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Important: This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

Childhood brain tumor


Table of Contents

GENERAL INFORMATION
CELLULAR CLASSIFICATION
STAGE INFORMATION
Medulloblastoma
Cerebellar astrocytoma
Infratentorial ependymoma
Brain stem glioma
Cerebral astrocytoma
Supratentorial ependymoma
Craniopharyngioma
Central nervous system germ cell tumor
Pineal parenchymal tumors
Supratentorial primitive neuroectodermal tumor
Visual pathway and hypothalamic glioma
TREATMENT OPTION OVERVIEW
CHILDHOOD MEDULLOBLASTOMA
CHILDHOOD CEREBELLAR ASTROCYTOMA
CHILDHOOD INFRATENTORIAL EPENDYMOMA
CHILDHOOD BRAIN STEM GLIOMA
CHILDHOOD CEREBRAL ASTROCYTOMA
CHILDHOOD SUPRATENTORIAL EPENDYMOMA
CHILDHOOD CRANIOPHARYNGIOMA
CHILDHOOD CENTRAL NERVOUS SYSTEM GERM CELL TUMOR
CHILDHOOD VISUAL PATHWAY AND HYPOTHALAMIC GLIOMA
CHILDHOOD SUPRATENTORIAL PRIMITIVE NEUROECTODERMAL AND PINEAL TUMORS
RECURRENT CHILDHOOD BRAIN TUMOR
Recurrent central nervous system tumors in children under age 3

GENERAL INFORMATION

Separate summaries containing information on childhood brain stem glioma, childhood cerebellar astrocytoma, childhood cerebral astrocytoma, childhood medulloblastoma, childhood supratentorial primitive neuroectodermal tumor (PNET, cerebral neuroblastoma, and pineoblastoma), and childhood visual pathway and hypothalamic glioma are available in PDQ.

This treatment information summary on childhood brain tumors is an overview of diagnosis, classification, patient treatment, and prognosis. The National Cancer Institute created the PDQ database to increase the availability of new treatment information and its use in treating patients. Information and references from the most recently published literature are included after review by 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 have recently been 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 occasionally invasive or high-grade)

2. medulloblastoma (primitive neuroectodermal tumor)

3. ependymoma

4. brain stem glioma (often diagnosed neuroradiologically without biopsy; may be high- or low-grade)

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 (chiasm, hypothalamic, and/or thalamic) gliomas generally of low grade

3. germinoma

Other tumors that occur supratentorially include the following:

1. low-grade 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

4. oligodendroglioma

5. primitive neuroectodermal tumor (cerebral neuroblastoma)

6. ependymoma

7. meningioma

8. choroid plexus tumors (papilloma and carcinoma)

9. pineal parenchymal tumors (pineoblastoma, pineocytoma, or mixed pineal parenchymal tumor)

10. neuronal and mixed neuronal glial tumor (ganglioglioma, desmoplastic infantile ganglioglioma, dysembryoplastic neuroepithelial tumor)

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, medical oncology, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.[1-3]

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 when therapy is begun.

4. For the majority of 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. In the United States, the two major cooperative groups are the Pediatric Oncology Group and the Childrens Cancer Group.

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.[4]

6. The cause of the vast majority of childhood brain tumors remains unknown.[5,6]

This summary discusses the treatment of childhood brain tumors.

For information on current clinical trials for children with brain tumors, consult the PDQ Protocol File.

References:

  1. Heideman RL, Packer RJ, Albright LA, et al.: Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. Philadelphia, PA: Lippincott-Raven, 3rd ed., 1997, pp 633-697.

  2. Pollack IF: Brain tumors in children. New England Journal of Medicine 331(22): 1500-1507, 1994.

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

  4. Sanders J, Glader B, Cairo M, et al.: 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): 139-141, 1997.

  5. Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiology, Biomarkers and Prevention 2(3): 277-288, 1993.

  6. 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 and Control 4(5): 455-464, 1993.


