial care
- hearing, vision monitoring
- ovarian dysfunction evaluation and treatment
- motor function evaluation and physical therapy
- complementary medicine
As a result of treatment, children may experience changes in intellectual and motor function. Among several programs addressing these needs are the School Liaison and Back to School Programs, which provide individualized services to ease children's return to school and maximize their ability to learn.
In addition to providing thorough and compassionate care, our Outcomes Clinic specialists conduct innovative survivorship research and provide continuing education for staff, patients and families.
Research and Innovations
Dana-Farber/Boston Children's is a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed (returning) or refractory (resistant to treatment) disease. It is also the New England Phase I Center of the Children's Oncology Group and a member of the Department of Defense Neurofibromatosis Clinical Trial Consortium.
We are home to the only dedicated pediatric low-grade glioma program, the Pediatric Low-Grade Astrocytoma (PLGA) Research Program
Purpose of This PDQ Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of select childhood brain and spinal cord tumors. It also provides links to the other PDQ childhood brain and spinal cord tumor summaries. This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board.
Information about the following is included in this summary:
- Classification of childhood brain and spinal cord tumors.
- General approach to care for childhood brain tumor patients.
- Stage information about select childhood brain and spinal cord tumors, and links to the other PDQ childhood brain and spinal cord tumor summaries.
- Treatment options about select childhood brain and spinal cord tumors, and links to the other PDQ childhood brain and spinal cord tumor summaries.
This summary is intended as a resource to inform and assist clinicians and other health professionals who care for pediatric cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
In this summary, treatments are described as “standard” or “conventional” and “under clinical evaluation.” These designations should not be used as a basis for reimbursement determinations.
This summary is also available in a patient version, which is written in less technical language, and in Spanish.
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General Information
Note: Separate PDQ summaries on Childhood Astrocytomas Treatment, Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment, Childhood Brain Stem Glioma Treatment, Childhood Central Nervous System Embryonal Tumors Treatment, Childhood Ependymoma Treatment, and Childhood Craniopharyngioma Treatment are also available.
The National Cancer Institute (NCI) provides the PDQ pediatric cancer information treatment summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public.
Information about ongoing clinical trials is available from the NCI Web site.
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Classification of Brain Tumors
Primary brain tumors are a diverse group of diseases that together constitute
the most common solid tumor in childhood. Between 2,500 and 3,500 children are diagnosed in the United States each year. Immunohistochemical analysis,
cytogenetic and molecular genetic findings, and measures of mitotic activity
are increasingly used in tumor diagnosis and classification, and will likely alter classification and nomenclature in the future.
The classification of childhood brain tumors is based on histology and location.[1] Tumors are classically categorized as infratentorial, supratentorial, sellar, or suprasellar.
Common
infratentorial (posterior fossa) tumors include:
- Cerebellar astrocytomas (usually pilocytic but also fibrillary and, less frequently,
high-grade).
- Medulloblastomas (primitive neuroectodermal tumors [PNETs]).
- Ependymomas (cellular, papillary, clear cell, tanycytic, or anaplastic).
- Brain stem gliomas are typically diffuse intrinsic pontine gliomas or diffuse intrinsic high-grade tumors that are diagnosed neuroradiographically without biopsy. Focal, tectal, and exophytic cervicomedullary tumors are generally low-grade tumors.
- Atypical teratoid/rhabdoid tumors.
Supratentorial tumors include:
- Low-grade cerebral hemispheric astrocytomas (grade 1 [pilocytic] or grade 2).
- High-grade or malignant astrocytomas (anaplastic astrocytomas, glioblastomas
multiforme [grade 3 or grade 4]).
- Mixed gliomas (low-grade or high-grade).
- Oligodendrogliomas (low-grade or high-grade).
- PNETs (including cerebral neuroblastomas, pineoblastomas, and ependymoblastomas).
- Atypical teratoid/rhabdoid tumors.
- Ependymomas (cellular or anaplastic).
- Meningiomas.
- Choroid plexus tumors (papillomas and carcinomas).
- Pineal parenchymal tumors (pineocytomas, mixed pineal
parenchymal tumors, and pineal parenchymal tumors of intermediate differentiation).
- Neuronal and mixed neuronal glial tumors (gangliogliomas, desmoplastic
infantile gangliogliomas, and dysembryoplastic neuroepithelial tumors).
