Childhood Brain Tumors

Summary Type: Treatment
Summary Audience: Health professionals
Summary Language: English
Summary Description: Expert-reviewed information summary about the treatment of various childhood brain tumors.


Childhood Brain Tumors

General Information

Note: Separate PDQ summaries on Childhood Brain Stem Glioma Treatment; Childhood Cerebellar Astrocytoma Treatment; Childhood Cerebral Astrocytoma/Malignant Glioma Treatment; Childhood Medulloblastoma Treatment; Childhood Supratentorial Primitive Neuroectodermal Tumors and PineoblastomaTreatment; and Childhood Visual Pathway and Hypothalamic Glioma Treatment are also available.

This cancer treatment information summary provides an overview of the diagnosis, classification, treatment, and prognosis of childhood brain tumors.

The National Cancer Institute provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. These summaries are updated regularly according to the latest published research findings by an Editorial Board of pediatric oncology specialists.

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 important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.

Approximately 50% of brain tumors in children are infratentorial, with three fourths of these located in the cerebellum or fourth ventricle. Common infratentorial (posterior fossa) tumors include the following:

  1. Cerebellar astrocytoma (usually pilocytic but also fibrillary and, less frequently, high-grade).
  2. Medulloblastoma (primitive neuroectodermal tumor [PNET]).
  3. Ependymoma (cellular, papillary, clear cell, tanycytic or anaplastic).
  4. Brain stem glioma (often diagnosed neuroradiologically without biopsy; more frequently high-grade and may be low-grade).
  5. Atypical teratoid/rhabdoid tumors.

Supratentorial tumors include those tumors that occur in the sellar or suprasellar region and/or in the cerebrum or diencephalon. Sellar/suprasellar tumors comprise approximately 20% of childhood brain tumors and include the following:

  1. Craniopharyngioma.
  2. Diencephalic (central tumors involving the chiasm, hypothalamic, and/or thalamic) gliomas generally of low grade (including astrocytoma, grade 1 or 2).
  3. Germ cell tumors (germinoma and nongerminomatous).

Other tumors that occur supratentorially include the following:

  1. Low-grade cerebral hemispheric astrocytoma or glioma (grade 1 or grade 2).
  2. High-grade or malignant astrocytoma (anaplastic astrocytoma, glioblastoma multiforme [grade 3 or grade 4]).
  3. Mixed glioma (low-grade or high-grade).
  4. Oligodendroglioma (low-grade or high-grade).
  5. Primitive neuroectodermal tumor (including cerebral neuroblastoma, pineoblastoma, ependymoblastoma).
  6. Ependymoma (cellular or anaplastic).
  7. Meningioma.
  8. Choroid plexus tumors (papilloma and carcinoma).
  9. Pineal parenchymal tumors (pineocytoma, or mixed pineal parenchymal tumor).
  10. Neuronal and mixed neuronal glial tumor (ganglioglioma, desmoplastic infantile ganglioglioma, dysembryoplastic neuroepithelial tumor).
  11. Metastasis (rare) from extra neural malignancies.

Spinal cord tumors

Primary central nervous system spinal cord tumors comprise approximately 1% to 2% of all childhood nervous system tumors.1,2,3 As is the case for primary brain tumors, such lesions are histologically heterogeneous. Approximately 70% of all intramedullary spinal cord tumors will be low-grade astrocytomas and/or gangliogliomas. Other tumor types which occur include ependymomas, higher-grade glial tumors, and (rarely) primitive neuroectodermal tumors. Myxopapillary ependymomas have a tendency to develop in the conus and cauda equina regions. Symptoms and signs of spinal cord tumors are highly dependent on the location of the tumor and its extent; some low-grade spinal cord tumors are associated with large cysts that extend rostrally and caudally and have been termed holocord astrocytomas. At times, it is impossible to distinguish a tumor which arises in the medulla from a tumor which arises in the upper cervical cord.