CELLULAR CLASSIFICATION

The classification of brain tumors is based on both histopathological characteristics and location in the brain. 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. 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. 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 primitive neuroectodermal tumors and for pineal small round cell tumors (pineoblastomas).[1] The clinical significance of such nomenclature is as yet unknown, and because the use of such nomenclature makes it difficult to relate a diagnosis to decades of accumulated clinical information,[2] this discussion is based on the traditional tumor classification. The newer systems will be cross referenced as possible.[3] The pathologic classification of pediatric brain tumors is a specialized area that is undergoing evolution; review of the diagnostic tissue by a neuropathologist who has particular expertise in this area is strongly recommended.

References:

  1. Kleihues P, Burger PC, Scheithauer BW, et al.: Histological typing of tumours of the central nervous system. Berlin: Springer-Verlag, 2nd ed., 1993.

  2. Burger PC, Sheithauer BW, Vogel FS: Surgical pathology of the nervous system and its coverings. New York: Churchill Livingstone, 3rd ed., 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): 1869-1886, 1985.


STAGE INFORMATION


Medulloblastoma

A separate summary containing information on childhood medulloblastoma is available in PDQ.


Cerebellar astrocytoma

A separate summary containing information on childhood cerebellar astrocytoma is available in PDQ.


Infratentorial ependymoma

Ependymomas arise from the ependymal lining of the ventricular system or central canal of the spinal cord and therefore may occur throughout the entire neuraxis. Seventy percent of ependymomas arise in the posterior fossa. Most arise in contiguity with the 4th ventricular surface. Spread via the cerebrospinal fluid (CSF) throughout the subarachnoid space is reported primarily with the higher grade tumors. Every patient with ependymoma should be evaluated with diagnostic imaging of the spinal cord and whole brain. The best method currently available for evaluating spinal cord subarachnoid metastasis is magnetic resonance imaging (MRI) with gadolinium enhancement. Cerebrospinal fluid should be examined cytologically and CSF glucose and protein determined. Prognosis depends on the extent of resection of the tumor and residual tumor following surgery.[1-4]


Brain stem glioma

A separate summary containing information on childhood brain stem glioma is available in PDQ.


Cerebral astrocytoma

A separate summary containing information on childhood cerebral astrocytoma is available in PDQ.


Supratentorial ependymoma

Thirty percent of ependymomas arise outside the posterior fossa. They are often found adjacent to the ventricular surface. There is no generally recognized staging system. Tumors with more anaplastic features (higher grade) and those within the ventricle have a greater tendency for subarachnoid spread. Every patient with anaplastic ependymoma 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 CSF glucose and protein determined.[4]


Craniopharyngioma

These are symptomatic benign tumors arising from remnants of Rathke's pouch. There is no generally accepted staging system and metastasis is rare.[5-7]


Central nervous system germ cell tumor

Germ cell brain tumors usually arise in the pineal or suprasellar regions. Histologic subtypes include teratoma, germinoma, and the more malignant tumors (i.e., embryonal cell carcinoma, choriocarcinoma, and endodermal sinus tumor). 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 CSF glucose, protein, alpha-fetoprotein (AFP), and human chorionic gonadotropin (HCG) determined. AFP and/or HCG may be elevated in the serum of such patients as well. Prognosis is related to histology; patients with germinoma are more likely to have a more favorable outcome than those with malignant germ cell tumors (nongerminomas).[8-12]


Pineal parenchymal tumors

(pineoblastoma, pineocytoma)
A separate summary containing information on childhood supratentorial primitive neuroectodermal tumors (PNET, cerebral neuroblastoma, and pineoblastoma) is available in PDQ.


Supratentorial primitive neuroectodermal tumor

(PNET, cerebral neuroblastoma)
A separate summary containing information on childhood supratentorial primitive neuroectodermal tumors (PNET, cerebral neuroblastoma, and pineoblastoma) is available in PDQ.


Visual pathway and hypothalamic glioma

A separate summary containing information on childhood visual pathway and hypothalamic glioma is available in PDQ.