- Metastasis (rare) from extraneural malignancies.
Sellar or suprasellar tumors include:
- Craniopharyngiomas.
- Diencephalic astrocytomas (central tumors involving the chiasm, hypothalamus, and/or thalamus) that are generally low-grade
(including astrocytomas, grade 1 [pilocytic] or grade 2).
- Germ cell tumors (germinomas or nongerminomatous).
References:
Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000.
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Classification of Spinal Cord Tumors
Primary central nervous system spinal cord tumors comprise approximately
1% to 2% of all childhood nervous system tumors.[1][2][3] As is the case for
primary brain tumors, such lesions are histologically heterogeneous.
Approximately 70% of all intramedullary spinal cord tumors will be low-grade
astrocytomas and/or gangliogliomas. Other tumor types that occur include
ependymomas (refer to the PDQ summary on Childhood Ependymoma Treatment for more information), higher-grade glial tumors, and (rarely) primitive neuroectodermal
tumors (refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information). Myxopapillary ependymomas have a tendency to develop in the conus
and cauda equina regions. Symptoms and signs of spinal cord tumors are highly
dependent on the location of the tumor and its extent; some low-grade spinal
cord tumors are associated with large cysts that extend rostrally and caudally. At times it is impossible to
distinguish a tumor that arises in the medulla from a tumor that arises in
the upper cervical cord.
The classification of spinal cord tumors is based on histopathologic
characteristics of the tumor and does not differ from that of primary brain
tumors.[1][2][3]
References:
Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.
Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.
Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.
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General Approach to Care for Children with Brain and Spinal Cord Tumors
Important concepts that should be understood by those treating and caring for a
child who has a brain or spinal cord tumor include the following:
- The cause of most childhood brain tumors remains unknown.[1][2][3]
- 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.[4][5][6] For example, radiation therapy of pediatric brain tumors is very technically demanding and should be carried out 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.
Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best accepted therapy available. Information about ongoing clinical trials is available from the NCI
Web site.
- While more than 50% of children diagnosed with brain tumors will survive more than 5
years from 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.[7][8][9] For more information about possible long-term or late effects, refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer.
- 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.[10]
References:
Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiol Biomarkers Prev 2 (3): 277-88, 1993 May-Jun.
Kuijten RR, Strom SS, Rorke LB, et al.: Family history of cancer and seizures in young children with brain tumors: a report from the Childrens Cancer Group (United States and Canada). Cancer Causes Control 4 (5): 455-64, 1993.
Fisher JL, Schwartzbaum JA, Wrensch M, et al.: Epidemiology of brain tumors. Neurol Clin 25 (4): 867-90, vii, 2007.
Strother DR, Poplack IF, Fisher PG, et al.: Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins, 2002, pp 751-824.
Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.
Cohen ME, Duffner PK, eds.: Brain Tumors in Children: Principles of Diagnosis and Treatment. 2nd ed. New York: Raven Press, 1994.
Ris MD, Packer R, Goldwein J, et al.: Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children's Cancer Group study. J Clin Oncol 19 (15): 3470-6, 2001.
Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.
Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.
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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Stage Information
Childhood Astrocytoma
Childhood astrocytomas are classified as low-grade or high-grade.
Childhood Low-Grade Astrocytomas:
- Pilocytic astrocytoma.
- Diffuse fibrillary astrocytoma.
Childhood High-Grade Astrocytomas:
- Anaplastic astrocytoma.
- Glioblastoma multiforme.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood Brain Stem Glioma
Childhood brain stem gliomas include:
- Diffuse intrinsic pontine gliomas.
- Focal or low-grade brain stem gliomas.
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.
Childhood CNS Embryonal Tumors
Childhood CNS embryonal tumors include:
- CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
- Ependymoblastomas.
- Medulloblastomas.
- Medulloepitheliomas.
- Pineal parenchymal tumors of intermediate differentiation.
- Pineoblastomas.
- Supratentorial primitive neuroectodermal tumors.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood CNS Germ Cell Tumors
Childhood CNS germ cell tumors include:
- Germinomas.
- Embryonal yolk sac tumors.
- Choriocarcinomas.
- Immature teratomas.
- Mature teratomas.
- Teratomas with malignant transformation.