Important general concepts that should be understood by those caring for a child who has a brain tumor include the following:

  1. Selection of an appropriate therapy can only occur if the correct diagnosis is made and the stage of the disease is accurately determined.
  2. Children with primary brain tumors represent a major therapy challenge that, for optimal results, requires the coordinated efforts of pediatric specialists in fields such as neurosurgery, neurology, rehabilitation, neuropathology, radiation oncology, pediatric oncology, neuro-oncology, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.4,5,6,
  3. More than one half of children diagnosed with brain tumors will survive 5 years from diagnosis. In some subgroups of patients, an even higher rate of survival and cure is possible. Each child’s treatment should be approached with curative intent, and the possible long-term sequelae of the disease and its treatment should be considered before therapy is begun.
  4. For most childhood brain tumors, the optimal treatment regimen has not been determined. Children who have brain 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.
  5. 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.7,
  6. The cause of most childhood brain tumors remains unknown.8,9,

This summary discusses the treatment of childhood brain tumors.

Information about ongoing clinical trials is available from the NCI Cancer.gov Web site.



1 Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.

2 Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.

3 Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.

4 Strother DR, Poplack IF, Fisher PG, et al.: Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins, 2002, pp 751-824.

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

6 Cohen ME, Duffner PK, eds.: Brain Tumors in Children: Principles of Diagnosis and Treatment. 2nd ed. New York: Raven Press, 1994.

7 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.

8 Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiol Biomarkers Prev 2 (3): 277-88, 1993 May-Jun.

9 Kuijten RR, Strom SS, Rorke LB, et al.: Family history of cancer and seizures in young children with brain tumors: a report from the Childrens Cancer Group (United States and Canada). Cancer Causes Control 4 (5): 455-64, 1993.

Cellular Classification

The classification of brain tumors is based on both histopathological characteristics and location in the brain.1 Undifferentiated neuroectodermal tumors of the cerebellum have historically been referred to as medulloblastomas, while tumors of identical histology in the pineal region would be diagnosed as pineoblastomas.1,2 The nomenclature of pediatric brain tumors is controversial and potentially confusing. Some pathologists advocate abandoning the traditional morphologically-based classifications such as medulloblastoma in favor of a terminology that relies more extensively on the phenotypic characteristics of the tumor.3 In such a system, medulloblastoma is referred to as primitive neuroectodermal tumor (PNET) and then subdivided on the basis of cellular differentiation. The most recent World Health Organization classification of brain tumors maintains the term medulloblastoma for posterior fossa undifferentiated tumors. It also maintains separate categories for supratentorial primitive neuroectodermal tumors, ependymoblastomas, and pineal small round cell tumors (pineoblastomas). The pathologic classification of pediatric brain tumors is a specialized area that is evolving; review of the diagnostic tissue by a neuropathologist who has particular expertise in this area is strongly recommended.

Spinal cord 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.4,5,6,



1 Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000.

2 Burger PC, Sheithauer BW, Vogel FS: Surgical pathology of the nervous system and its coverings. 3rd ed. New York, NY: Churchill Livingstone, 1991.

3 Rorke LB, Gilles FH, Davis RL, et al.: Revision of the World Health Organization classification of brain tumors for childhood brain tumors. Cancer 56 (7 Suppl): 1869-86, 1985.

4 Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.

5 Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.

6 Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.

Stage Information

Medulloblastoma

Refer to the PDQ summary on Childhood Medulloblastoma Treatment for more information.

Cerebellar astrocytoma

Refer to the PDQ summary on Childhood Cerebellar Astrocytoma Treatment for more information.

Ependymoma

Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.

Brain stem glioma

Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.

Cerebral astrocytoma

Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information.

