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Childhood Brain Tumor

Posted by About Cancer Labels: Cancer Types

Table of Contents

General Information
Cellular Classification
Stage Information
Medulloblastoma
Cerebellar astrocytoma
Ependymoma
Brain stem glioma
Cerebral astrocytoma
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

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 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 high-grade)
2. medulloblastoma (primitive neuroectodermal tumor)
3. ependymoma (low-grade or anaplastic)
4. brain stem glioma (often diagnosed neuroradiologically without biopsy; may be high-grade or low-grade)
5. atypical teratoid

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. germ cell tumors (germinoma and nongerminomatous)

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 (low-grade or high-grade)
4. oligodendroglioma (low-grade or high-grade)
5. primitive neuroectodermal tumor (cerebral neuroblastoma)
6. ependymoma (low-grade or anaplastic)
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)
11. metastasis (rare) from extra neural malignancies

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 before 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.
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

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.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 primitive neuroectodermal tumors, ependymoblastomas, and pineal small round cell tumors (pineoblastomas). 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, 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. 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

Cerebellar astrocytoma

Ependymoma

Brain stem glioma

Cerebral astrocytoma

Craniopharyngioma

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

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 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 germinoma have a more favorable outcome than those with nongerminomatous germ cell tumors (nongerminomas).4-8

Pineal parenchymal tumors

(pineoblastoma, pineocytoma)

Supratentorial primitive neuroectodermal tumor

(PNET, cerebral neuroblastoma)

Visual pathway and hypothalamic glioma

References:

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

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

3. 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.

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

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

6. 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.

7. 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.

8. 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.

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 (Huntington NY) 10(5): 715-719, (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. 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

Childhood Cerebellar Astrocytoma

Childhood Infratentorial Ependymoma

Childhood Brain Stem Glioma

Childhood Cerebral Astrocytoma

Childhood Supratentorial Ependymoma

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 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 5000-5500 cGy to the tumor and 2340-3600 cGy to the whole brain and spine is usually curative. In selected cases, germinoma can be effectively treated with local radiation therapy and pre-irradiation chemotherapy.1 Although experience with pre-irradiation chemotherapy has shown that the majority of these tumors respond to cyclophosphamide and platinum-containing drugs, 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 5400 cGy with 3500-4000 cGy to the whole brain and spine, although it has been suggested that 4500 cGy to the tumor and 3000 cGy to the meninges may be adequate.5,6 Although nongerminomatous germ cell tumors, such as embryonal carcinoma, yolk cell tumor, and mixed germ cell tumors, may respond to chemotherapeutic agents such as bleomycin, cisplatin, etoposide, cyclophosphamide, and vinblastine, 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

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

Childhood Supratentorial Primitive Neuroectodermal and Pineal Tumors

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 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 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

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.4-9 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.

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. 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? Journal of Neuro-Oncology 27(1): 87-98, 1996.

8. Dunkel IJ, Boyett JM, et al, for the Children's Cancer Group: High-dose carboplatin, thiotepa, and etoposide with autologous stem-cell rescue for patients with recurrent medulloblastoma. Journal of Clinical Oncology 16(1): 222-228, 1998.

9. Guruangan S, Dunkel IJ, Goldman S, et al.: Myeloablative chemotherapy with autologous bone marrow rescue in young children with recurrent malignant brain tumor. Journal of Clinical Oncology 16(7): 2486-2493, 1998.

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Childhood Cerebellar Astrocytoma

Posted by About Cancer Labels: Cancer Types

Table of Contents

General Information
Cellular Classification
Stage Information
Treatment Option Overview
Untreated Childhood Cerebellar Astrocytoma
Recurrent Childhood Cerebellar Astrocytoma

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Childhood Brain Stem Glioma

Posted by About Cancer Labels: Cancer Types

Table of Contents

General Information
Cellular Classification
Stage Information
Treatment Option Overview
Untreated Childhood Brain Stem Glioma
Diffuse intrinsic brain stem gliomas
Focal or low-grade brain stem gliomas
Neurofibromatosis
Recurrent Childhood Brain Stem Glioma

General Information

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 high-grade)
2. medulloblastoma (primitive neuroectodermal tumor)
3. ependymoma (low-grade or anaplastic)
4. brain stem glioma (often diagnosed neuroradiographically without biopsy; may be high-grade or low-grade)
5. atypical teratoid

Supratentorial tumors include those tumors that occur in the sellar or suprasellar region and/or other areas of the cerebrum. 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. germ cell tumors (germinoma and nongerminomatous)

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, glioblastomas 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 (cerebral neuroblastoma)
6. ependymoma (low-grade or anaplastic)
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)
11. metastasis (rare) from extra neural malignancies

Important general concepts that should be understood by those caring for a child with 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 before 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 clinical trials 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.4
6. The cause of the vast majority of childhood brain tumors remains unknown.5,6

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

Brain stem gliomas are classified according to their location, extent of spread, radiographic appearance, and histology. Brain stem gliomas may occur in the pons, the midbrain, the tectum, the dorsum of the medulla at the cervicomedullary junction, or in multiple regions of the brain stem. The tumor may contiguously involve the cerebellar peduncles, cerebellum and/or thalamus. The majority of childhood brain stem gliomas are diffuse, intrinsic tumors that involve the pons, often with contiguous involvement of other brain stem sites.1-4 Another prognostically more favorable subset is focal pilocytic astrocytomas. These most frequently arise in the tectum of the midbrain, focally, within the pons, or the cervicomedullary junction, and have a far better prognosis than diffuse intrinsic tumors.2,3,5-7

Primary tumors of the brain stem are often diagnosed based on clinical findings and on neuroimaging studies,8 and there is a substantial amount of histologic variability within an individual tumor. Histologic confirmation is usually unnecessary in diffuse, intrinsic tumors and is not obtained unless the diagnosis is in doubt. The majority of diffuse, intrinsic tumors are fibrillary or malignant gliomas. Biopsy is almost never indicated for diffuse intrinsic tumors involving the pons unless the diagnosis is in doubt. Biopsy specimens of intrinsic brain stem gliomas may be misleading because of sampling error. Biopsy may be indicated for brain stem tumors that are not diffuse and intrinsic. New approaches with stereotactic needle biopsy may make biopsy safer.9

References:

1. Cohen ME, Duffner PK, Heffner RR, et al.: Prognostic factors in brainstem gliomas. Neurology 36(5): 602-605, 1986.

2. Albright AL, Guthkelch AN, Packer RJ, et al.: Prognostic factors in pediatric brain-stem gliomas. Journal of Neurosurgery 65(6): 751-755, 1986.

