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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:
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.
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.
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.
We understand that you may have a lot of questions when your child is diagnosed with a choroid plexus tumor:
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.
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)
Choroid plexus carcinomas (CPC)
In rare cases, a choroid plexus tumor may appear to have characteristics of both CPP and CPC.
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.
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.
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:
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.
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:
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.
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.
A: We offer a variety of services to help you, your child and your family get through this difficult time.
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.
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.
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:
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.
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:
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:
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 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:
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.
Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.
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).
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.
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:
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.
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.
For many children with rare or hard-to-treat conditions, clinical trials provide new options.
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.
Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are also important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of proliferative activity are increasingly used in tumor diagnosis and classification.
Primary central nervous system tumors are a diverse group of diseases that together constitute
the most common solid tumor in childhood. The Central Brain Tumor Registry of the United States (CBTRUS) estimates that approximately 4,300 U.S. children are diagnosed each year.
Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.
Ostrom QT, Gittleman H, Farah P, et al.: CBTRUS statistical report: Primary brain and central nervous system tumors diagnosed in the United States in 2006-2010. Neuro Oncol 15 (Suppl 2): ii1-56, 2013.
The classification of childhood central nervous system (CNS) tumors is based on histology and location. Tumors are classically categorized as infratentorial, supratentorial, parasellar, or spinal.
Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of proliferative activity are increasingly used in tumor diagnosis and classification and will likely affect classification and nomenclature in the future.
Primary CNS spinal cord tumors comprise approximately 1% to 2% of all childhood CNS tumors. The classification of spinal cord tumors is based on histopathologic characteristics of the tumor and does not differ from that of primary brain tumors.
Infratentorial (posterior fossa) tumors include the following:
Supratentorial tumors include the following:
Parasellar tumors include the following:
Spinal cord tumors include the following:
Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.
Important concepts that should be understood by those treating and caring for a
child who has a brain tumor or spinal cord tumor include the following:
(Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information about possible long-term or late effects.)
Fisher JL, Schwartzbaum JA, Wrensch M, et al.: Epidemiology of brain tumors. Neurol Clin 25 (4): 867-90, vii, 2007.
Blaney SM, Haas-Kogan D, Young Poussaint T, et al.: Gliomas, ependymomas, and other nonembryonal tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia, Pa: Lippincott Williams and Wilkins, 2011, pp 717-771.
Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.
Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010.
Reimers TS, Mortensen EL, Nysom K, et al.: Health-related quality of life in long-term survivors of childhood brain tumors. Pediatr Blood Cancer 53 (6): 1086-91, 2009.
Iuvone L, Peruzzi L, Colosimo C, et al.: Pretreatment neuropsychological deficits in children with brain tumors. Neuro Oncol 13 (5): 517-24, 2011.
Armstrong GT: Long-term survivors of childhood central nervous system malignancies: the experience of the Childhood Cancer Survivor Study. Eur J Paediatr Neurol 14 (4): 298-303, 2010.
Duffner PK, Horowitz ME, Krischer JP, et al.: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328 (24): 1725-31, 1993.
Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11 (5): 850-6, 1993.
Mason WP, Grovas A, Halpern S, et al.: Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16 (1): 210-21, 1998.
Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.
Presently, there is no uniformly accepted staging system for most childhood brain tumors. These tumors are classified and treated based on their histology and location within the brain (see Table below). However, with advances in molecular data, it is conceivable that genomic factors will refine classification approaches for certain groups of tumors, such as medulloblastomas  and low-grade gliomas.
Related PDQ Treatment Summary
Astrocytomas and Other Tumors of Glial Origin
– Low-Grade Astrocytomas
Diffuse fibrillary astrocytoma
Childhood Astrocytomas Treatment
Subependymal giant cell astrocytoma
– High-Grade Astrocytomas
Giant cell glioblastoma
Brain Stem Glioma
Diffuse intrinsic pontine glioma
Childhood Brain Stem Glioma Treatment
Focal or low-grade brain stem glioma
CNS Embryonal Tumors
Childhood CNS Embryonal Tumors Treatment
Medulloblastoma with extensive nodularity
– CNS Primitive Neuroectodermal Tumors (PNETs)
– Pineal Parenchymal Tumors
– CNS Atypical Teratoid/Rhabdoid Tumor
Childhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment
CNS Germ Cell Tumors
Childhood CNS Germ Cell Tumors Treatment
Teratoma with malignant transformation
– Non-Germinomatous Germ Cell Tumors
Mixed germ cell tumor
Yolk sac tumor
Childhood Craniopharyngioma Treatment
Subependymoma (WHO Grade I)
Childhood Ependymoma Treatment
Myxopapillary ependymoma (WHO Grade I)
Ependymoma (WHO Grade II)
Anaplastic ependymoma (WHO Grade III)
Tumors of the Choroid Plexus
CNS = central nervous system; WHO = World Health Organization.