- Mixed germ cell tumors.
- Nongerminomatous germ cell tumors.
Germ cell brain tumors usually arise in the pineal or suprasellar regions.
Histologic subtypes include teratomas (both mature and immature), germinomas,
choriocarcinomas, and nongerminomatous germ cell tumors (i.e., embryonal cell
carcinoma, yolk cell or endodermal sinus tumors, and mixed germ cell tumors). These tumors have a
propensity for subarachnoid spread. Every patient with a germinoma or
malignant germ cell tumor should be evaluated with diagnostic imaging of the
spinal cord and whole brain. The best method for evaluating spinal cord
subarachnoid metastasis is magnetic resonance imaging with gadolinium enhancement. Cerebrospinal
fluid CSF) should be examined cytologically and levels of alpha-fetoprotein (AFP)
and human chorionic gonadotropin (HCG) determined. AFP and/or HCG may be
elevated in the serum of such patients. Prognosis is related to histology;
patients with pure germinoma have a more favorable outcome than those with
nongerminomatous germ cell tumors (nongerminomas).[1][2]
Childhood Craniopharyngioma
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Childhood Ependymoma
Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.
Childhood Ependymoblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Malignant Glioma
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Medulloblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more
information.
Childhood Medulloepithelioma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Pineal Parenchymal Tumors
Childhood pineal parenchymal tumors include:
- Pineoblastomas.
- Pineocytomas.
- Pineal parenchymal tumors of intermediate differentiation.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Spinal Cord Tumors
There is no uniformly accepted staging system for childhood primary spinal cord
tumors. These tumors are classified based on their location within the spinal
cord and histology. Low-grade spinal cord tumors rarely disseminate elsewhere
in the nervous system; however, higher grade tumors may disseminate.[3][4]
Despite this, because of the location of the tumor and concerns over causing
further neurologic deterioration by CSF attainment, routine
lumbar spinal punctures are not indicated in the evaluation of a child with a
spinal cord tumor. For high-grade glial spinal cord tumors, and possibly lower
grade tumors and ependymomas, (refer to the PDQ summary on Childhood Ependymoma Treatment for more information) neuroimaging of the entire neuroaxis (brain and
entire spine) is indicated at the time of diagnosis for determination of extent
of disease.
Childhood Supratentorial Primitive Neuroectodermal Tumors
Childhood supratentorial primitive neuroectodermal tumors include:
- Primitive neuroectodermal tumors.
- Cerebral neuroblastomas.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
References:
Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.
Balmaceda C, Modak S, Finlay J: Central nervous system germ cell tumors. Semin Oncol 25 (2): 243-50, 1998.
Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.
Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.
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Treatment Option Overview
Many of the improvements in survival in childhood cancer have been made as a
result of clinical trials that have attempted to improve on 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 comparing the results with those previously obtained
with existing therapy.
Because of the relative rarity of cancer in children, all patients with brain
and spinal cord tumors should be considered for entry into a clinical trial. To determine and
implement optimum treatment, treatment planning by a multidisciplinary team of
cancer specialists who have experience treating childhood brain tumors is
required. Radiation therapy of pediatric brain tumors is very technically demanding and should be carried out in centers that have experience in this
area to ensure optimal results.
Debilitating effects on growth and neurologic development have frequently been
observed following radiation therapy, especially in younger children.[1][2][3]
Secondary tumors have increasingly been diagnosed in long-term survivors.[4]
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.[5][6][7]
Long-term management of these patients is complex and requires a
multidisciplinary approach.
References:
Ris MD, Packer R, Goldwein J, et al.: Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children's Cancer Group study. J Clin Oncol 19 (15): 3470-6, 2001.
Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.
Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.
Jenkin D: Long-term survival of children with brain tumors. Oncology (Huntingt) 10 (5): 715-9; discussion 720, 722, 728, 1996.
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.
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Treatment of Newly Diagnosed Childhood Brain and Spinal Cord Tumors
Childhood Astrocytoma
Childhood astrocytomas are classified as low-grade or high-grade.
Childhood Low-Grade Astrocytomas:
- Pilocytic astrocytoma.
- Diffuse fibrillary astrocytoma.
Childhood High-Grade Astrocytomas:
- Anaplastic astrocytoma.