3. Halperin EC, Wehn SM, Scott JW, et al.: Selection of a management strategy for pediatric brainstem tumors. Medical and Pediatric Oncology 17(2): 116-125, 1989.

4. Freeman CR, Farmer JP: Pediatric brain stem gliomas: a review. International Journal of Radiation Oncology, Biology, Physics 40(2): 265-271, 1998.

5. Epstein F, McCleary EL: Intrinsic brain-stem tumors of childhood: surgical indications. Journal of Neurosurgery 64(1): 11-15, 1986.

6. Edwards MS, Wara WM, Ciricillo SF, et al.: Focal brain-stem astrocytomas causing symptoms of involvement of the facial nerve nucleus: long-term survival in six pediatric cases. Journal of Neurosurgery 80(1): 20-25, 1994.

7. Pollack IF, Pang D, Albright AL, et al.: The long-term outcome in children with late-onset aqueductal stenosis resulting from benign intrinsic tectal tumors. Journal of Neurosurgery 80(4): 681-688, 1994.

8. Albright AL, Packer RJ, Zimmerman R, et al.: Magnetic resonance scans should replace biopsies for the diagnosis of diffuse brain stem gliomas: a report from the Children's Cancer Group. Neurosurgery 33(6): 1026-1029, 1993.

9. Cartmill M, Punt J: Diffuse brain stem glioma. A review of stereotactic biopsies. Child's Nervous System 15(5): 235-237, 1999.

Stage Information

There is no generally applied staging system for childhood brain stem gliomas.1-3 It is uncommon for these tumors to have spread outside the brain stem itself at the time of initial diagnosis. Diffuse intrinsic tumors of the brain stem are associated with a very low likelihood of long-term survival. The less common tumors of the midbrain, especially in the tectal plate region, have been viewed separately from those of the brain stem because they are more likely to be low grade and to have a greater likelihood of long-term survival (approximately 80% 5-year progression-free survival versus less than 20% for tumors of the pons and medulla).1-8 Similarly, dorsally exophytic and cervicomedullary tumors may have a better prognosis than diffuse pontine gliomas. Spread of malignant brain stem tumors is usually contiguous; metastasis via the subarachnoid space has been reported in up to 30% of cases diagnosed antemortem.9 Such dissemination may occur prior to local relapse but usually occurs simultaneously with or after local disease relapse.

References:

1. Cohen ME, Duffner PK, Heffner RR, et al.: Prognostic factors in brainstem gliomas. Neurology 36(5): 602-605, 1986.

2. Albright AL, Guthkelch AN, Packer RJ, et al.: Prognostic factors in pediatric brain-stem gliomas. Journal of Neurosurgery 65(6): 751-755, 1986.

3. Freeman CR, Farmer JP: Pediatric brain stem gliomas: a review. International Journal of Radiation Oncology, Biology, Physics 40(2): 265-271, 1998.

4. Halperin EC, Wehn SM, Scott JW, et al.: Selection of a management strategy for pediatric brainstem tumors. Medical and Pediatric Oncology 17(2): 116-125, 1989.

5. Epstein F, McCleary EL: Intrinsic brain-stem tumors of childhood: surgical indications. Journal of Neurosurgery 64(1): 11-15, 1986.

6. Edwards MS, Wara WM, Ciricillo SF, et al.: Focal brain-stem astrocytomas causing symptoms of involvement of the facial nerve nucleus: long-term survival in six pediatric cases. Journal of Neurosurgery 80(1): 20-25, 1994.

7. Pollack IF, Pang D, Albright AL, et al.: The long-term outcome in children with late-onset aqueductal stenosis resulting from benign intrinsic tectal tumors. Journal of Neurosurgery 80(4): 681-688, 1994.

8. Mandell LR, Kadota R, Freeman C, et al.: There is no role for hyperfractionated radiotherapy in the management of children with newly diagnosed diffuse intrinsic brainstem tumors: results of a Pediatric Oncology Group phase III trial comparing conventional vs. hyperfractionated radiotherapy. International Journal of Radiation Oncology, Biology, Physics 43(5): 959-964, 1999.

9. Packer RJ, Allen J, Nielsen S, et al.: Brainstem glioma: clinical manifestations of meningeal gliomatosis. Annals of Neurology 14(2): 177-182, 1983.

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 that were 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 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.4,5 Long- term management of these patients is complex and requires a multidisciplinary approach.

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. 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.

5. 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.

Untreated Childhood Brain Stem Glioma

Diffuse intrinsic brain stem gliomas

Conventional treatment for children with diffuse intrinsic brain stem glioma is radiation therapy to involved areas. Such treatment will result in transient benefit for the majority of patients, but over 90% of patients will succumb to the disease within 18 months of diagnosis. The conventional dose of radiation therapy ranges between 5400 cGy and 6000 cGy given locally to the primary tumor site in single daily fractions.

Hyperfractionated (twice daily) radiation therapy techniques have been used to deliver a higher dose, and studies using doses as high as 7800 cGy have been completed. There is no evidence that these increased radiation therapy doses improve the duration or rate of survival for patients with diffuse and/or primary pontine tumors.1,2 Studies evaluating the efficacy of various radiosensitizers as a means for enhancing the therapeutic effect of this modality are under study but to date have failed to show significant improvement in outcome.2,3

The role of chemotherapy in the treatment of patients with newly diagnosed brain stem gliomas is limited.2,4,5 To date neither adjuvant or neoadjuvant chemotherapy nor immunotherapy when added to radiation therapy has been demonstrated to improve survival for children with diffuse intrinsic tumors. Studies using chemotherapy with radiation are ongoing. Children younger than 3 years of age with diffuse intrinsic tumors may benefit from chemotherapy to delay or modify radiation therapy.6

Focal or low-grade brain stem gliomas

Selected patients, primarily those with low-grade dorsally exophytic and focal tumors, may be treated surgically.7 Patients with extensive resection may be observed prior to the initiation of further therapy, preferably as part of a prospective clinical study.

Patients with small tectal lesions and hydrocephalus but no other neurological deficits may be treated with cerebrospinal fluid diversion and have follow-up with sequential neuroradiographic studies until there is evidence of progressive disease.7

Neurofibromatosis

Children with neurofibromatosis type I and brain stem gliomas may have a different prognosis than other patients who have intrinsic lesions. Patients with neurofibromatosis may present with a long history of symptoms or be identified on screening tests; a period of observation may be indicated before instituting any treatment.8 Brain stem gliomas in these children may be indolent and may require no specific treatment for years.9

References:

1. Freeman CR, Krischer JP, Sanford RA, et al.: Final results of a study of escalating doses of hyperfractionated radiotherapy in brain stem tumors in children: a Pediatric Oncology Group study. International Journal of Radiation Oncology, Biology, Physics 27(2): 197-206, 1993.

