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Information from PDQ -- for Health Professionals
Adult brain tumor
208/01143
** GENERAL INFORMATION **
Prognoses of primary brain tumors are determined by histologic type and 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). (Refer to the PDQ summary on Primary Central Nervous
System Lymphoma Treatment for more information.)
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 Williams & Wilkins,
6th ed., 2001, pp 2100-2160.
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. 2nd ed., Berlin: Springer-Verlag,
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.
(Refer to the PDQ summary on Primary Central Nervous System Lymphoma Treatment
for more information.)
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 Williams & Wilkins,
6th ed., 2001, pp 2100-2160.
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 half
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.
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. 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.
3. 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.
4. 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 Williams & Wilkins,
6th ed., 2001, pp 2100-2160.
** ADULT NONINFILTRATING ASTROCYTOMA **
-- Juvenile pilocytic and subependymal astrocytomas --
Noninfiltrating astrocytic tumors are often curable.[1]
Standard treatment options:
1. Surgery alone if totally resectable.
2. Surgery followed by radiation therapy to known or suspected residual
tumor.[2]
Treatment options 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.
Standard treatment options:
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.[1,2]
Treatment options 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.
2. Kaye AH, Walker DG: Low grade astrocytomas: controversies in management.
Journal of Clinical Neuroscience 7(6): 475-483, 2000.
** 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.
Standard treatment options:
1. Surgery plus radiation therapy.
2. Surgery plus radiation therapy and chemotherapy.[2-7]
Treatment options 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.[8-12] 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.[13,14]
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. Macdonald DR: Temozolomide for recurrent high-grade glioma. Seminars in
Oncology 28(4 suppl 13): 3-12, 2001.
8. 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.
9. Levin VA: Chemotherapy of primary brain tumors. Neurologic Clinics 3(4):
855-866, 1985.
10. Shapiro WR: Therapy of adult malignant brain tumors: what have the
clinical trials taught us? Seminars in Oncology 13(1): 38-45, 1986.
11. 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.
12. 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.
13. 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.
14. 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.
Standard treatment options:
1. Surgery plus radiation therapy and chemotherapy.[1-5]
2. Surgery plus radiation therapy.
Treatment options 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.[6-9] Cooperative group studies that evaluate hyperfractionated
irradiation and interstitial brachytherapy are in progress.[10]
Carmustine (BCNU) impregnated polymer may be implanted during surgery.[11,12]
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. Macdonald DR: Temozolomide for recurrent high-grade glioma. Seminars in
Oncology 28(4 suppl 13): 3-12, 2001.
6. 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.
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. 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.
9. 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.
10. 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.
11. 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.
12. 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]
Standard treatment options:
Radiation therapy.[1-6]
Treatment options under clinical evaluation:
At recurrence, patients should be considered for clinical trials that evaluate
new drugs and biological response modifiers.[7,8]
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. Surma-aho O, Niemela M, Vilkki J, et al.: Adverse long-term effects of
brain radiotherapy in adult low-grade glioma patients. Neurology
56(10): 1285-1290, 2001.
7. Fulton DS, Levin VA, Wara WM, et al.: Chemotherapy of pediatric
brain-stem tumors. Journal of Neurosurgery 54(6): 721-725, 1981.
8. 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.
Standard treatment options:
1. Surgery alone if totally resectable.
2. Surgery followed by radiation therapy to known or suspected residual
tumor.[1,2]
Treatment options 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.
Standard treatment options:
Surgery plus radiation therapy.[1,2]
Treatment options 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.
Standard treatment options:
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]
Treatment options 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.
Standard treatment options:
1. Surgery plus radiation therapy.[2-5]
2. Surgery plus radiation therapy plus chemotherapy.[6]
Treatment options 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.
Standard treatment options:[1]
1. Surgery plus radiation therapy.[2]
2. Surgery plus radiation therapy plus chemotherapy.
Treatment options under clinical evaluation:[1]
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 **
Standard treatment options:
Surgery plus craniospinal irradiation for good-risk patients.[1,2]
Treatment options under clinical evaluation:
For poor-risk patients, in addition to surgery plus craniospinal irradiation,
various chemotherapy programs are being evaluated.[3] (Refer to the PDQ
summary on Childhood Medulloblastoma Treatment for more information.)
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.
Standard treatment options:
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]
Treatment options 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.
Standard treatment options:
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.
Standard treatment options:
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]
3. Radiation therapy for patients with unresectable tumors.[5]
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.
5. Debus J, Wuendrich M, Pirzkall A, et al.: High efficacy of fractionated
stereotactic radiotherapy of large base-of-skull meningiomas: long-term
results. Journal of Clinical Oncology 19(15): 3547-3553, 2001.
** 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]
Standard treatment options:
Surgery plus radiation therapy.
Treatment options 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.: "Malignancy" in
meningiomas: a clinicopathologic study of 116 patients, with grading
implications. Cancer 85(9): 2046-2056, 1999.
** RECURRENT ADULT BRAIN TUMOR **
Standard treatment options:
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]
4. Chemotherapy.[5]
Treatment options 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.[6,7]
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. Chinot OL, Honore S, Dufour H et al.: Safety and efficacy of temozolomide
in patients with recurrent anaplastic oligodendrogliomas after standard
radiotherapy and chemotherapy. Journal of Clinical Oncology 19(9):
2449-2455, 2001.
6. 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.
7. 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.
Date Last Modified: 07/2002
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