References:

  1. Robertson PL, Zeltzer PM, Boyett JM, et al.: Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group. Journal of Neurosurgery 88(4): 695-703, 1998.

  2. Ernestus RI, Schroder R, Stutzer H, et al.: The clinical and prognostic relevance of grading in intracranial ependymomas. British Journal of Neurosurgery 11(5): 421-428, 1997.

  3. Pollack IF, Gerszten PC, Martinez AJ, et al.: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37(4): 655-666; discussion 666-667, 1995.

  4. Bouffet E, Perilongo G, Canete A, et al.: Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Medical and Pediatric Oncology 30(6): 319-329; discussion 329-331, 1998.

  5. Lapras C, Patet JD, Mottolese C: Craniopharyngiomas in childhood: analysis of 42 cases. Progress in Experimental Tumor Research 30: 350-358, 1987.

  6. Fischer EG, Welch K, Belli JA, et al.: Treatment of craniopharyngiomas in children: 1972-1981. Journal of Neurosurgery 62(4): 496-501, 1985.

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

  8. Jennings MT, Gelman R, Hochberg F: Intracranial germ-cell tumors: natural history and pathogenesis. Journal of Neurosurgery 63(2): 155-167, 1985.

  9. Packer RJ, Sutton LN, Rosenstock JG, et al.: Pineal region tumors of childhood. Pediatrics 74(1): 97-102, 1984.

  10. Neuwelt EA, Frenkel EP: Germinomas and other pineal tumors: chemotherapeutic responses. In: Neuwelt EA, Ed.: Diagnosis and Treatment of Pineal Region Tumors. Baltimore: Williams and Wilkins, 1984, pp 332-343.

  11. Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. Journal of Neurosurgery 86(3): 446-455, 1997.

  12. Balmaceda C, Modak S, Finlay J: Central nervous system germ cell tumors. Seminars in Oncology 25(2): 243-250, 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 available, accepted therapy. 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.[1-3] Secondary tumors have increasingly been diagnosed in long-term survivors.[4] For this reason, the role of chemotherapy in allowing a delay in the administration of radiation therapy is under study, and preliminary results suggest that chemotherapy can be used to delay, and sometimes obviate, the need for radiation therapy in children with benign and malignant lesions.[5-7] 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.

References:

  1. Packer RJ, Sutton LN, Atkins TE, et al.: A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. Journal of Neurosurgery 70(5): 707-713, 1989.

  2. Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. Journal of Neurosurgery 80(6): 1004-1010, 1994.

  3. Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. Journal of Neurosurgery 74(3): 433-440, 1991.

  4. Jenkin D: Long-term survival of children with brain tumors. Oncology 10(5): 715-719, 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. New England Journal of Medicine 328(24): 1725-1731, 1993.

  6. Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. Journal of Clinical Oncology 11(5): 850-856, 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. Journal of Clinical Oncology 16(1): 210-221, 1998.


CHILDHOOD MEDULLOBLASTOMA

A separate summary containing information on childhood medulloblastoma is available in PDQ.


CHILDHOOD CEREBELLAR ASTROCYTOMA

A separate summary containing information on childhood cerebellar astrocytoma is available in PDQ.


CHILDHOOD INFRATENTORIAL EPENDYMOMA

Treatment options:

Radiation therapy with doses of 5,400 cGy to the posterior fossa is the usual treatment for patients with ependymoma.[1] Craniospinal irradiation is used only in cases of disseminated disease or, sometimes, in anaplastic lesions. Whole brain and spine doses of 3,600 cGy are common. Patients whose tumors are grossly totally resected have a more favorable outcome.[1] There is no defined benefit for adjuvant chemotherapy in the treatment of ependymoma. Children less than 3 years of age may benefit from chemotherapy to delay, modify, or possibly obviate the need for radiation therapy.[2,3]

References:

  1. Robertson PL, Zeltzer PM, Boyett JM, et al.: Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group. Journal of Neurosurgery 88(4): 695-703, 1998.

  2. 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. New England Journal of Medicine 328(24): 1725-1731, 1993.