2. Mandell LR, Kadota R, Freeman C, et al.: There is no role for hyperfractionated radiotherapy in the management of children with newly diagnosed diffuse intrinsic brainstem tumors: results of a Pediatric Oncology Group phase III trial comparing conventional vs. hyperfractionated radiotherapy. International Journal of Radiation Oncology, Biology, Physics 43(5): 959-964, 1999.

3. Freeman CR, Kepner J, Kun LE, et al.: A detrimental effect of a combined chemotherapy-radiotherapy approach in children with diffuse intrinsic brain stem gliomas? International Journal of Radiation Oncology, Biology, Physics 47(3): 561-564, 2000.

4. Jenkin RD, Boesel C, Ertel I, et al.: Brain-stem tumors in childhood: a prospective randomized trial of irradiation with and without adjuvant CCNU, VCR, and prednisone. A report of the Children's Cancer Study Group. Journal of Neurosurgery 66(2): 227-233, 1987.

5. Blaney SM, Phillips PC, Packer RJ, et al.: Phase II evaluation of topotecan for pediatric central nervous system tumors. Cancer 78(3): 527-531, 1996.

6. 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.

7. Vandertop WP, Hoffman HJ, Drake JM, et al.: Focal midbrain tumors in children. Neurosurgery 31(2): 186-194, 1992.

8. Bilaniuk LT, Molloy PT, Zimmerman RA, et al.: Neurofibromatosis type 1: brain stem tumours. Neuroradiology 39(9): 642-653, 1997.

9. Molloy PT, Bilaniuk LT, Vaughan SN, et al.: Brainstem tumors in patients with neurofibromatosis type 1: a distinct clinical entity. Neurology 45(10): 1897-1902, 1995.

Recurrent Childhood Brain Stem Glioma

Recurrence may occur in both benign and malignant childhood brain stem gliomas and may develop many years after initial treatment. 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 the time of recurrence, a complete evaluation to determine the extent of the relapse is indicated for all malignant tumors and, selectively, for more benign lesions. Biopsy or surgical resection should be considered for confirmation of relapse when other entities such as secondary tumor and treatment-related brain necrosis which may be clinically indistinguishable from tumor recurrence are in the differential. This confirmation is usually not necessary in children with diffuse, intrinsic tumors. Other tests, such as positron-emission tomography plus single-photon emission computed tomography, have not yet been shown to be reliable in distinguishing necrosis from tumor recurrence in brain stem gliomas. The need for surgical intervention must be individualized on the basis of the initial tumor type, the length of time between initial treatment and the reappearance of the mass lesion, and the clinical picture.

Chemotherapy with agents such as a carboplatin and vincristine may be effective in children with low-grade, recurrent exophytic gliomas.1 Patients with recurrent diffuse, intrinsic brain stem glioma should be considered for entry into trials of novel therapeutic approaches because there are no "standard" agents that have demonstrated a high degree of activity. Alternatively, palliative care may be indicated for such individuals.

References:

1. 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.

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Adult Brain Tumor

Posted by About Cancer Labels: Cancer Types

Table of Contents

General Information
Cellular Classification
Stage Information
Noninfiltrating astrocytomas
Well-differentiated mildly and moderately anaplastic astrocytomas
Anaplastic astrocytomas
Glioblastoma multiforme
Well-differentiated ependymomas
Anaplastic ependymomas
Ependymoblastomas
Well-differentiated oligodendrogliomas
Anaplastic oligodendrogliomas
Meningiomas
Malignant meningiomas
Treatment Option Overview
Adult Noninfiltrating Astrocytoma
Juvenile pilocytic and subependymal astrocytomas 1
Adult Well-Differentiated Mildly and Moderately Anaplastic Astrocytoma
Adult Anaplastic Astrocytoma
Adult Glioblastoma Multiforme
Adult Brain Stem Glioma
Adult Well-Differentiated Ependymoma
Myxopapillary ependymoma and well-differentiated ependymoma
Adult Malignant Ependymoma
Anaplastic ependymoma and ependymoblastoma
Adult Well-Differentiated Oligodendroglioma
Adult Anaplastic Oligodendroglioma
Mixed Gliomas
Mixed astrocytoma-ependymoma, mixed astrocytoma-oligodendroglioma, and mixed astrocytoma-ependymoma-oligodendroglioma
Adult Medulloblastoma
Adult Pineal Parenchymal Tumor
Pineocytoma and pineoblastoma
Pineal astrocytoma
Adult Central Nervous System Germ Cell Tumor
Germinoma, embryonal carcinoma, choriocarcinoma, and teratoma
Adult Craniopharyngioma
Adult Meningioma
Adult Malignant Meningioma
Malignant meningioma, hemangiopericytoma, and papillary meningioma
Recurrent Adult Brain Tumor

General Information

Prognoses of primary brain tumors are determined by histologic type, grade, postoperative size, and extent of the tumor and by the patient's age, the performance status, and the duration of symptoms.1,2 Some primary brain tumors are curable by surgery alone, and some are curable by surgery and radiation therapy; the remainder require surgery, radiation therapy, and chemotherapy. Tumors that require all 3 modalities are infrequently curable.3

Metastases to the brain from a primary tumor that is outside the central nervous system (CNS) are more common than primary tumors of the brain. The most common primary tumors that metastasize to the brain are lung, breast, melanoma, and kidney. Metastases to the brain are usually multiple, but solitary metastases may also occur. Brain involvement can occur with cancers of the nasopharyngeal region by direct extension along the cranial nerves or through the foramina at the base of the skull. Metastatic meningeal involvement can also occur, especially with leukemia, lymphoma, small cell lung cancer, breast cancer, and some primary CNS tumors (such as medulloblastoma and ependymal gliomas).

A lesion in the brain should not be assumed to be a metastasis just because a patient has had a previous cancer; such an assumption could result in overlooking appropriate treatment of a curable tumor. Primary brain tumors rarely spread to other areas of the body, but they can spread to other parts of the brain and to the spinal axis.

References:

1. Levin VA, Leibel SA, Gutin PH: Neoplasms of the central nervous system. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. Philadelphia, Pa: Lippincott-Raven Publishers, 5th ed., 1997, pp 2022-2082.

2. Mahaley MS, Mettlin C, Natarajan N, et al.: National survey of patterns of care for brain-tumor patients. Journal of Neurosurgery 71(6): 826-836, 1989.