- Glioblastoma multiforme.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood Brain Stem Glioma
Childhood brain stem gliomas include:
- Diffuse intrinsic pontine gliomas.
- Focal or low-grade brain stem gliomas.
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more
information
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood brain stem glioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood CNS Embryonal Tumors
Childhood CNS embryonal tumors include:
- CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
- Ependymoblastomas.
- Medulloblastomas.
- Medulloepitheliomas.
- Pineal parenchymal tumors of intermediate differentiation.
- Pineoblastomas.
- Supratentorial primitive neuroectodermal tumors.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood embryonal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood CNS Germ Cell Tumors
Childhood CNS germ cell tumors include:
- Germinomas.
- Embryonal yolk sac tumors.
- Choriocarcinomas.
- Immature teratomas.
- Mature teratomas.
- Teratomas with malignant transformation.
- Mixed germ cell tumors.
- Nongerminomatous germ cell tumors.
Surgery, other than biopsy to establish the diagnosis, rarely plays a role in the
treatment of CNS germinomas. The role of surgical
resection for nongerminomatous germ cell tumors and teratomas remains to be
defined.[1] For germinomas, irradiation with doses of 45 Gy to 54 Gy to the
tumor and 21 Gy to 36 Gy to the whole brain and spine is usually curative. In
selected cases, germinoma can be effectively treated with ventricular field radiation
therapy and at lower dose levels (30–36 Gy) following response to chemotherapy.[1] Although experience with
pre-irradiation chemotherapy has shown that most of these tumors
respond to cyclophosphamide and platinum-containing drugs, the definitive role
of chemotherapy has yet to be determined.[1] Disseminated germinomas are treated with craniospinal
irradiation,[2][3] alone or in combination with chemotherapy. The usual dose to the
tumor is 45 Gy to 54 Gy with 27 Gy to 36 Gy to the whole brain and spine. Although nongerminomatous germ cell tumors (e.g., embryonal carcinomas, yolk cell tumors, and mixed germ cell tumors) may respond
to chemotherapeutic agents (e.g., cisplatin or carboplatin, etoposide,
cyclophosphamide, and vinblastine) as do such histologies outside of the CNS,
optimal combination of agents, and the timing of chemotherapy in relation to radiation therapy
remains to be determined.[4][5][6]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood central nervous system germ cell tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Craniopharyngioma
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood craniopharyngioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Ependymoma
Refer to the PDQ summary on Childhood Ependymoma Treatment for more
information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
newly diagnosed childhood ependymoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Ependymoblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood ependymoblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Malignant Glioma
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood cerebral astrocytoma/malignant glioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Medulloblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more
information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
untreated childhood medulloblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Medulloepithelioma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood medulloepithelioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Pineal Parenchymal Tumors
Childhood pineal parenchymal tumors include:
- Pineoblastomas.
- Pineocytomas.
- Pineal parenchymal tumors of intermediate differentiation.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood pineal parenchymal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Spinal Cord Tumors
The optimal treatment for intrinsic/intramedullary glial spinal cord tumors has
not been determined by prospective randomized trials. Therapeutic options
include surgery alone, surgery plus local radiation therapy, and possibly
adjuvant chemotherapy in selected cases.[7][8] Extensive surgical resections
are technically possible for many patients with intramedullary spinal cord
tumors, but may result in worsening neurologic status in at least 10%
of cases.[7][8] Surgery is usually indicated at least to determine the type of
tumor present; for low-grade glial tumors this may be the only treatment
required.[7] In one recent series of 164 children and young adults with
intramedullary low-grade glial tumors or ganglioglial spinal cord tumors, 70%
were controlled for 5 years after extensive surgical resections.[7] Radiation therapy has been demonstrated to control disease in some patients with
low-grade glial tumors after subtotal resections.[8][9][10] The role of
chemotherapy for spinal cord tumors is poorly characterized, but some very
young children with low-grade glial tumors have been successfully treated with
a carboplatin and vincristine drug regimen.[11] Outcomes for patients with
high-grade glial tumors have been extremely poor; most
develop progressive disease within 3 years of treatment with surgery,
radiation, and/or chemotherapy.[7][9][10]
The optimal treatment for children with spinal ependymomas has not been well characterized (refer to the PDQ summary on Childhood Ependymoma Treatment for more information). As is the case for glial tumors, treatment options
predominantly consist of either surgery alone or surgery followed by local
radiation therapy.[7][8] Management of primitive neuroectodermal tumors of the
spinal cord is also not well delineated, and most patients are treated on
treatment protocols designed for children with high-risk medulloblastoma.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood spinal cord neoplasm. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Supratentorial Primitive Neuroectodermal Tumors
Childhood supratentorial primitive neuroectodermal tumors include:
- Primitive neuroectodermal tumors.
