Two related drugs have been shown to shrink or stabilize subependymal giant cell tumors: rapamycin and everolimus. These both belong to the mTOR inhibitor class of immunosuppressants, and are both contraindicated in patients with severe infections.

Rapamycin showed efficacy in five cases of SEGA in TSC patients, shrinking their tumor volumes by an average of 65%. However, after the drug was stopped, the tumors regrew.

Everolimus, which has a similar structure as rapamycin, but with slightly increased bioavailability and shorter half-life, was studied in 28 patients with SEGA. There was a significant reduction in SEGA size in 75% of the patients, and a mild improvement in their seizures. Everolimus was approved for the treatment of SEGA by the US Food and Drug Administration (FDA) in October, 2010.

A NIH Consensus Conference report in 1999 recommends that any SEGA that is growing or causing symptoms should be surgically removed. Tumors are also removed in cases where a patient is suffering from a high seizure burden. If a tumor is rapidly growing or causing symptoms of hydrocephalus, deferring surgery may lead to vision loss, need for ventricular shunt, and ultimately death. Total removal of the tumor is curative.

Surgery to remove intraventricular tumors also carries risks of complications or death. Potential complications include transient memory impairment, hemiparesis, infection, chronic ventriculoperitoneal shunt placement, stroke, and death.

Treatment options include surgery, radiotherapy, radiosurgery, and chemotherapy.

The infiltrating growth of microscopic tentacles in fibrillary astrocytomas makes complete surgical removal difficult or impossible without injuring brain tissue needed for normal neurological function. However, surgery can still reduce or control tumor size. Possible side effects of surgical intervention include brain swelling, which can be treated with steroids, and epileptic seizures. Complete surgical excision of low grade tumors is associated with a good prognosis. However, the tumor may recur if the resection is incomplete, in which case further surgery or the use of other therapies may be required.

Standard radiotherapy for fibrillary astrocytoma requires from ten to thirty sessions, depending on the sub-type of the tumor, and may sometimes be performed after surgical resection to improve outcomes and survival rates. Side effects include the possibility of local inflammation, leading to headaches, which can be treated with oral medication. Radiosurgery uses computer modelling to focus minimal radiation doses at the exact location of the tumor, while minimizing the dose to the surrounding healthy brain tissue. Radiosurgery may be a complementary treatment after regular surgery, or it may represent the primary treatment technique.

Although chemotherapy for fibrillary astrocytoma improve overall survival, it is effective only in about 20% of cases. Researchers are currently investigating a number of promising new treatment techniques including gene therapy, immunotherapy, and novel chemotherapies.

Chemotherapy is the preferred secondary treatment after resection. The treatment kills astroblastoma cells left behind after surgery and induces a non-dividing, benign state for remaining tumor cells. Normally, chemotherapy is not recommended until the second required resection, implying that the astroblastoma is a high-grade tumor continuing to recur every few months. A standard chemotherapy protocol starts with two rounds of nimustine hydrochoride (ACNU), etoposide, vincristine, and interferon-beta. The patient undergoes a strict drug regimen until another surgery is required. By the third surgery, should recurrence in the astroblastoma occur, a six-round program of ifosfamide, cisplatin, and etoposide will "shock" the patient's system to the point where recurrence halts. Unfortunately, chemotherapy may not always be successful with patients requiring further resection of the tumor, since the tumor cell begins to show superior vasculature and a strong likelihood of compromising a patient's well-being. Oral ingestion of temozolomide for at-home bedside use may be preferred by the patient.

The goal of radiation therapy is to kill tumor cells while leaving normal brain tissue unharmed. In standard external beam radiation therapy, multiple treatments of standard-dose "fractions" of radiation are applied to the brain. This process is repeated for a total of 10 to 30 treatments, depending on the type of tumor. This additional treatment provides some patients with improved outcomes and longer survival rates.

Radiosurgery is a treatment method that uses computerized calculations to focus radiation at the site of the tumor while minimizing the radiation dose to the surrounding brain. Radiosurgery may be an adjunct to other treatments, or it may represent the primary treatment technique for some tumors. Forms used include stereotactic radiosurgery, such as Gamma knife, Cyberknife or Novalis Tx radiosurgery.

Radiotherapy may be used following, or in some cases in place of, resection of the tumor. Forms of radiotherapy used for brain cancer include external beam radiation therapy, the most common, and brachytherapy and proton therapy, the last especially used for children.

Radiotherapy is the most common treatment for secondary brain tumors. The amount of radiotherapy depends on the size of the area of the brain affected by cancer. Conventional external beam "whole-brain radiotherapy treatment" (WBRT) or "whole-brain irradiation" may be suggested if there is a risk that other secondary tumors will develop in the future. Stereotactic radiotherapy is usually recommended in cases involving fewer than three small secondary brain tumors.