3. Surawicz TS, Davis F, Freels S, et al.: Brain tumor survival; results from the National Cancer Data Base. Journal of Neuro-Oncology 40(2): 151-160, 1998.

Cellular Classification

Histological classification for adult brain tumors is as follows:1

Glial tumors:

astrocytic tumors
#noninfiltrating
--juvenile pilocytic subependymal
#infiltrating
--well-differentiated mildly and moderately anaplastic astrocytoma anaplastic astrocytoma glioblastoma multiforme

#ependymal tumors
--myxopapillary and well-differentiated ependymoma anaplastic ependymoma ependymoblastoma
#oligodendroglial tumors
--well-differentiated oligodendroglioma anaplastic oligodendroglioma
#mixed tumors
--mixed astrocytoma-ependymoma mixed astrocytoma-oligodendroglioma mixed astrocytoma-ependymoma-oligodendroglioma
#medulloblastoma

Nonglial tumors:

#pineal parenchymal tumors
--pineocytoma pineoblastoma astrocytoma (see above)
#germ cell tumors
--germinoma embryonal carcinoma choriocarcinoma teratoma
#craniopharyngioma meningiomas
--meningioma malignant meningiomas
----anaplastic meningioma hemangiopericytoma papillary meningioma
#choroid plexus tumors
--choroid plexus papilloma anaplastic choroid plexus papilloma

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.

Stage Information

Brain tumors are classified on the basis of tumor cell type and histologic grade. For some tumors, location and metastatic spread within the cerebrospinal fluid are also used in classification.1

Cerebral Astrocytic Gliomas

Gliomas constitute the most common primary central nervous system (CNS) tumors. Of the gliomas, astrocytomas of variable malignancy are the most prevalent. Cerebral astrocytomas are subdivided into categories (grades) based on the degree of tumor anaplasia and the presence or absence of necrosis.

Noninfiltrating astrocytomas

These astrocytomas are relatively slow-growing tumors such as juvenile pilocytic and subependymal astrocytomas, which occur most frequently in children but can occur in adults.

Well-differentiated mildly and moderately anaplastic astrocytomas

These tumors are more infiltrative than the juvenile pilocytic and subependymal astrocytomas but are still relatively slow-growing tumors.

Anaplastic astrocytomas

These tumors are highly anaplastic with obvious vascular abnormalities. This grade III astrocytoma grows more rapidly than the more differentiated astrocytomas.

Glioblastoma multiforme

This grade IV astrocytoma is a poorly differentiated, rapidly growing tumor that occurs most often in adults.

Brain Stem Gliomas

Brain stem gliomas are usually diagnosed on clinical evidence because diagnosis by biopsy might be hazardous. Tumors that diffusely enlarge the brain stem carry a worse prognosis than those that are more focal. Higher grades of malignancy (see above) carry poorer prognoses as well.

Cerebellar Astrocytomas

Although the majority of these tumors are of lower grade and frequently are curable, they vary in grade of malignancy. The higher grade lesions carry a worse prognosis, but prognosis is generally better than for their cerebral counterparts.

Ependymal Tumors

Ependymal tumors are considered to arise from ependymal cells that line the ventricles and from ependymal rests. They vary in grade of malignancy.

Well-differentiated ependymomas

These tumors include myxopapillary ependymoma and well-differentiated ependymoma, and are often curable.

Anaplastic ependymomas

These tumors have more features of anaplasia and appear mitotically more active than the myxopapillary or well-differentiated ependymomas. Although previously considered to do worse than the well-differentiated ependymoma, conflicting evidence suggests that patients treated with surgery and radiation therapy might do nearly as well.

Ependymoblastomas

These are generally tumors of childhood and are considered by some to be primitive neuroectodermal tumors. They are rare.

Oligodendroglial Tumors

Oligodendroglial tumors are gliomas that arise from the oligodendroglia. They vary in grade of malignancy, and prognosis is related to grade.

Well-differentiated oligodendrogliomas

These tumors are usually slow-growing and well circumscribed.

Anaplastic oligodendrogliomas

These tumors are comparable to the highly anaplastic gliomas in prognosis.

Mixed Gliomas

Mixed gliomas can occur with combinations of generally 2, but sometimes 3, different cell types: astrocytoma, ependymoma, and/or oligodendroglioma. Survival statistics are inexact for this group because the cell types and grade of the most malignant-appearing cells influence prognosis. In general, these tumors carry a prognosis that is between the prognoses of well-differentiated and anaplastic astrocytomas.

Medulloblastoma

Medulloblastoma is a rapidly growing tumor arising in the posterior fossa and is found almost exclusively in children and young adults. It has the tendency to spread from the brain to the spinal axis. Prognosis is dependent on the staging following surgical resection.

Pineal Region Tumors

Pineal parenchymal tumors vary in histology and grade of malignancy relative to patient age at occurrence. They can vary from the slow-growing pineocytoma to the more malignant and faster growing pineoblastoma. Astrocytomas can also grow in this location (see above), as can a variety of primary germ cell tumors: germinoma, embryonal carcinoma, choriocarcinoma, and teratoma. These uncommon tumors vary in prognosis. The absence of biopsy specimens in many series make the prognosis for each tumor type difficult to evaluate.

Craniopharyngiomas

Craniopharyngioma is a tumor that arises from the remains of a structure found in the developing embryo in the region of the pituitary gland. This tumor causes symptoms and signs by pressing on vital areas of the brain and the optic nerves; it also causes internal hydrocephalus by obstructing the foramen of Monro in children.

Meningiomas

Meningiomas arise from the meninges surrounding the brain and spinal cord and are generally slow-growing. There are other variants that constitute a group called malignant meningioma and include malignant meningioma, hemangiopericytoma, papillary meningioma, and meningeal sarcoma. Malignant meningiomas are more likely than other meningiomas to metastasize within the craniospinal axis.

Meningiomas

Meningioma is usually curable with surgery if the initial resection is complete. The shape of the tumor is a prognostic factor and should be considered in planning surgery. Incomplete resection associated with lobulated and mushrooming patterns of tumor growth is associated with a higher risk of recurrence.2

Malignant meningiomas

The prognosis for patients with malignant meningioma is worse than for patients with the more well-differentiated meningiomas.

Choroid Plexus Tumors

Choroid plexus tumors are rare tumors arising from choroid plexus epithelial cells. The more benign form is choroid plexus papilloma; the more malignant form is called anaplastic choroid plexus papilloma. These latter tumors are most likely to spread within the craniospinal axis.