- Cerebral Neuroblastomas.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood supratentorial primitive neuroectodermal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References:
Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.
Dearnaley DP, A'Hern RP, Whittaker S, et al.: Pineal and CNS germ cell tumors: Royal Marsden Hospital experience 1962-1987. Int J Radiat Oncol Biol Phys 18 (4): 773-81, 1990.
Linstadt D, Wara WM, Edwards MS, et al.: Radiotherapy of primary intracranial germinomas: the case against routine craniospinal irradiation. Int J Radiat Oncol Biol Phys 15 (2): 291-7, 1988.
Balmaceda C, Heller G, Rosenblum M, et al.: Chemotherapy without irradiation--a novel approach for newly diagnosed CNS germ cell tumors: results of an international cooperative trial. The First International Central Nervous System Germ Cell Tumor Study. J Clin Oncol 14 (11): 2908-15, 1996.
Bouffet E, Baranzelli MC, Patte C, et al.: Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience. Société Française d'Oncologie Pédiatrique. Br J Cancer 79 (7-8): 1199-204, 1999.
Robertson PL, DaRosso RC, Allen JC: Improved prognosis of intracranial non-germinoma germ cell tumors with multimodality therapy. J Neurooncol 32 (1): 71-80, 1997.
Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.
Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.
Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.
O'Sullivan C, Jenkin RD, Doherty MA, et al.: Spinal cord tumors in children: long-term results of combined surgical and radiation treatment. J Neurosurg 81 (4): 507-12, 1994.
Hassall TE, Mitchell AE, Ashley DM: Carboplatin chemotherapy for progressive intramedullary spinal cord low-grade gliomas in children: three case studies and a review of the literature. Neuro-oncol 3 (4): 251-7, 2001.
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Treatment of Recurrent Childhood Brain and Spinal Cord Tumors
Recurrence is not uncommon in both low-grade and malignant childhood brain tumors
and may occur many years after initial treatment.[1] 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 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.
Childhood Astrocytoma
Childhood astrocytomas are classified as low-grade or high-grade.
Childhood Low-Grade Astrocytomas:
- Pilocytic astrocytoma.
- Diffuse fibrillary astrocytoma.
Childhood High-Grade Astrocytomas:
- Anaplastic astrocytoma.
- Glioblastoma multiforme.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood Brain Stem Glioma
Childhood brain stem gliomas include:
- Diffuse intrinsic pontine gliomas.
- Focal or low-grade brain stem gliomas.
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more
information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood brain stem glioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
CNS Tumors in Children Aged 3 Years and Younger
Studies have addressed the treatment of infants who have progressive disease in
spite of chemotherapy. Approaches that have been used include further surgery,
chemotherapy, local and/or craniospinal radiation therapy, high-dose chemotherapy
supported by autologous stem cell rescue, or combinations of chemotherapy and
radiation therapy. Overall salvage rates have been less than optimal, but a
subgroup of children, primarily those with localized disease at the time of
relapse, may experience prolonged disease control and possible cure with
treatment after recurrence.[2][3][4][5][6][7] For children aged 2 years and younger, the use of high-dose craniospinal irradiation has been associated with poor neurocognitive outcome. Treatment for young children with multiple
recurrent and/or disseminated brain tumors is even more problematic and entry
into phase I and phase II trials is indicated to identify more effective and less
toxic agents.
Childhood CNS Embryonal Tumors
Childhood CNS embryonal tumors include:
- CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
- Ependymoblastomas.
- Medulloblastomas.
- Medulloepitheliomas.
- Pineal parenchymal tumors of intermediate differentiation.
- Pineoblastomas.
- Supratentorial primitive neuroectodermal tumors.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood CNS Germ Cell Tumors
Childhood CNS germ cell tumors include:
- Germinomas.