People who receive stereotactic radiosurgery (SRS) and whole-brain radiation therapy (WBRT) for the treatment of metastatic brain tumors have more than twice the risk of developing learning and memory problems than those treated with SRS alone.

Patients undergoing chemotherapy are administered drugs designed to kill tumor cells. Although chemotherapy may improve overall survival in patients with the most malignant primary brain tumors, it does so in only about 20 percent of patients. Chemotherapy is often used in young children instead of radiation, as radiation may have negative effects on the developing brain. The decision to prescribe this treatment is based on a patient's overall health, type of tumor, and extent of the cancer. The toxicity and many side effects of the drugs, and the uncertain outcome of chemotherapy in brain tumors puts this treatment further down the line of treatment options with surgery and radiation therapy preferred.

UCLA Neuro-Oncology publishes real-time survival data for patients with a diagnosis of glioblastoma multiforme. They are the only institution in the United States that displays how brain tumor patients are performing on current therapies. They also show a listing of chemotherapy agents used to treat high-grade glioma tumors.

Supportive treatment focuses on relieving symptoms and improving the patient’s

neurologic function. The primary supportive agents are anticonvulsants and

corticosteroids.

- Historically, around 90% of patients with glioblastoma underwent anticonvulsant treatment, although it has been estimated that only approximately 40% of patients required this treatment. Recently, it has been recommended that neurosurgeons not administer anticonvulsants prophylactically, and should wait until a seizure occurs before prescribing this medication. Those receiving phenytoin concurrent with radiation may have serious skin reactions such as erythema multiforme and Stevens–Johnson syndrome.

- Corticosteroids, usually dexamethasone given 4 to 8 mg every 4 to 6 h, can reduce peritumoral edema (through rearrangement of the blood–brain barrier), diminishing mass effect and lowering intracranial pressure, with a decrease in headache or drowsiness.

Conventional radiotherapy, limited to the involved area of tumour, is the mainstay of treatment for DIPG. A total radiation dosage ranging from 5400 to 6000 cGy, administered in daily fractions of 150 to 200 cGy over 6 weeks, is standard. Hyperfractionated (twice-daily) radiotherapy was used previously to deliver higher radiation dosages, but did not lead to improved survival. Radiosurgery (e.g., gamma knife or cyberknife) has no role in the treatment of DIPG.

Radiation therapy selectively kills astroblastoma cells while leaving surrounding normal brain tissue unharmed. The use of radiation therapy after an astroblastoma excision has variable results. Conventional external beam radiation has both positive and negative effects on patients, but it is not recommended at this point to treat all types. All in all, the radiosensitivity of astroblastoma to therapy remains unclear, since some research advocate its effectiveness while others diminish the effects. Future studies must be done on patients with both total excision and sub-excision of the tumor to accurately assess whether radiation benefits patients under different circumstances.

Unlike most brain tumors, brainstem glioma is not often treated with neurosurgery due to complications in vital parts of the brain. More often, it is treated with chemotherapy and/or radiation therapy (though past use of radiation therapy has yielded mixed results.)

There are several new clinical trials in process. One such trial is dendritic cell immunotherapy which uses the patient’s tumor cells and white blood cells to produce a chemotherapy that directly attacks the tumor.

However, these treatments do produce side effects; most often including nausea, the breakdown of the immune system, and fatigue. Hair loss can occur from both chemotherapy and radiation, but usually grows back after chemotherapy has ceased. Steroids such as Decadron may be required to treat swelling in the brain. Decadron can lead to weight gain and infection. Patients may also experience seizures, which need to be treated to avoid complications. For some patients there is a chance of a neurological break down, this can include, but is not limited to, confusion and memory loss.

The use of topotecan has been investigated.

Children with cerebellar pilocytic astrocytoma may experience side effects related to the tumor itself depending on the location and related to the treatment. Strabismus.

- Symptoms related to increased pressure in the brain often disappear after surgical removal of the tumor.

- Effects on coordination and balance improved and might progressively (to completely) disappear as recovery progresses.

- Steroid-treatment is often used to control tissue swelling that may occur pre- and post-operatively.

- Children Diagnosed can also suffer long term side effects due to the type of treatment they may receive.

Most studies show no benefit from the addition of chemotherapy. However, a large clinical trial of 575 participants randomized to standard radiation versus radiation plus temozolomide chemotherapy showed that the group receiving temozolomide survived a median of 14.6 months as opposed to 12.1 months for the group receiving radiation alone. This treatment regime is now standard for most cases of glioblastoma where the person is not enrolled in a clinical trial. Temozolomide seems to work by sensitizing the tumor cells to radiation.

High doses of temozolomide in high-grade gliomas yield low toxicity, but the results are comparable to the standard doses.

Antiangiogenic therapy with medications such as bevacizumab control symptoms but do not affect overall survival.