References:

1. Levin VA, Leibel SA, Gutin PH: Neoplasms of the central nervous system. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. Philadelphia, Pa: Lippincott-Raven Publishers, 5th ed., 1997, pp 2022-2082.

2. Nakasu S, Nakasu Y, Nakajima M, et al.: Preoperative identification of meningiomas that are highly likely to recur. Journal of Neurosurgery 90(3): 455-462, 1999.

Treatment Option Overview

Surgical removal of brain tumors is recommended for most types and in most locations and should be as complete as possible within the constraints of preservation of neurologic function.1 An exception to this role for surgery is for deep-seated tumors, such as pontine gliomas, which are diagnosed on clinical evidence and are treated without initial surgery approximately 50% of the time. In the majority of cases, however, diagnosis by biopsy is preferred. Stereotaxic biopsy can be used for lesions that are difficult to reach and resect.

Radiation therapy has a major role in the treatment of most tumor types and can increase the cure rate or prolong disease-free survival. Radiation therapy may also be useful in the treatment of recurrences in patients treated initially with surgery alone.

Chemotherapy may prolong survival in some tumor types and has been reported to lengthen disease-free survival in patients with gliomas, medulloblastoma, and some germ cell tumors.2 Local chemotherapy with a nitrosourea applied to a polymer placed directly in the brain during surgery has been shown to be a safe modality and is under clinical evaluation.1,3

Patients who have brain tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials that evaluate radiosensitizers, hyperthermia, or interstitial brachytherapy used in conjunction with external-beam radiation therapy to improve local control of the tumor or for studies that evaluate new drugs and biological response modifiers.

References:

1. Brem H, Piantadosi S, Burger PC, et al.: Placebo-controlled trial of safety and efficacy of intraoperative controlled delivery by biodegradable polymers of chemotherapy for recurrent gliomas. Lancet 345(8956): 1008-1012, 1995.

2. Cokgor I, Friedman HS, Friedman AH: Chemotherapy for adults with malignant glioma. Cancer Investigation 17(4): 264-272, 1999.

Brem H, Ewend MG, Piantadosi S, et al.: The safety of interstitial chemotherapy with BCNU-loaded polymer followed by radiation therapy in the treatment of newly diagnosed malignant gliomas: phase I trial. Journal of Neuro-Oncology 26(2): 111-123, 1995.

3. Levin VA, Leibel SA, Gutin PH: Neoplasms of the central nervous system. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. Philadelphia, Pa: Lippincott-Raven Publishers, 5th ed., 1997, pp 2022-2082.

Adult Noninfiltrating Astrocytoma

Juvenile pilocytic and subependymal astrocytomas 1

Noninfiltrating astrocytic tumors are often curable.

Treatment options:

Standard:

1. Surgery alone if totally resectable.
2. Surgery followed by radiation therapy to known or suspected residual tumor.2

Under clinical evaluation:

At recurrence following surgery, patients should be considered for reoperation and radiation therapy if not previously given. Patients who have received radiation therapy should be considered candidates for nitrosourea-based chemotherapies and for clinical trials that evaluate new drugs and biological response modifiers.

References:

1. Wallner KE, Gonzales MF, Edwards MS, et al.: Treatment results of juvenile pilocytic astrocytoma. Journal of Neurosurgery 69(2): 171-176, 1988.

2. Shaw EG, Daumas-Duport C, Scheithauer BW, et al.: Radiation therapy in the management of low-grade supratentorial astrocytomas. Journal of Neurosurgery 70(6): 853-861, 1989.

Adult Well-Differentiated Mildly and Moderately Anaplastic Astrocytoma

Well-differentiated mildly and moderately anaplastic astrocytomas are less often curable.

Treatment options:

Standard:

Surgery plus radiation therapy, although some controversy exists and some physicians treat these patients with surgery alone if the patient is younger than 35 years of age and the tumor does not contrast-enhance on a computed tomographic scan.1

Under clinical evaluation:

Clinical trials in progress are evaluating the effect of adding drugs to local therapy, for example, radiation therapy with or without chemotherapy for incompletely resected well-differentiated mildly and moderately anaplastic astrocytomas. Other trials are evaluating the effect of deferring irradiation until the time of tumor progression and of high-dose versus low-dose irradiation.

References:

1. Shaw EG, Daumas-Duport C, Scheithauer BW, et al.: Radiation therapy in the management of low-grade supratentorial astrocytomas. Journal of Neurosurgery 70(6): 853-861, 1989.

Adult Anaplastic Astrocytoma

For anaplastic astrocytomas of higher grade, the cure rate is low with standard local treatment.1 These patients are appropriate candidates for clinical trials designed to improve local control by adding newer forms of treatment to standard treatment.

Treatment options:

Standard:

1. Surgery plus radiation therapy.
2. Surgery plus radiation therapy and chemotherapy.2-6

Under clinical evaluation:

Patients with brain tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials that evaluate hyperfractionated irradiation, accelerated fraction radiation, stereotactic radiosurgery, radiosensitizers, hyperthermia, interstitial brachytherapy, or intraoperative radiation therapy used in conjunction with external-beam radiation therapy to improve local control of the tumor and/or for studies that evaluate new drugs and biological response modifiers following radiation therapy.7-11 Cooperative group trials that evaluate chemoradiotherapy administered with either hyperfractionated irradiation or a combination of brachytherapy and external-beam irradiation are now in progress. Carmustine (BCNU) impregnated polymer may be implanted during surgery.12,13

References:

1. Nelson DF, Nelson JS, Davis DR, et al.: Survival and prognosis of patients with astrocytoma with atypical or anaplastic features. Journal of Neuro-Oncology 3(2): 99-103, 1985.

2. Rodriguez L, Levin V: Does chemotherapy benefit the patient with a central nervous system glioma? Oncology (Huntington NY) 1(9): 29-36, 1987.

3. Chang CH, Horton J, Schoenfeld D, et al.: Comparison of postoperative radiotherapy and combined postoperative radiotherapy and chemotherapy in the multidisciplinary management of malignant gliomas: a joint Radiation Therapy Oncology and Eastern Cooperative Oncology Group Study. Cancer 52(6): 997-1007, 1983.

4. Levin VA, Silver P, Hannigan J, et al.: Superiority of post-radiotherapy adjuvant chemotherapy with CCNU, procarbazine, and vincristine (PCV) over BCNU for anaplastic gliomas: NCOG 6G61 final report. International Journal of Radiation Oncology, Biology, Physics 18(2): 321-324, 1990.

5. Friedman HS, Kerby T, Calvert H: Temozolomide and treatment of malignant glioma. Clinical Cancer Research 6(7): 2585-2597, 2000.