  3. Mason WP, Grovas A, Halpern S, et al.: Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. Journal of Clinical Oncology 16(1): 210-221, 1998.


CHILDHOOD BRAIN STEM GLIOMA

A separate summary containing information on childhood brain stem glioma is available in PDQ.


CHILDHOOD CEREBRAL ASTROCYTOMA

A separate summary containing information on childhood cerebral astrocytoma is available in PDQ.


CHILDHOOD SUPRATENTORIAL EPENDYMOMA

Treatment options:

Therapy includes surgery followed by radiation therapy to the primary tumor in low-grade tumors, or to the entire craniospinal axis in high-grade tumors or those located adjacent to the ventricular lining.[1] A role for adjuvant chemotherapy remains under clinical evaluation.[2] Children less than 3 years of age may benefit from chemotherapy to delay or modify radiation therapy.[3] Clinical trials evaluating radiation therapy with or without chemotherapy are ongoing. Consult the PDQ Protocol File for a listing of ongoing clinical trials.

References:

  1. Goldwein JW, Leahy JM, Packer RJ, et al.: Intracranial ependymomas in children. International Journal of Radiation Oncology, Biology, Physics 19(6): 1497-1502, 1990.

  2. Needle MN, Goldwein JW, Grass J, et al.: Adjuvant chemotherapy for the treatment of intracranial ependymoma of childhood. Cancer 80(2): 341-347, 1997.

  3. 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. New England Journal of Medicine 328(24): 1725-1731, 1993.


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.[1-5] Treatment of cystic tumors with intracavitary chemotherapy has also been reported.[6]

References:

  1. Fischer EG, Welch K, Belli JA, et al.: Treatment of craniopharyngiomas in children: 1972-1981. Journal of Neurosurgery 62(4): 496-501, 1985.

  2. Regine WF, Kramer S: Pediatric craniopharyngiomas: long term results of combined treatment with surgery and radiation. International Journal of Radiation Oncology, Biology, Physics 24(4): 611-617, 1992.

  3. Hetelekidis S, Barnes PD, Tao ML, et al.: 20-year experience in childhood craniopharyngioma. International Journal of Radiation Oncology, Biology, Physics 27(2): 189-195, 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 Neurochirurgica (Wien) 99(1-2): 11-19, 1989.

  5. Pollock BE, Lunsford LD, Kondziolka D, et al.: Phosphorus-32 intracavitary irradiation of cystic craniopharyngiomas: current technique and long-term results. International Journal of Radiation Oncology, Biology, Physics 33(2): 437-446, 1995.

  6. Takahashi H, Nakazawa S, Shimura T: Evaluation of postoperative intratumoral injection of bleomycin for craniopharyngioma in children. Journal of Neurosurgery 62(1): 120-127, 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 5,000-5,500 cGy to the tumor and 2,340-3,600 cGy to the whole brain and spine is usually curative. In selected cases, germinoma can be effectively treated with local radiation therapy or pre-irradiation chemotherapy.[1] Although experience with pre-irradiation chemotherapy has shown that the majority of these tumors respond to cyclophosphamide, the definitive role of chemotherapy has yet to be determined.[1] Disseminated germinoma and nongerminoma germ cell tumors are often treated with craniospinal irradiation,[2,3] although not all authors agree.[4] The usual dose to the tumor is 5,400 cGy with 3,500-4,000 cGy to the whole brain and spine, although it has been suggested that 4,500 cGy to the tumor and 3,000 cGy to the meninges may be adequate.[5,6] Although nongerminoma germ cell tumors, such as embryonal carcinoma, endodermal sinus tumor, and choriocarcinoma, may respond to chemotherapeutic agents such as bleomycin, cisplatin, etoposide, cyclophosphamide, and vincristine, as do such histologies outside of the CNS, the role for chemotherapy as adjuvant therapy in addition to radiation therapy remains to be determined.[7] Consult the PDQ Protocol File for a listing of ongoing clinical trials.

References:

  1. Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. Journal of Neurosurgery 86(3): 446-455, 1997.