Craniopharyngioma

These are symptomatic benign tumors arising from remnants of Rathke’s pouch. There is no generally accepted staging system and metastasis is rare.1,2,3,4,

Central nervous system germ cell tumor

Germ cell brain tumors usually arise in the pineal or suprasellar regions. Histologic subtypes include teratoma (both mature and immature), germinoma, choriocarcinomas, and nongerminomatous germ cell tumors (i.e., embryonal cell carcinoma, yolk cell or endodermal sinus tumor, and mixed germ cell tumors). These tumors have a propensity for subarachnoid spread. Every patient with a germinoma or malignant germ cell tumor should be evaluated with diagnostic imaging of the spinal cord and whole brain. The best method for evaluating spinal cord subarachnoid metastasis is MRI with gadolinium enhancement. Cerebrospinal fluid should be examined cytologically and levels of alpha-fetoprotein (AFP), and human chorionic gonadotropin (HCG) determined. AFP and/or HCG may be elevated in the serum of such patients. Prognosis is related to histology; patients with pure germinoma have a more favorable outcome than those with nongerminomatous germ cell tumors (nongerminomas).5,6,

Pineal parenchymal tumors

Pineoblastoma, pineocytoma. (Refer to the PDQ summary on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment for more information.)

Supratentorial primitive neuroectodermal tumor

PNETand cerebral neuroblastoma. (Refer to the PDQ summary on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment for more information.)

Visual pathway and hypothalamic glioma

Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic Glioma Treatment for more information.

Spinal cord tumors

There is no uniformly accepted staging system for childhood primary spinal cord tumors. These tumors are classified based on their location within the spinal cord and histology. Low-grade spinal cord tumors rarely disseminate elsewhere in the nervous system; however, higher grade tumors may disseminate.7,8 Despite this, because of the location of the tumor and concerns over causing further neurologic deterioration by cerebrospinal fluid attainment, routine lumbar spinal punctures are not indicated in the evaluation of a child with a spinal cord tumor. For high-grade glial spinal cord tumors, and possibly lower grade tumors and ependymomas, neuroimaging of the entire neuroaxis (brain and entire spine) is indicated at the time of diagnosis for determination of extent of disease.



1 Lapras C, Patet JD, Mottolese C, et al.: Craniopharyngiomas in childhood: analysis of 42 cases. Prog Exp Tumor Res 30: 350-8, 1987.

2 Habrand JL, Ganry O, Couanet D, et al.: The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature. Int J Radiat Oncol Biol Phys 44 (2): 255-63, 1999.

3 Yasargil MG, Curcic M, Kis M, et al.: Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J Neurosurg 73 (1): 3-11, 1990.

4 Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46 (2): 291-302; discussion 302-5, 2000.

5 Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.

6 Balmaceda C, Modak S, Finlay J: Central nervous system germ cell tumors. Semin Oncol 25 (2): 243-50, 1998.

7 Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.

8 Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.

Treatment Option Overview

Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best accepted therapy available. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those previously obtained with existing therapy.

Because of the relative rarity of cancer in children, all patients with brain tumors should be considered for entry into a clinical trial. To determine and implement optimum treatment, treatment planning by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required. Radiation therapy of pediatric brain tumors is technically very demanding and should be carried out in centers that have experience in that area in order to ensure optimal results.

Debilitating effects on growth and neurologic development have frequently been observed following radiation therapy, especially in younger children.1,2,3 Secondary tumors have increasingly been diagnosed in long-term survivors.4 For this reason, the role of chemotherapy in allowing a delay or reduction in the administration of radiation therapy is under study, and preliminary results suggest that chemotherapy can be used to delay, limit, and sometimes obviate, the need for radiation therapy in children with benign and malignant lesions.5,6,7 Long-term management of these patients is complex and requires a multidisciplinary approach.

The designations in PDQ that treatments are “standard” or “under clinical evaluation” are not to be used as a basis for reimbursement determinations.



1 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.

2 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.

3 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.

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

5 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.

6 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.

7 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.

Childhood Medulloblastoma

Refer to the PDQ summary on Childhood Medulloblastoma Treatment for more information.

