Choroid Plexus Tumor

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    Choroid plexus tumor is a rare type of cancer that occurs in the ventricles of the brain. It usually occurs in children younger than 2 years. Learn about choroid plexus tumors in children and find information on how we support and care for children with choroid plexus tumor 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:

  • Quick and accurate diagnosis from our dedicated pediatric neuropathologist
  • Access to advanced technologies like the intraoperative MRI, which allows our neurosurgeons to see detailed images of the brain during surgery
  • Advanced pediatric radiation oncology services, including targeted radiosurgery and low-dose radiation therapy that minimize exposure to radiation
  • Outpatient and oral chemotherapy, which may minimize the number of times your child will need to visit the hospital
  • Innovative therapies offered through clinical trials at Dana-Farber, Boston Children's Hospital, and nationally
  • Specialized programs for the treatment of low- and high-grade gliomas, and medulloblastoma

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.

Information for: Patients | Healthcare Professionals

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

Children with choroid plexus brain tumors are treated through the Brain Tumor Center at 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 Brain Tumor Center is a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors.

After treatment, your child will receive expert follow-up care through the Stop & Shop Neuro-Oncology Outcomes Clinic at Dana-Farber/Boston Children's, 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 through neurosurgery. 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.

Clinical trials 

For many children with rare or hard-to-treat conditions, clinical trials provide new options.

 


General Information About Childhood Brain and Spinal Cord Tumors

Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2002, childhood cancer mortality decreased by more than 50%.[1] 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.

Incidence

Primary central nervous system 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.

References:

  1. 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.

Classification of Central Nervous System Tumors

The classification of childhood central nervous system (CNS) tumors is based on histology and location.[1] 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.[1]

Infratentorial (posterior fossa) tumors include the following:

  1. Cerebellar astrocytomas (most commonly pilocytic, but also fibrillary and less frequently, high grade).
  2. Medulloblastomas (including classic, desmoplastic/nodular, extensive nodularity, anaplastic, or large cell variants).
  3. Ependymomas (cellular, papillary, clear cell, tanycytic, or anaplastic).
  4. Brain stem gliomas (typically diffuse intrinsic pontine gliomas and focal, tectal, and exophytic cervicomedullary gliomas are most frequently pilocytic astrocytomas).
  5. Atypical teratoid/rhabdoid tumors.
  6. Choroid plexus tumors (papillomas and carcinomas).
  7. Rosette-forming glioneuronal tumors of the fourth ventricle.

Supratentorial tumors include the following:

  1. Low-grade cerebral hemispheric astrocytomas (grade I [pilocytic] astrocytomas or grade II [diffuse] astrocytomas).
  2. High-grade or malignant astrocytomas (anaplastic astrocytomas and glioblastoma [grade III or grade IV]).
  3. Mixed gliomas (low- or high-grade).
  4. Oligodendrogliomas (low- or high-grade).
  5. Primitive neuroectodermal tumors (PNETs) (cerebral neuroblastomas, pineoblastomas, and ependymoblastomas).
  6. Atypical teratoid/rhabdoid tumors.
  7. Ependymomas (cellular or anaplastic).
  8. Meningiomas (grades I, II, and III).
  9. Choroid plexus tumors (papillomas and carcinomas).
  10. Tumors of the pineal region (pineocytomas, pineoblastomas, pineal parenchymal tumors of intermediate differentiation, and papillary tumors of the pineal region), and germ cell tumors.
  11. Neuronal and mixed neuronal glial tumors (gangliogliomas, desmoplastic infantile astrocytoma/gangliogliomas, dysembryoplastic neuroepithelial tumors, and papillary glioneuronal tumors).
  12. Other low-grade gliomas (including subependymal giant cell tumors and pleomorphic xanthoastrocytoma).
  13. Metastasis (rare) from extraneural malignancies.

Parasellar tumors include the following:

  1. Craniopharyngiomas.
  2. Diencephalic astrocytomas (central tumors involving the chiasm, hypothalamus, and/or thalamus) that are generally low-grade (including astrocytomas, grade I [pilocytic] or grade II [diffuse]).
  3. Germ cell tumors (germinomas or nongerminomatous).

Spinal cord tumors include the following:

  1. Low-grade cerebral hemispheric astrocytomas (grade I [pilocytic] astrocytomas or grade II [diffuse] astrocytomas).
  2. High-grade or malignant astrocytomas (anaplastic astrocytomas and glioblastoma [grade III or grade IV]).
  3. Gangliogliomas.
  4. Ependymomas (often myxopapillary).