6. Prados MD, Levin V: Biology and treatment of malignant glioma. Seminars in Oncology 27(3 suppl 6): 1-10, 2000.

7. Nelson DF, Urtasun RC, Saunders WM, et al.: Recent and current investigations of radiation therapy of malignant gliomas. Seminars in Oncology 13(1): 46-55, 1986.

8. Levin VA: Chemotherapy of primary brain tumors. Neurologic Clinics 3(4): 855-866, 1985.

9. Shapiro WR: Therapy of adult malignant brain tumors: what have the clinical trials taught us? Seminars in Oncology 13(1): 38-45, 1986.

10. Loeffler JS, Alexander E, Shea WM, et al.: Radiosurgery as part of the initial management of patients with malignant gliomas. Journal of Clinical Oncology 10(9): 1379-1385, 1992.

11. Yung WK, Prados MD, et al., for the Temodal Brain Tumor Group: Multicenter phase II trial of temozolomide in patients with anaplastic astrocytoma or anaplastic oligoastrocytoma at first relapse. Journal of Clinical Oncology 17(9): 2762-2771, 1999.

12. Brem H, Piantadosi S, Burger PC, et al.: Placebo-controlled trial of safety and efficacy of intraoperative controlled delivery by biodegradable polymers of chemotherapy for recurrent gliomas. Lancet 345(8956): 1008-1012, 1995.

13. Brem H, Ewend MG, Piantadosi S, et al.: The safety of interstitial chemotherapy with BCNU-loaded polymer followed by radiation therapy in the treatment of newly diagnosed malignant gliomas: phase I trial. Journal of Neuro-Oncology 26(2): 111-123, 1995.

Adult Glioblastoma Multiforme

For glioblastoma multiforme, the cure rate is very low with standard local treatment. These patients are appropriate candidates for clinical trials designed to improve local control by adding newer forms of treatment to standard treatment.

Treatment options:

Standard:

1. Surgery plus radiation therapy and chemotherapy.1-3
2. Surgery plus radiation therapy.4

Under clinical evaluation:

Patients with brain tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials that evaluate hyperfractionated irradiation, accelerated fraction irradiation, stereotactic radiosurgery, radiosensitizers, hyperthermia, interstitial brachytherapy, or intraoperative radiation therapy used in conjunction with external-beam radiation therapy to improve local control of the tumor and/or for studies that evaluate new drugs and biological response modifiers following radiation therapy.5-8 Cooperative group studies that evaluate hyperfractionated irradiation and interstitial brachytherapy are in progress.9 Carmustine (BCNU) impregnated polymer may be implanted during surgery.10,11

References:

1. Shapiro WR: Therapy of adult malignant brain tumors: what have the clinical trials taught us? Seminars in Oncology 13(1): 38-45, 1986.

2. Rodriguez L, Levin V: Does chemotherapy benefit the patient with a central nervous system glioma? Oncology (Huntington NY) 1(9): 29-36, 1987.

3. Prados MD, Levin V: Biology and treatment of malignant glioma. Seminars in Oncology 27(3 suppl 6): 1-10, 2000.

4. Friedman HS, Kerby T, Calvert H: Temozolomide and treatment of malignant glioma. Clinical Cancer Research 6(7): 2585-2597, 2000.

5. Leibel SA, Gutin PH, Sneed PK, et al.: Interstitial irradiation for the treatment of primary and metastatic brain tumors. Cancer: Principles and Practice of Oncology Updates 3(7): 1-11, 1989.

6. Nelson DF, Urtasun RC, Saunders WM, et al.: Recent and current investigations of radiation therapy of malignant gliomas. Seminars in Oncology 13(1): 46-55, 1986.

7. Loeffler JS, Alexander E, Shea WM, et al.: Radiosurgery as part of the initial management of patients with malignant gliomas. Journal of Clinical Oncology 10(9): 1379-1385, 1992.

8. Fontanesi J, Clark WC, Weir A, et al.: Interstitial iodine 125 and concomitant cisplatin followed by hyperfractionated external beam irradiation for malignant supratentorial glioma. American Journal of Clinical Oncology 16(5): 412-417, 1993.

9. Scharfen CO, Sneed PK, Wara WM, et al.: High activity iodine-125 interstitial implant for gliomas. International Journal of Radiation Oncology, Biology, Physics 24(1): 583-591, 1992.

10. Brem H, Piantadosi S, Burger PC, et al.: Placebo-controlled trial of safety and efficacy of intraoperative controlled delivery by biodegradable polymers of chemotherapy for recurrent gliomas. Lancet 345(8956): 1008-1012, 1995.

11. Brem H, Ewend MG, Piantadosi S, et al.: The safety of interstitial chemotherapy with BCNU-loaded polymer followed by radiation therapy in the treatment of newly diagnosed malignant gliomas: phase I trial. Journal of Neuro-Oncology 26(2): 111-123, 1995.

Adult Brain Stem Glioma

Brain stem gliomas have a relatively poor prognosis that is correlated with histology (when biopsies are performed), location, and extent of tumor. The overall median survival time of patients in studies has been 44 to 74 weeks.1-5 The best results have been attained with hyperfractionated radiation therapy.5

Treatment options:

Standard:

Radiation therapy.1-5

Under clinical evaluation:

At recurrence, patients should be considered for clinical trials that evaluate new drugs and biological response modifiers.6,7

References:

1. Greenberger JS, Cassady JR, Levene MB: Radiation therapy of thalamic, midbrain and brain stem gliomas. Radiology 122(2): 463-468, 1977.

2. Levin VA, Edwards MS, Wara WM, et al.: 5-Fluorouracil and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU) followed by hydroxyurea, misonidazole and irradiation for brain stem gliomas: a pilot study of the Brain Tumor Research Center and the Children's Cancer Study Group. Neurosurgery 14(6): 679-681, 1984.

3. Allen JC, Bloom J, Ertel I, et al.: Brain tumors in children: current cooperative and institutional chemotherapy trials in newly diagnosed and recurrent disease. Seminars in Oncology 13(1): 110-122, 1986.

4. Eifel PJ, Cassady JR, Belli JA: Radiation therapy of tumors of the brainstem and midbrain in children: experience of the Joint Center for Radiation Therapy and Children's Hospital Medical Center (1971-1981). International Journal of Radiation Oncology, Biology, Physics 13(6): 847-852, 1987.

5. Shrieve DC, Wara WM, Edwards MS, et al.: Hyperfractionated radiation therapy for gliomas of the brainstem in children and in adults. International Journal of Radiation Oncology, Biology, Physics 24(1): 599-610, 1992.