Childhood Cerebellar Astrocytoma

Refer to the PDQ summary on Childhood Cerebellar Astrocytoma Treatment for more information.

Childhood Infratentorial Ependymoma

Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.

Childhood Brain Stem Glioma

Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.

Childhood Cerebral Astrocytoma

Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information.

Childhood Supratentorial Ependymoma

Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.

Childhood Craniopharyngioma

Treatment options

Therapies for craniopharyngioma include surgery and conventional external radiation therapy, and in selected cases, stereotactic radiosurgery or intracavitary irradiation. In general, each of these modalities, either alone or in combination, can give a high rate of long-term disease control in the majority of patients. Debate centers on the relative morbidity of the different approaches.1,2,3,4,5,6,7 Treatment of cystic tumors with intracavitary chemotherapy has also been reported.8,



1 Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46 (2): 291-302; discussion 302-5, 2000.

2 Regine WF, Kramer S: Pediatric craniopharyngiomas: long term results of combined treatment with surgery and radiation. Int J Radiat Oncol Biol Phys 24 (4): 611-7, 1992.

3 Hetelekidis S, Barnes PD, Tao ML, et al.: 20-year experience in childhood craniopharyngioma. Int J Radiat Oncol Biol Phys 27 (2): 189-95, 1993.

4 Backlund EO, Axelsson B, Bergstrand CG, et al.: Treatment of craniopharyngiomas--the stereotactic approach in a ten to twenty-three years' perspective. I. Surgical, radiological and ophthalmological aspects. Acta Neurochir (Wien) 99 (1-2): 11-9, 1989.

5 Pollock BE, Lunsford LD, Kondziolka D, et al.: Phosphorus-32 intracavitary irradiation of cystic craniopharyngiomas: current technique and long-term results. Int J Radiat Oncol Biol Phys 33 (2): 437-46, 1995.

6 Van Effenterre R, Boch AL: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97 (1): 3-11, 2002.

7 De Vile CJ, Grant DB, Kendall BE, et al.: Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg 85 (1): 73-81, 1996.

8 Takahashi H, Nakazawa S, Shimura T: Evaluation of postoperative intratumoral injection of bleomycin for craniopharyngioma in children. J Neurosurg 62 (1): 120-7, 1985.

Childhood Central Nervous System Germ Cell Tumor

Treatment options

Surgery other than biopsy to establish the diagnosis rarely plays a role in the treatment of central nervous system (CNS) germinomas. The role of surgical resection for nongerminomatous germ cell tumors and teratomas remains to be defined.1 For germinomas, irradiation with doses of 4,500 cGy to 5,400 cGy to the tumor and 2,150 cGy to 3,600 cGy to the whole brain and spine is usually curative. In selected cases, germinoma can be effectively treated with ventricular field radiation therapy and at lower dose levels (3,000 - 3,600 cGy) following response to chemotherapy.1 Although experience with pre-irradiation chemotherapy has shown that most of these tumors respond to cyclophosphamide and platinum-containing drugs, the definitive role of chemotherapy has yet to be determined.1 Disseminated germinoma is treated with craniospinal irradiation,2 3 alone or in combination with chemotherapy. The usual dose to the tumor is 4,500 cGy to 5,400 cGy with 2,700 cGy to 3,600 cGy to the whole brain and spine. Although nongerminomatous germ cell tumors, such as embryonal carcinoma, yolk cell tumor, and mixed germ cell tumors, may respond to chemotherapeutic agents such as cisplatin or carboplatin, etoposide, cyclophosphamide, and vinblastine, as do such histologies outside of the CNS, optimal combination of agents, and the timing of chemotherapy in relation to radiation therapy remains to be determined.4,5,6 Information about ongoing clinical trials is available from the NCI Cancer.gov Web site.



1 Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.