References:

  1. 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.

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 tumor or spinal cord tumor include the following:

  1. The cause of most childhood brain tumors remains unknown.[1]
  2. Selection of an appropriate therapy can only occur if the correct diagnosis is made and the stage of the disease is accurately determined.
  3. 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.
  4. 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.
  5. 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.[7][8][9] Secondary tumors have increasingly been diagnosed in long-term survivors.[10] 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.[11][12][13] 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.)

  6. 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.[14]

References:

  1. Fisher JL, Schwartzbaum JA, Wrensch M, et al.: Epidemiology of brain tumors. Neurol Clin 25 (4): 867-90, vii, 2007.

  2. 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.

  3. Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.

  4. 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.

  5. 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.

  6. Iuvone L, Peruzzi L, Colosimo C, et al.: Pretreatment neuropsychological deficits in children with brain tumors. Neuro Oncol 13 (5): 517-24, 2011.

  7. 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.

  8. 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.

  9. 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.

  10. Jenkin D: Long-term survival of children with brain tumors. Oncology (Huntingt) 10 (5): 715-9; discussion 720, 722, 728, 1996.

  11. 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.

  12. 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.

  13. 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.

  14. 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.

Stage Information and Treatment of Newly Diagnosed and Recurrent Childhood Brain Tumors

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 [1][2] and low-grade gliomas.[3][4]

Newly Diagnosed or Recurrent Tumor Type and Its Related PDQ Treatment Summary

Tumor Type

Pathologic Subtype

Related PDQ Treatment Summary

Astrocytomas and Other Tumors of Glial Origin

Low-Grade Astrocytomas

Diffuse fibrillary astrocytoma

Childhood Astrocytomas Treatment

Gemistocytic astrocytoma

Oligoastrocytoma

Oligodendroglioma

Pilocytic astrocytoma

Pilomyxoid astrocytoma

Pleomorphic xanthoastrocytoma

Protoplasmic astrocytoma

Subependymal giant cell astrocytoma

High-Grade Astrocytomas

Anaplastic astrocytoma

Childhood Astrocytomas Treatment

Anaplastic oligoastrocytoma

Anaplastic oligodendroglioma

Giant cell glioblastoma

Glioblastoma

Gliomatosis cerebri

Gliosarcoma

Brain Stem Glioma

Diffuse intrinsic pontine glioma

Childhood Brain Stem Glioma Treatment

Focal or low-grade brain stem glioma

CNS Embryonal Tumors

Medulloblastomas

Anaplastic

Childhood CNS Embryonal Tumors Treatment

Classic

Desmoplastic/nodular

Large cell

Medulloblastoma with extensive nodularity

CNS Primitive Neuroectodermal Tumors (PNETs)

CNS ganglioneuroblastoma

CNS neuroblastoma

Ependymoblastoma

Medulloepithelioma

Pineal Parenchymal Tumors

Pineoblastoma

CNS Atypical Teratoid/Rhabdoid Tumor

Childhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment

CNS Germ Cell Tumors

Germinomas

Childhood CNS Germ Cell Tumors Treatment

Teratomas

Immature teratoma

Mature teratoma

Teratoma with malignant transformation

Non-Germinomatous Germ Cell Tumors

Choriocarcinoma

Embryonal carcinoma

Mixed germ cell tumor

Yolk sac tumor

Craniopharyngioma

Childhood Craniopharyngioma Treatment

Ependymoma

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.

References:

  1. Northcott PA, Shih DJ, Peacock J, et al.: Subgroup-specific structural variation across 1,000 medulloblastoma genomes. Nature 488 (7409): 49-56, 2012.

  2. 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.

  3. 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.

  4. 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.

Stage Information and Treatment of Newly Diagnosed and Recurrent Childhood Spinal Cord Tumors

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:

  • Childhood Astrocytomas Treatment.
  • Childhood Central Nervous System Embryonal Tumors Treatment.
  • Childhood Ependymoma Treatment.

This information is provided by the National Cancer Institute.

This information was last updated on January 28, 2014.

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      Boston Children's Cancer and Blood Disorders Center is the top ranked pediatric cancer hospital in the U.S. News & World Report 2014-15 Best Children's Hospitals guide.