- Embryonal yolk sac tumors.
- Choriocarcinomas.
- Immature teratomas.
- Mature teratomas.
- Teratomas with malignant transformation.
- Mixed germ cell tumors.
- Nongerminomatous germ cell tumors.
Germ cell tumors may be chemoresponsive. Patients may benefit from the types
of agents that are used to treat germ cell tumors in other locations; these agents include cisplatin, etoposide, and cyclophosphamide. Patients with recurrent germ cell tumors for whom the
standard chemotherapy options have failed may be entered into phase I and phase
II studies that are designed to determine the activity and toxic effects of
agents new to the treatment of this tumor.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood central nervous system germ cell tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Craniopharyngioma
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Childhood Ependymoma
Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood ependymoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Ependymoblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Low-Grade Glial Tumors
Surgical resection, radiation therapy (especially if not previously given),
and chemotherapy may result in prolonged disease stabilization for children
with recurrent low-grade tumors. Resection is an option for those patients
with a surgically accessible lesion and has the advantage of documenting the
histology of the recurrent tumor. Radiation therapy, if not previously given,
may result in tumor shrinkage and long-term disease control.
Chemotherapy with drugs such as carboplatin and vincristine has recently been
shown to result in tumor shrinkage and disease control for children with
low-grade glial neoplasms.[8] Similar results have been demonstrated for
hypothalamic and chiasmatic tumors treated with etoposide.[9] Entry into phase
I and phase II trials is indicated to identify more effective and less toxic
agents.
Childhood Medulloblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood medulloblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Medulloepithelioma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood medulloepithelioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Pineal Parenchymal Tumors
Childhood pineal parenchymal tumors include:
- Pineoblastomas.
- Pineocytomas.
- Pineal parenchymal tumors of intermediate differentiation.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood pineoblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Spinal Cord Tumors
At the time of recurrence, low-grade spinal cord glial tumors can be
treated with re-resection with or without the use of radiation therapy.
Recurrent low-grade and high-grade tumors which cannot be re-resected can be
treated on protocols designed for histologically similar brain tumors.
For more information, refer to the PDQ summaries on Childhood Ependymoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood spinal cord neoplasm. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Supratentorial Primitive Neuroectodermal Tumors
Childhood suprantentorial primitive neuroectodermal tumors include:
- Primitive neuroectodermal tumors.
- Cerebral neuroblastomas.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood supratentorial primitive neuroectodermal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References:
Jenkin D, Greenberg M, Hoffman H, et al.: Brain tumors in children: long-term survival after radiation treatment. Int J Radiat Oncol Biol Phys 31 (3): 445-51, 1995.
Fisher PG, Needle MN, Cnaan A, et al.: Salvage therapy after postoperative chemotherapy for primary brain tumors in infants and very young children. Cancer 83 (3): 566-74, 1998.
Walter AW, Mulhern RK, Gajjar A, et al.: Survival and neurodevelopmental outcome of young children with medulloblastoma at St Jude Children's Research Hospital. J Clin Oncol 17 (12): 3720-8, 1999.
Goldwein JW, Glauser TA, Packer RJ, et al.: Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66 (3): 557-63, 1990.
Dupuis-Girod S, Hartmann O, Benhamou E, et al.: Will high dose chemotherapy followed by autologous bone marrow transplantation supplant cranio-spinal irradiation in young children treated for medulloblastoma? J Neurooncol 27 (1): 87-98, 1996.
Dunkel IJ, Boyett JM, Yates A, et al.: High-dose carboplatin, thiotepa, and etoposide with autologous stem-cell rescue for patients with recurrent medulloblastoma. Children's Cancer Group. J Clin Oncol 16 (1): 222-8, 1998.
Guruangan S, Dunkel IJ, Goldman S, et al.: Myeloablative chemotherapy with autologous bone marrow rescue in young children with recurrent malignant brain tumors. J Clin Oncol 16 (7): 2486-93, 1998.
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.
Chamberlain MC, Grafe MR: Recurrent chiasmatic-hypothalamic glioma treated with oral etoposide. J Clin Oncol 13 (8): 2072-6, 1995.
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More Information
About PDQ
Additional PDQ Summaries
Important:
This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
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This information is provided by the National Cancer Institute.
This information was last updated on October 14, 2009.