6. Fulton DS, Levin VA, Wara WM, et al.: Chemotherapy of pediatric brain-stem tumors. Journal of Neurosurgery 54(6): 721-725, 1981.

7. Rodriguez LA, Prados M, Fulton D, et al.: Treatment of recurrent brain stem gliomas and other central nervous system tumors with 5-fluorouracil, CCNU, hydroxyurea, and 6-mercaptopurine. Neurosurgery 22(4): 691-693, 1988.

Adult Well-Differentiated Ependymoma

Myxopapillary ependymoma and well-differentiated ependymoma

These ependymomas are often curable.

Treatment options:

Standard:

1. Surgery alone if totally resectable.
2. Surgery followed by radiation therapy to known or suspected residual tumor.1,2

Under clinical evaluation:

At recurrence following surgery, patients should be considered for reoperation and radiation therapy if not previously used. Patients who have received radiation therapy should be considered candidates for nitrosourea-based chemotherapies and for clinical trials that evaluate new drugs and biological response modifiers.

References:

1. Wallner KE, Wara WM, Sheline GE, et al.: Intracranial ependymomas: results of treatment with partial or whole brain irradiation without spinal irradiation. International Journal of Radiation Oncology, Biology, Physics 12(11): 1937-1941, 1986.

2. Shaw EG, Evans RG, Scheithauer BW, et al.: Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients. International Journal of Radiation Oncology, Biology, Physics 13(10): 1457-1462, 1987.

Adult Malignant Ependymoma

Anaplastic ependymoma and ependymoblastoma

Malignant ependymomas have variable prognoses that depend on location and extent of disease. Frequently, but not invariably, anaplastic ependymomas have a worse prognosis than well-differentiated ependymomas. The rare ependymoblastoma has a much worse prognosis.

Treatment options:

Standard:

Surgery plus radiation therapy.1,2

Under clinical evaluation:

Adjuvant chemotherapy before, during, and after radiation are treatment options being evaluated. At recurrence, patients should be considered candidates for nitrosourea-based chemotherapies and for clinical trials that evaluate new drugs and biological response modifiers.

References:

1. Wallner KE, Wara WM, Sheline GE, et al.: Intracranial ependymomas: results of treatment with partial or whole brain irradiation without spinal irradiation. International Journal of Radiation Oncology, Biology, Physics 12(11): 1937-1941, 1986.

2. Shaw EG, Evans RG, Scheithauer BW, et al.: Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients. International Journal of Radiation Oncology, Biology, Physics 13(10): 1457-1462, 1987.

Adult Well-Differentiated Oligodendroglioma

These tumors behave very similarly to the well-differentiated mildly and moderately anaplastic astrocytomas.

Treatment options:

Standard:

Surgery plus radiation therapy; however, some controversy exists. Some physicians treat these patients with surgery alone if the patient is younger than 45 years of age and the tumor is not contrast-enhanced on a computed tomographic scan.1

Under clinical evaluation:

Clinical trials in progress are evaluating the effect of adding drugs to local therapy, e.g., radiation therapy with or without chemotherapy for incompletely resected well-differentiated mildly or moderately anaplastic astrocytomas.

References:

1. Salazar OM, Castro-Vita H, Van Houtte P, et al.: Improved survival in cases of intracranial ependymoma after radiation therapy: late report and recommendations. Journal of Neurosurgery 59(4): 652-659, 1983.

Adult Anaplastic Oligodendroglioma

For anaplastic oligodendrogliomas of higher grade, the cure rate is low with standard local treatment.1 Such patients are appropriate candidates for clinical trials designed to improve local control by adding newer forms of treatment.

Treatment options:

Standard:

1. Surgery plus radiation therapy.2-5
2. Surgery plus radiation therapy plus chemotherapy.6

Under clinical evaluation:

Patients with brain tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials that evaluate interstitial brachytherapy, radiosensitizers, hyperthermia, or intraoperative radiation therapy in conjunction with external-beam radiation therapy to improve local control of the tumor and/or for studies that evaluate new drugs and biological response modifiers following radiation therapy.

References:

1. Kyritsis AP, Yung WK, Bruner J, et al.: The treatment of anaplastic oligodendrogliomas and mixed gliomas. Neurosurgery 32(3): 365-371, 1993.

2. Bullard DE, Rawlings CE, Phillips BW, et al.: Oligodendroglioma: an analysis of the value of radiation therapy. Cancer 60(9): 2179-2188, 1987.

3. Burger PC, Rawlings CE, Cox EB, et al.: Clinicopathologic correlations in the oligodendroglioma. Cancer 59(7): 1345-1352, 1987.

4. Lindegaard KF, Mork SJ, Eide GE, et al.: Statistical analysis of clinicopathological features, radiotherapy, and survival in 170 cases of oligodendroglioma. Journal of Neurosurgery 67(2): 224-230, 1987.

5. Wallner KE, Gonzales M, Sheline GE: Treatment of oligodendrogliomas with or without postoperative irradiation. Journal of Neurosurgery 68(5): 684-688, 1988.

6. Cairncross JG, Macdonald DR: Successful chemotherapy for recurrent malignant oligodendroglioma. Annals of Neurology 23(4): 360-364, 1988.

Mixed Gliomas

Mixed astrocytoma-ependymoma, mixed astrocytoma-oligodendroglioma, and mixed astrocytoma-ependymoma-oligodendroglioma

These mixed glial tumors have a prognosis similar to that for anaplastic astrocytomas and can be treated as such.

Treatment options:1

Standard:

1. Surgery plus radiation therapy.2
2. Surgery plus radiation therapy plus chemotherapy.

Under clinical evaluation:

Patients with brain tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials that evaluate interstitial brachytherapy, radiosensitizers, hyperthermia, or intraoperative radiation therapy in conjunction with external-beam radiation therapy to improve local control of the tumor and/or for studies that evaluate new drugs and biological response modifiers following radiation therapy.

References:

1. Kyritsis AP, Yung WK, Bruner J, et al.: The treatment of anaplastic oligodendrogliomas and mixed gliomas. Neurosurgery 32(3): 365-371, 1993.

2. Shaw EG, Daumas-Duport C, Scheithauer BW, et al.: Radiation therapy in the management of low-grade supratentorial astrocytomas. Journal of Neurosurgery 70(6): 853-861, 1989.