2 Dearnaley DP, A'Hern RP, Whittaker S, et al.: Pineal and CNS germ cell tumors: Royal Marsden Hospital experience 1962-1987. Int J Radiat Oncol Biol Phys 18 (4): 773-81, 1990.

3 Linstadt D, Wara WM, Edwards MS, et al.: Radiotherapy of primary intracranial germinomas: the case against routine craniospinal irradiation. Int J Radiat Oncol Biol Phys 15 (2): 291-7, 1988.

4 Balmaceda C, Heller G, Rosenblum M, et al.: Chemotherapy without irradiation--a novel approach for newly diagnosed CNS germ cell tumors: results of an international cooperative trial. The First International Central Nervous System Germ Cell Tumor Study. J Clin Oncol 14 (11): 2908-15, 1996.

5 Bouffet E, Baranzelli MC, Patte C, et al.: Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience. Société Française d'Oncologie Pédiatrique. Br J Cancer 79 (7-8): 1199-204, 1999.

6 Robertson PL, DaRosso RC, Allen JC: Improved prognosis of intracranial non-germinoma germ cell tumors with multimodality therapy. J Neurooncol 32 (1): 71-80, 1997.

Childhood Visual Pathway and Hypothalamic Glioma

Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic Glioma Treatment for more information.

Childhood Spinal Cord Tumors

The optimal treatment for intrinsic/intramedullary glial spinal cord tumors has not been determined by prospective randomized trials. Therapeutic options include surgery alone, surgery plus local radiation therapy, and possibly adjuvant chemotherapy in selected cases.1,2 Extensive surgical resections are technically possible for many patients with intramedullary spinal cord tumors, but may result in worsening neurologic status in ≥10% of cases.1,2 Surgery is usually indicated to at least determine the type of tumor present; for low-grade glial tumors this may be the only treatment required.1 In one recent series of 164 children and young adults with intramedullary low-grade glial tumors or ganglioglial spinal cord tumors, 70% were controlled for 5 years after extensive surgical resections.1 Radiotherapy has been demonstrated to control disease in some patients with low-grade glial tumors after subtotal resections.2,3,4 The role of chemotherapy for spinal cord tumors is poorly characterized, but some very young children with low-grade glial tumors have been successfully treated with a carboplatin and vincristine drug regimen.5 Outcomes for patients with high-grade glial tumors has been extremely poor; most develop progressive disease within three years of treatment with surgery, radiation, and/or chemotherapy.1,3,4,

The optimal treatment for children with spinal ependymomas has not been well characterized and as is the case for glial tumors, treatment options predominantly consist of either surgery alone or surgery followed by local radiotherapy.1,2 Management of primitive neuroectodermal tumors of the spinal cord is also not well delineated, and most patients are treated on treatment protocols designed for children with high-risk medulloblastoma.



1 Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.

2 Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.

3 Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.

4 O'Sullivan C, Jenkin RD, Doherty MA, et al.: Spinal cord tumors in children: long-term results of combined surgical and radiation treatment. J Neurosurg 81 (4): 507-12, 1994.

5 Hassall TE, Mitchell AE, Ashley DM: Carboplatin chemotherapy for progressive intramedullary spinal cord low-grade gliomas in children: three case studies and a review of the literature. Neuro-oncol 3 (4): 251-7, 2001.

Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

Refer to the PDQ summary on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment for more information.

Recurrent Childhood Brain Tumor

Recurrence is not uncommon in both low-grade and malignant childhood brain tumors and may occur many years after initial treatment.1 Disease may occur at the primary tumor site or, especially in malignant tumors, at noncontiguous central nervous system 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, as other entities, such as secondary tumor and treatment-related brain necrosis, may be clinically indistinguishable from tumor recurrence. The need for surgical intervention must be individualized based on the initial tumor type, the length of time between initial treatment and the reappearance of the lesion, and the clinical picture.

