Germ Cell Tumor of the Brain

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    A germ cell tumor of the brain arises from primitive developing cells that form in the embryo and may otherwise become the reproductive system. Learn about brain tumors in children and find information on how we support and care for children with germ cell tumors of the brain 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

Germ Cell Tumor of the Brain

Overview

A germ cell tumor develops from cells primitive developing cells in the embryo that should migrate and create the reproductive system. 

  • Germ cell tumors in the brain are very rare.
  • They are usually treated with surgery, but radiation therapy and chemotherapy may also be used, if completely removing the tumor removal surgically isn’t possible.
  • Certain germ cell tumors release measurable substances into the blood. These tumor markers can be tested to follow the tumor’s response to treatment.

As you read further, you will find general information about germ cell tumors in the brain.

How Dana-Farber/Boston Children's Cancer and Blood Disorders Center approaches germ cell tumors of the brain

Children with germ cell tumors of the brain 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.

We understand that you may have a lot of questions when your child is diagnosed with acute a germ cell tumor of the brain. Is it dangerous? Will it affect my child long-term? What do we do next? We’ve tried to provide some answers to those questions here, and our experts can explain your child’s condition fully.

We hold a weekly brain tumor clinic for newly diagnosed patients currently receiving treatment. Each time you come for an appointment, you meet with every specialist on your child’s team, from your pediatric neuro-oncologist, neurologist and neurosurgeon, to your pediatric endocrinologist, psycho-oncologist and school liaison.

Dana-Farber/Boston Children's Pediatric Brian Tumor Program offers the following services:

  • Access to high-tech resources, like the intra-operative MRI, which allows our pediatric neurosurgeons to visualize the tumor as they operate with MRI scans. This means they can remove as much of the tumor as possible, and sometimes eliminate additional surgeries.
  • Expert neuropathological review, using advanced molecular diagnostic testing, to identify your child’s exact type of tumor. This information helps predict which treatments are more likely to work.
  • Access to unique Phase I clinical trials, from our own investigators, the Children’s Oncology Group and the Pediatric Oncology Experimental Therapeutics Investigators Consortium. Studies offer treatment options beyond standard therapy.
  • Ongoing care from pediatric neurologists familiar with the early symptoms and side effects of brain tumors and their treatments.
  • Access to one of the nation’s few dedicated pediatric brain tumor survivorship programs. This weekly clinic offers ongoing care to manage late effects caused by your child’s tumor or the treatment they received.

In-depth

What is a germ cell tumor of the brain?

A germ cell tumor develops from cells primitive developing cells in the embryo that should migrate and create the reproductive system.

What causes a germ cell tumor of the brain?

Usually germ cells migrate to the gonads during fetal development and become an egg in the female ovaries or sperm in a man’s testes. If they don’t move to the right area, they begin to multiply in areas where they shouldn’t be.

  • Most germ cell tumors occur outside the brain, in the chest or abdomen. 
  • Germ cell tumors in the brain are most commonly found in the pineal and suprasellar regions.
  • They account for about 2 percent of all pediatric brain tumors.
  • Around half of these tumors occur in young people between ages 10 and 20.
What are the symptoms of germ cell tumors in the brain?

Symptoms vary depending on size and location of tumor. This tumor can block the normal flow of depending on size and location of tumor. Sometimes the tumor may block the normal flow of the cerebral spinal fluid (CSF), causing increased pressure on the brain (hydrocephalus) and enlargement of the skull and a variety of symptoms. Common symptoms may include:  

  • headaches ( especially upon awakening).
  • nausea and vomiting (especially upon awakening).
  • lethargy and irritability.
  • problems feeding or walking.
  • enlarged head size or fontanels, the soft "spot" that occurs before the bones in the head become solid, in infants.

For germ cell tumors found in other areas of the brain, common symptoms may include:

  • vision loss
  • hormone abnormalities

The symptoms of a brain tumor may resemble other conditions or medical problems, ranging from the simple to the serious. Always consult your child's physician for diagnosis and treatment.

What are the different kinds of germ cell tumors in the brain?

Germ cell tumors are a widely varied group of tumors. They range from very low grade or benign to highly malignant or aggressively growing cancers.  

  • Germinomas are the most common form of germ cell tumors in the brain.
  • Teratomas range from mature to immature and even malignant.
  • Mixed non-germinomas contain cancerous, or malignant, forms of these tumors, including:
    • embryonal carcinoma
    • choriocarcinoma
    • endodermal sinus [yolk sac] tumors
     

The prognosis and treatment of each of these depends on their location, size, and other characteristics.

Tests

How are germ cell tumors in the brain diagnosed?