  2. Edwards MS, Hudgins RJ, Wilson CB, et al.: Pineal region tumors in children. Journal of Neurosurgery 68(5): 689-697, 1988.

  3. Dearnaley DP, A'Hern RP, Whittaker S, et al.: Pineal and central nervous system germ cell tumors: Royal Marsden Hospital experience 1962-1987. International Journal of Radiation Oncology, Biology, Physics 18(4): 773-781, 1990.

  4. Lindstadt D, Wara WM, Edwards MS, et al.: Radiotherapy of primary intracranial germinomas: the case against routine craniospinal irradiation. International Journal of Radiation Oncology, Biology, Physics 15(2): 291-297, 1988.

  5. Fields JN, Fulling KH, Thomas PR, et al.: Suprasellar germinoma: radiation therapy. Radiology 164(1): 247-249, 1987.

  6. Dattoli MJ, Newall J: Radiation therapy for intracranial germinoma: the case for limited volume treatment. International Journal of Radiation Oncology, Biology, Physics 19(2): 429-433, 1990.

  7. Jennings MT, Gelman R, Hochberg F: Intracranial germ-cell tumors: natural history and pathogenesis. Journal of Neurosurgery 63(2): 155-167, 1985.


CHILDHOOD VISUAL PATHWAY AND HYPOTHALAMIC GLIOMA

A separate summary containing information on childhood visual pathway and hypothalamic glioma is available in PDQ.


CHILDHOOD SUPRATENTORIAL PRIMITIVE NEUROECTODERMAL AND PINEAL TUMORS

A separate summary containing information on childhood supratentorial primitive neuroectodermal tumors (PNET, cerebral neuroblastoma, and pineoblastoma) is available in PDQ.


RECURRENT CHILDHOOD BRAIN TUMOR

Recurrence is not uncommon in both benign 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 more benign 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 relatively 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 regimens that are used in germ cell tumors in other locations, such as PVB (cisplatin, vinblastine, bleomycin) and VAC (vincristine, dactinomycin, 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 under age 3

Two studies have addressed the treatment of infants who have progressive disease in spite of chemotherapy. One paper describes the treatment of 40 children with multiple tumor types who had progressive disease during or following chemotherapy.[4] Two year progression-free survival was 29%, regardless of the modality or combination of modalities employed. A second study of 13 children with either progressive or residual medulloblastoma describes the use of radiation therapy alone.[5] Four of 11 patients with progressive disease following chemotherapy survived following radiation therapy. These data suggest that radiation therapy is effective in some children with progressive tumors following chemotherapy, and could also be considered part of first-line therapy, if it could be administered safely.

References:

  1. Jenkin D, Greenberg M, Hoffman H, et al.: Brain tumors in children: long-term survival after radiation treatment. International Journal of Radiation Oncology, Biology, Physics 31(3): 445-451, 1995.

  2. Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. Journal of Clinical Oncology 11(5): 850-856, 1993.

  3. Chamberlain MC, Grafe MR: Recurrent chiasmatic-hypothalamic glioma treated with oral etoposide. Journal of Clinical Oncology 13(8): 2072-2076, 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): 566-574, 1998.

  5. Gajjar A, Mulhern RK, Heideman RL, et al.: Medulloblastoma in very young children: outcome of definitive craniospinal irradiation following incomplete response to chemotherapy. Journal of Clinical Oncology 12(6): 1212-1216, 1994.

  6. Goldwein JW, Glauser TA, Packer RJ, et al.: Recurrent intracranial ependymomas in children: survival, patterns of failure, and prognostic factors. Cancer 66(3): 557-563, 1990.

  7. Khan AB, D'Souza BJ, Wharam MD, et al.: Cisplatin therapy in recurrent childhood brain tumors. Cancer Treatment Reports 66(12): 2013-2020, 1982.

  8. Walker RW, Allen JC: Cisplatin in the treatment of recurrent childhood primary brain tumors. Journal of Clinical Oncology 6(1): 62-66, 1988.

Date Last Modified: 08/1999



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