Recurrence is not uncommon in both low-grade and malignant childhood brain tumors and may occur many years after initial treatment. Disease may occur at the primary tumor site or, especially in malignant tumors, at noncontiguous central nervous system (CNS) sites. Systemic relapse is rare but may occur. At time of recurrence, a complete evaluation for extent of relapse is indicated for all malignant tumors and, at times, for lower-grade lesions. Biopsy or surgical re-resection may be necessary for confirmation of relapse; other entities, such as secondary tumor and treatment-related brain necrosis, may be clinically indistinguishable from tumor recurrence. The determination of the need for surgical intervention must be individualized based on the initial tumor type, the length of time between initial treatment and the reappearance of the lesion, and the clinical picture.
Early-phase therapeutic trials may be available for selected patients via Children's Oncology Group phase I institutions, the Pediatric Brain Tumor Consortium, or other entities.
Northcott PA, Shih DJ, Peacock J, et al.: Subgroup-specific structural variation across 1,000 medulloblastoma genomes. Nature 488 (7409): 49-56, 2012.
Kool M, Korshunov A, Remke M, et al.: Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol 123 (4): 473-84, 2012.
Jones DT, Kocialkowski S, Liu L, et al.: Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas. Cancer Res 68 (21): 8673-7, 2008.
Pfister S, Janzarik WG, Remke M, et al.: BRAF gene duplication constitutes a mechanism of MAPK pathway activation in low-grade astrocytomas. J Clin Invest 118 (5): 1739-49, 2008.
There is no uniformly accepted staging system for childhood primary spinal cord tumors. These tumors are classified and treated based on their location within the spinal cord and histology. Refer to the following PDQ summaries for more information on the staging and treatment of newly diagnosed and recurrent childhood spinal cord tumors:
This information is provided by the National Cancer Institute.
This information was last updated on August 12, 2014.
Many children with cancer receive treatment in the outpatient setting, which allows them to stay in school and continue to develop intellectually and socially. However, returning to school can be an emotional experience; our Back to School Program is designed to ease your child's transition back to the classroom.
Concierge Services is your one-stop place to learn about Dana-Farber programs, services and resources, as well as information on getting around Boston, finding lodging or restaurants, and activities in the area.
The Expressive Arts Therapy program, sponsored by the Leonard P. Zakim Center for Integrative Therapies, provides adult patients, family members, and caregivers with a variety of options to support well-being during cancer treatment. From live music meditation to painting technique workshops, the program offers a range of creative outlets to suit every interest.
Dana-Farber and Children's Hospital, including parking facilities, are fully accessible to people with disabilities. There are wheelchairs at the main entrance, and security staff can provide personal assistance. We also have many educational materials available in large print and audiotape formats.
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Find practical tips and suggestions for individuals caring for a family member or friend with cancer, including creating a caregiving plan, finding community resources, and looking after your own well-being.
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The Friends' Corner Gift Shop, located on the first floor of the Yawkey Center for Cancer Care, offers a wide selection of unique gifts and everyday items for patients, families and staff.
Every year, thousands of patients with cancer from around the world come to Dana-Farber for their care. We provide a wide array of logistical and other services for individuals who live outside the United States.
Dana-Farber provides interpreting services for patients whose first language is not English. Interpreters may be requested for any activity, including registration, booking appointments, attending treatments and exams, support groups, and meetings with doctors and other members of your health care team.
Just for Teens provides programs and activities for teens and young adults with cancer at the Jimmy Fund Clinic and Children's Hospital Boston. We offer activities and events both inside and out of the hospital so that you have creative ways to pass the time and can meet other teens who are going through similar experiences.
Our nutritionists are registered dietitians who can assist you in planning an optimal diet during any stage of your cancer journey, cope with any side effects you may experience, and answer your questions about the latest findings on cancer and nutrition.
The Eleanor and Maxwell Blum Patient and Family Resource Center and its satellite resource rooms are staffed by health care professionals and provide computer stations, books, brochures, videos, and CDs to help you find information and support on a variety of issues about cancer treatment and care.
Patients websites help friends and family members stay up-to-date on their loved ones' condition and write messages of support and encouragement.
The Dana-Farber pharmacy fills prescriptions for all pediatric and adult patients. Our pharmacists are an extension of the patient care team and work closely with your physicians to provide seamless, convenient, safe care.
More than 1,200 Dana-Farber patients and their families have enjoyed free trips to baseball games, theater shows, museums, and other attractions this year through the Recreational Resources program.
The School Liaison Program is for pediatric patients who are diagnosed with or have completed a treatment that involves the central nervous system. We provide consultation about the cognitive late effects of treatment to help parents understand and advocate for their child's learning needs.
Through all stages of cancer treatment and survivorship, our Spiritual Care staff is available 24 hours a day to provide emotional and spiritual support for adults and pediatric patients and family members.
Integrative therapies, also known as complementary therapies, range from acupuncture and massage to nutritional guidance and music therapy. Patients treated at the Zakim Center credit its services with easing nausea, improving circulation, and reducing pain, stress, and anxiety associated with cancer treatment.
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