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Treatment options in astrocytomas include operative intervention and the use of chemotherapy and radiation

therapy. Treatment decisions are generally best made by a team approach, including input from the involved
neurosurgeon, radiation oncologist, and medical oncologist or neurologist. Generally, care is primarily directed
by a neurologist or specialist in neurooncology.
A study by Ishkanian et al found that adjuvant radiotherapy for pilocytic astrocytoma significantly prolonged
progression-free survival (PFS) at both 5 years and 10 years compared with observation alone. However, the
overall survival was equivalent.[22]
There is no accepted standard of treatment for low-grade or anaplastic astrocytoma. Typically, anaplastic
astrocytomas are treated with surgery, radiotherapy, and adjuvant temozolomide. Some practitioners add
concomitant temozolomide, though no data from controlled trials exist to support concomitant temozolomide. [2,
3]

In a retrospective study of 165 adult patients with anaplastic astrocytoma who were receiving adjuvant
radiation, concurrent treatment with temozolomide were associated with improved survival, as was isocitrate
dehydrogenase (IDH) mutation. On univariable analysis, improved 5-year survival was independently
associated with concurrent temozolomide (46.2 vs. 29.3%, P = 0.02) and IDH mutation (78.9 vs. 22.0%, P <
0.001).[23]
Anaplastic astrocytomas are usually more responsive to chemotherapy than glioblastomas. [24, 25] For recurrent
anaplastic astrocytomas previously treated with nitrosoureas, temozolomide showed a 35% response rate, and
compared to therapies with lower response rates, temozolomide provided an increased 6-month survival rate
(46% vs 31%).[26, 27] Some smaller survival benefit has been shown with adjuvant carmustine. [4]
Treatment of low-grade astrocytomas remains more controversial. The role of maximal surgical resection,
timing of radiotherapy, and the role, timing, and appropriate agents of chemotherapy are not clear. The
controversy due to a lack of strong data is compounded by the relatively young age of the patients, the
relatively indolent natural history of low-grade astrocytomas, and the morbidity associated with these
interventions.[28, 29]
Patients with an astrocytoma and a history of seizures should receive anticonvulsant therapy with monitoring of
the drug concentration in the blood. The use of anticonvulsants prophylactically in astrocytoma patients with no
prior history of seizures has been reported but remains controversial.
The use of corticosteroids, such as dexamethasone, yields rapid improvement in most patients secondary to a
reduction of tumor mass effect. Concurrent prophylaxis for gastrointestinal ulcers should be prescribed with
corticosteroid administration.
See Brain Cancer Treatment Protocols for summarized information.

Brainstem gliomas
Brainstem tumors account for 10-20%[30] of all central nervous system (CNS) tumors in the pediatric population,
and are typically diagnosed in children 7-9 years old.[31, 32] Though many classification schemes exist, treatment
and prognosis for brainstem gliomas typically depend on whether the tumor is diffuse or focal.
Diffuse brainstem gliomas

Diffuse brainstem gliomas make up 58-75%[33] of all brainstem tumors, typically arise in the pons, and are
noncircumscribed on MRI. They are often malignant fibrillary astrocytomas (WHO grade III or IV) that infiltrate
along white-matter tracts into the midbrain and thalamus and have a rapidly progressive and fatal course.
Clinical presentation of these tumors often involves ataxia, cerebellar signs, and long tract signs. [34] When
clinical and radiographic evidence suggests diffuse brainstem glioma, biopsy is of limited use as tumor
histology does not often alter treatment.[33, 35, 36, 37, 38, 39, 40, 41]
No treatment has been shown to cure or prolong survival in these patients, and radiation necrosis and
chemotherapy side effects can be significant. No benefit of surgical resection has been shown, largely due to
the eloquence of the region and diffuse and aggressive nature of the tumor.[34, 42] Corticosteroids may provide
temporary benefit by reducing edema.
Irradiation has been shown to provide temporary clinical improvement and is sometimes employed, but a large
phase III trial showed no benefit.[43] Even with radiation therapy, 1-year survival has been shown to be 35-46%,
and 3-year survival 11-17%.[44, 45]
Chemotherapy is also sometimes used.[46] Convection-enhanced delivery of chemotherapy offers one potential
avenue for improving the prognosis of these patients, and studies are ongoing.
Focal brainstem gliomas
Focal brainstem gliomas are usually WHO grade I or II, well-circumscribed on MRI with variable contrast
enhancement, are more often found in the medulla and midbrain and have a much better prognosis than
diffuse brainstem gliomas. Surgery is often the primary treatment for focal brainstem gliomas as well as dorsal
exophytic brainstem gliomas, though the decision to operate, surgical approach, and extent of resection
depend on location, patient factors, and the surgeon's judgment.
Obstructive hydrocephalus is common. It is usually treated by a separate procedure, either endoscopic third
ventriculostomy or shunt placement.[5]
In a study of 39 children (median age, 10 years) with low-grade glioma, treatment with intensity-modulated
radiotherapy (IMRT) after incomplete resection or disease progression was found to provide local control rates
comparable to those provided by 2-dimensional and 3-dimensional radiotherapy. The 8-year progression-free
and overall survival rates with IMRT were 78.2% and 93.7%, respectively.[47, 48]
The researchers used three approaches to identify the target area for IMRT: The first method (n=19) was to
delineate the gross tumor volume (GTV) and add a 1-cm margin to create the clinical target volume. In the
second method (n=6), a 0.5-cm margin was added around the GTV to create the clinical target volume. For
both methods, the prescribed GTV dose was the same as the clinical treatment volume: a median dose of 50.4
Gy.
Method 3 (n=14) was dose painting: the GTV was delineated and a second target volume was created by
adding 1 cm to the GTV. The second target volume was treated with a lower radiation dose than the GTV
(median, 41.4 Gy versus 50.4 Gy). Multivariate analysis showed no difference in progression according to the
method of target delineation, suggesting that a 1-cm margin may not be necessary.[47, 48]

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