Adult Medulloblastoma

Treatment options:

Standard:

Surgery plus craniospinal irradiation for good-risk patients.1,2

Under clinical evaluation:

For poor-risk patients, in addition to surgery plus craniospinal irradiation, various chemotherapy programs are being evaluated.3

References:

1. Levin VA, Vestnys PS, Edwards MS, et al.: Improvement in survival produced by sequential therapies in the treatment of recurrent medulloblastoma. Cancer 51(8): 1364-1370, 1983.

2. Carrie C, Lasset C, Alapetite C, et al.: Multivariate analysis of prognostic factors in adult patients with medulloblastoma: retrospective study of 156 patients. Cancer 74(8): 2352-2360, 1994.

3. Allen JC, Bloom J, Ertel I, et al.: Brain tumors in children: current cooperative and institutional chemotherapy trials in newly diagnosed and recurrent disease. Seminars in Oncology 13(1): 110-122, 1986.

Adult Pineal Parenchymal Tumor

Pineocytoma and pineoblastoma

These diverse tumors require special consideration. Pineocytomas are slow growing and carry variable prognoses for cure. Pineoblastomas are more rapidly growing and have a worse prognosis.

Pineal astrocytoma

Depending on the degree of anaplasia, pineal astrocytomas vary in prognosis. Higher grades have a worse prognosis.

Treatment options:

Standard:

1. Surgery plus radiation therapy for pineocytoma and lower grades of astrocytoma.1,2
2. Surgery plus radiation therapy and chemotherapy for pineoblastoma and higher grades of astrocytoma.1,2

Under clinical evaluation:

Patients with brain tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials that evaluate radiosensitizers, hyperthermia, or intraoperative radiation therapy in conjunction with external-beam radiation therapy to improve local control of the tumor and/or for studies that evaluate new drugs and biological response modifiers following radiation therapy.

References:

1. Stein BM, Fetell MR: Therapeutic modalities for pineal region tumors. Clinical Neurosurgery 32: 445-455, 1985.

2. Rich TA, Cassady JR, Strand RD, et al.: Radiation therapy for pineal and suprasellar germ cell tumors. Cancer 55(5): 932-940, 1985.

Adult Central Nervous System Germ Cell Tumor

Germinoma, embryonal carcinoma, choriocarcinoma, and teratoma

The prognosis and treatment of germ cell tumors depends on the histology, location, presence and amount of biological markers, and surgical resectability.1,2

References:

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

2. 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.

Adult Craniopharyngioma

Craniopharyngioma is often curable.

Treatment options:

Standard:

1. Surgery alone if totally resectable.1
2. Debulking surgery plus radiation therapy if unresectable.2

References:

1. Baskin DS, Wilson CB: Surgical management of craniopharyngiomas: a review of 74 cases. Journal of Neurosurgery 65(1): 22-27, 1986.

2. Rajan B, Ashley S, Gorman C, et al.: Craniopharyngioma - long-term results following limited surgery and radiotherapy. Radiotherapy and Oncology 26(1): 1-10, 1993.

Adult Meningioma

Meningioma is usually curable when resectable.

Treatment options:

Standard:

1. Surgery.1
2. Surgery plus radiation therapy (in selected cases, such as for patients with known or suspected residual disease or with recurrence after previous surgery).2-4

References:

1. Black PM: Meningiomas. Neurosurgery 32(4): 643-657, 1993.

2. Wara WM, Sheline GE, Newman H, et al.: Radiation therapy of meningiomas. American Journal of Roentgenology, Radium Therapy and Nuclear Medicine 123(3): 453-458, 1975.

3. Barbaro NM, Gutin PH, Wilson CB, et al.: Radiation therapy in the treatment of partially resected meningiomas. Neurosurgery 20(4): 525-528, 1987.

4. Taylor BW, Marcus RB, Friedman WA, et al.: The meningioma controversy: postoperative radiation therapy. International Journal of Radiation Oncology, Biology, Physics 15(2): 299-304, 1988.

Adult Malignant Meningioma

Malignant meningioma, hemangiopericytoma, and papillary meningioma

The prognosis for patients with malignant meningioma is worse than for the more well-differentiated meningiomas because complete resections are less common and the proliferative capacity is greater.1,2

Treatment options:

Standard:

Surgery plus radiation therapy.

Under clinical evaluation:

Patients with brain tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials that evaluate interstitial brachytherapy, radiosensitizers, hyperthermia, or intraoperative radiation therapy in conjunction with external-beam radiation therapy to improve local control of the tumor and/or for studies that evaluate new drugs and biological response modifiers following radiation therapy.

References:

1. Alvarez F, Roda JM, Perez-Romero M, et al.: Malignant and atypical meningiomas: a reappraisal of clinical, histological, and computed tomographic features. Neurosurgery 20(5): 688-694, 1987.

2. Perry A, Scheithauer BW, Stafford SL, et al.: "Malgnancy" in meningiomas: a clinicopathologic study of 116 patients, with grading implications. Cancer 85(9): 2046-2056, 1999.

Recurrent Adult Brain Tumor

Treatment options:

Standard:

1. Surgery alone or in conjunction with chemotherapy.1-3
2. Radiation therapy if not previously used, alone or with chemotherapy.
3. Interstitial irradiation.4

Under clinical evaluation:

Numerous clinical trials (particularly phase II trials) are evaluating the use of newer drugs in the treatment of brain tumors. Carmustine (BCNU) impregnated polymer may be implanted during surgery.5,6

References:

1. Salcman M, Kaplan RS, Ducker TB, et al.: Effect of age and reoperation on survival in the combined modality treatment of malignant astrocytoma. Neurosurgery 10(4): 454-463, 1982.

2. Rodriguez L, Levin V: Does chemotherapy benefit the patient with a central nervous system glioma? Oncology (Huntington NY) 1(9): 29-36, 1987.

3. Young B, Oldfield EH, Markesbery WR, et al.: Reoperation for glioblastoma. Journal of Neurosurgery 55(6): 917-921, 1981.

4. Leibel SA, Gutin PH, Sneed PK, et al.: Interstitial irradiation for the treatment of primary and metastatic brain tumors. Cancer: Principles and Practice of Oncology Updates 3(7): 1-11, 1989.

5. Brem H, Piantadosi S, Burger PC, et al.: Placebo-controlled trial of safety and efficacy of intraoperative controlled delivery by biodegradable polymers of chemotherapy for recurrent gliomas. Lancet 345(8956): 1008-1012, 1995.

6. Brem H, Ewend MG, Piantadosi S, et al.: The safety of interstitial chemotherapy with BCNU-loaded polymer followed by radiation therapy in the treatment of newly diagnosed malignant gliomas: phase I trial. Journal of Neuro-Oncology 26(2): 111-123, 1995.

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