Recurrent low-grade glial tumors

Surgical resection, radiation therapy (especially if not previously given), and chemotherapy may result in prolonged disease stabilization for children with recurrent low-grade tumors. Resection is an option for those patients with a surgically accessible lesion and has the advantage of documenting the histology of the recurrent tumor. Radiation therapy, if not previously given, may result in tumor shrinkage and long-term disease control. Chemotherapy with drugs such as carboplatin and vincristine has recently been shown to result in tumor shrinkage and disease control for children with low-grade glial neoplasms.2 Similar results have been demonstrated for hypothalamic and chiasmatic tumors treated with etoposide.3 Entry into phase I and phase II trials is indicated to identify more effective and less toxic agents.

Recurrent central nervous system germ cell tumors

Germ cell tumors may be chemoresponsive. Patients may benefit from the types of agents that are used in germ cell tumors in other locations, such as cisplatin, etoposide, or cyclophosphamide. Patients with recurrent germ cell tumors for whom the standard chemotherapy options have failed may be entered into phase I and phase II studies that are designed to determine the activity and toxic effects of agents new to the treatment of this tumor.

Recurrent central nervous system tumors in children younger than age 3

Studies have addressed the treatment of infants who have progressive disease in spite of chemotherapy. Approaches that have been used include further surgery, chemotherapy, local and/or craniospinal radiotherapy, high-dose chemotherapy supported by autologous stem cell rescue, or combinations of chemotherapy and radiotherapy. Overall salvage rates have been less than optimal, but a subgroup of children, primarily those with localized disease at the time of relapse, may experience prolonged disease control and possible “cure” with treatment after recurrence.4 5,6,7,8,9 For children younger than 3 years, the use of high-dose craniospinal irradiation has been associated with poor neurocognitive outcome. Treatment for young children with multiple recurrent and/or disseminated brain tumors is even more problematic and entry into phase I and II trials is indicated to identify more effective and less toxic agents.

Recurrent Spinal Cord Tumors

At the time of recurrence, recurrent low-grade spinal cord glial tumors can be treated with re-resection with or without the use of radiation therapy. Recurrent low-grade and high-grade tumors which cannot be re-resected can be treated on protocols designed for histologically similar brain tumors.



1 Jenkin D, Greenberg M, Hoffman H, et al.: Brain tumors in children: long-term survival after radiation treatment. Int J Radiat Oncol Biol Phys 31 (3): 445-51, 1995.

2 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.

3 Chamberlain MC, Grafe MR: Recurrent chiasmatic-hypothalamic glioma treated with oral etoposide. J Clin Oncol 13 (8): 2072-6, 1995.

4 Fisher PG, Needle MN, Cnaan A, et al.: Salvage therapy after postoperative chemotherapy for primary brain tumors in infants and very young children. Cancer 83 (3): 566-74, 1998.

5 Walter AW, Mulhern RK, Gajjar A, et al.: Survival and neurodevelopmental outcome of young children with medulloblastoma at St Jude Children's Research Hospital. J Clin Oncol 17 (12): 3720-8, 1999.

6 Goldwein JW, Glauser TA, Packer RJ, et al.: Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66 (3): 557-63, 1990.

7 Dupuis-Girod S, Hartmann O, Benhamou E, et al.: Will high dose chemotherapy followed by autologous bone marrow transplantation supplant cranio-spinal irradiation in young children treated for medulloblastoma? J Neurooncol 27 (1): 87-98, 1996.

8 Dunkel IJ, Boyett JM, Yates A, et al.: High-dose carboplatin, thiotepa, and etoposide with autologous stem-cell rescue for patients with recurrent medulloblastoma. Children's Cancer Group. J Clin Oncol 16 (1): 222-8, 1998.

9 Guruangan S, Dunkel IJ, Goldman S, et al.: Myeloablative chemotherapy with autologous bone marrow rescue in young children with recurrent malignant brain tumors. J Clin Oncol 16 (7): 2486-93, 1998.

Changes to This Summary (04/15/2004)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

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2004-04-15