Diagnostic procedures for germ cell tumors, like other brain tumors, determine the exact type of tumor and whether the tumor has spread. These may include a:

  • physical exam to test neurologic function including: reflexes, muscle strength, eye and mouth movement, coordination, and alertness.
  • magnetic resonance imaging (MRI) to produce detailed images of organs and structures within the brain and/or spine.
  • computerized tomography scan (also called a CT or CAT scan) to capture a detailed view of the brain, in some cases.
  • Biopsy or tissue sample from the tumor to provide definitive information about the type of tumor. This is collected during surgery.
  • lumbar puncture (spinal tap) to remove a small sample of cerebrospinal fluid (CSF) and determine if any tumor cells have started to spread. In young children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.
  • Blood tests to measure if markers exist. Certain germ cell tumors release measurable substances into the blood and CSF (e.g. alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (B-HCG)). These tumor markers can be repeatedly tested to follow the response of a tumor to treatment.

Treatment & care

At Dana-Farber/Boston Children's, we know how difficult a diagnosis of germ cell tumors 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.

What are the treatments for a germ cell tumors of the brain?

Treatment may include the following, alone or in combination.

Surgery
Usually first step of treatment is surgery: a pediatric neurosurgeon removes as much of the tumor as possible while preserving your child’s neurological function. Tumor specimens are examined by neuropathologists.

  • Surgery is usually limited to well encapsulated teratomas, a particular tumor that includes all three cellular layers of germ cells, or in the case of blocked CSF flow.
     
  • Complete resection or surgical removal of the entire tumor is ideal when possible. However, tumor location and other characteristics may limit removal to a partial or sub-total resection.
     
  • A biopsy is the surgical removal of a sample of the tumor for diagnostic purposes. This is frequently done if the tumor is in an area with sensitive structures around it that may be injured during removal.

Ventriculo-peritoneal shunt (VP shunt)
When a tumor causes blockage of cerebral spinal fluid (CSF) flow, special tubing can be surgically implanted in the ventricles to drain excess CSF into the abdomen. This bypasses the tumor blockage and relieves symptoms of hydrocephalus, the build up of fluid inside the skull.

Radiation therapy
Precisely targeted and dosed radiation therapy is used to kill cancer cells left behind after surgery. This treatment is important to control the local growth of tumor.

  • Tumors that are not likely to spread receive radiation only to the tumor and the area surrounding it.
  • If we determine the tumor is more likely to spread beyond its original tumor location, radiation to other parts of the brain and spinal cord may be recommended.
  • If the tumor has spread, radiation to the whole brain and spinal cord are used to treat certain germ cell tumors.

Chemotherapy
Chemotherapy is a drug that interferes with the cancer cell’s ability to grow or reproduce. Chemotherapy before surgery may help shrink the tumor, making it possible to remove.

  • Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
  • Often, a combination of chemotherapy drugs is used.
  • Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.

While chemotherapy can be quite effective in treating certain cancers, the agents don’t differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.

Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:

  • as a pill to swallow.
  • as an injection into the muscle or fat tissue.
  • Intravenously, directly to the bloodstream (also called IV).
  • Intrathecally, which means directly delivering the chemotherapy into the spinal column with a needle.
What is the expected prognosis after treatment for my child?

Outcomes vary widely, depending on the precise type of germ cell tumor your child has. In general, germinomas are cured in greater than 85 percent of cases with combined treatment. 

  • Mature teratomas are curable with complete resection alone.
  • Immature teratomas usually require additional therapy.
  • Other germ cell tumors, the mixed and malignant types, are more difficult to treat.
What is the recommended long-term care for my child?

Children treated for a germ cell tumor in the brain should visit a survivorship clinic every year to:

  • manage disease complications
  • screen for early recurrence of tumor
  • manage late effects of treatment

A typical follow-up visit may include some or all of the following:

  • a physical exam
  • laboratory testing
  • imaging scans

Through the Stop and Shop Family Pediatric Neuro-Oncology Outcomes Clinic as Dana-Farber Cancer Institute, children are able to meet with their neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit.

  • Endocrinologists, neuro-psychologists, alternative/complementary therapy specialists, and school liaison and psychosocial personnel from the pediatric brain tumor team are also available.
  • In addition, children needing rehabilitation may meet with speech, physical, and occupational therapists during and after treatments.

Research and innovations

The pediatric neurosurgeons at Dana-Farber/Boston Children's have access to the most recent technological advances such as the intra-operative MRI, which allow them to visualize the tumor as they operate with MRI scans, so they can remove as much of the tumor as possible.

We also have access to high-tech imaging, such as PET, CT and functional MRI, which enable us to understand exactly where the tumor tissue is, and to map out surgeries and treatments that minimize risk to healthy brain tissue.

Our pediatric neuropathologists see hundreds of tissue samples each year, giving them in-depth knowledge of tissue subtypes so you receive a quick and accurate diagnosis.

What is the latest research on germ cell tumors?

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 or refractory disease. It is also the New England Phase I Center of the Children’s Oncology Group. Children with progressive/recurrent gangliogliomas may be eligible for experimental therapies available through these consortiums.

Participation in any clinical trial is completely voluntary: We will take care to fully explain all elements of the treatment plan prior to the start of the trial, and you may remove your child from the medical study at any time.


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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