The most common form of treatment is having the tumor surgically removed however total resection is often not possible. The location could prohibit access to the neoplasm and lead to incomplete or no resection at all. Removal of the tumor will generally allow functional survival for many years. In particular for pilocytic astrocytomas (that are commonly indolent bodies that may permit normal neurologic function) surgeons may decide to monitor the neoplasm's evolution and postpone surgical intervention for some time. However, left unattended these tumors may eventually undergo neoplastic transformation.

If surgery is not possible, recommendations such as chemotherapy or radiation be suggested however side effects from these treatments can be extensive and long term.

The role of chemotherapy in DIPG remains unclear. Studies have shown little improvement in survival, although efforts (see below) through the Children's Oncology Group (COG), Paediatric Brain Tumour Consortium (PBTC), and others are underway to explore further the use of chemotherapy and other drugs. Drugs that increase the effect of radiotherapy (radiosensitizers) have shown no added benefit, but promising new agents are under investigation. Immunotherapy with beta-interferon and other drugs has also had little effect in trials. Intensive or high-dose chemotherapy with autologous bone marrow transplantation or peripheral blood stem cell rescue has not demonstrated any effectiveness in brain stem gliomas. Future clinical trials may involve medicines designed to interfere with cellular pathways (signal transfer inhibitors), or other approaches that alter the tumor or its environment.

Treatment typically consists of radiotherapy and steroids for palliation of symptoms. Radiotherapy may result in minimally extended survival time. Prognosis is very poor, with only 37% of treated patients surviving one year or more. Topotecan has been studied in the treatment of brainstem glioma, otherwise, chemotherapy is probably ineffective, though further study is needed.

Total resection of the tumour, followed by radiation therapy is the standard treatment modality. Medulloepithelioma of the ciliary body may necessitate enucleation of the eye. Radiation therapy alone may prolong survival. Aggressive chemotherapy with autologous bone marrow transplant is used for metastatic medulloepitheliomas.

The treatment for hemangioblastoma is surgical excision of the tumor. Although usually straightforward to carry out, recurrence of the tumor or more tumors at a different site develop in approximately 20% of patients. Gamma Knife Radiosurgery as well as LINAC have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas.

Germinomas, like several other types of germ cell tumor, are sensitive to both chemotherapy and radiotherapy. For this reason, treatment with these methods can offer excellent chances of longterm survival, even cure.

Although chemotherapy can shrink germinomas, it is not generally recommended alone unless there are contraindications to radiation. In a study in the early 1990s, carboplatinum, etoposide and bleomycin were given to 45 germinoma patients, and about half the patients relapsed. Most of these relapsed patients were then recovered with radiation or additional chemotherapy.

Medulloepithelioma carries a dismal prognosis with a median survival of 5 months.

Treatment to remove these tumors always involve radical surgery. The reported recurrence rate for a subtotal removal is 30% after a mean interval period of 8.1 years.

Surgery is the primary treatment for removal of the brain tumor. Use of an endoscope may assist on obtaining a more complete surgical removal.

It has been seen that a few patients have tumors that grow unusually fast, especially after surgery. After surgery it is highly suggested the patients get quarterly MRI's to monitor their tumors or as per neurosurgeons/neurologists order. If monitoring the tumor, it is suggested to use the same facility for each scan. Using different facilities can result in minor variations in the scan which can result in false measurements of the brain tumor.

Intracranial epidermoid tumors are slow growing lesions, which may recur after incomplete removal during surgery, although it will most likely take many years. These slow growing benign brain tumors envelop nerves and arteries rather than displacing them.

Based on a survey of >800, surgical removal of the entire involved kidney plus the peri-renal fat appeared curative for the majority of all types of mesoblastic nephroma; the patient overall survival rate was 94%. Of the 4% of non-survivors, half were due to surgical or chemotherapeutic treatments. Another 4% of these patients suffered relapses, primarily in the local area of surgery rare cases of relapse due to lung or bone metastasis.. About 60% of these recurrent cases had a complete remission following further treatment. Recurrent disease was treated with a second surgery, radiation, and/or chemotherapy that often vincristine and actinomycin treatment. Removal of the entire afflicted kidney plus the peri-renal fat appears critical to avoiding local recurrences. In general, patients who were older than 3 months of age at diagnosis or had the cellular form of the disease, stage III disease, or involvement of renal lymph nodes had a higher recurrence rate. Among patients with these risk factors, only those with lymph node involvement are recommended for further therapy.

It has been suggested that mesoblastic nephroma patients with lymph node involvement or recurrent disease might benefit by adding the ALK inhibitor, crizotinib, or a tyrosine kinase inhibitor, either larotrectinib or entrectinib, to surgical, radiation, and/or chemotherapy treatment regimens. These drugs inhibit NTRK3's tyrosine kinase activity. Crizotinib has proven useful in treating certain cases of acute lymphoblastic leukemia that are associated with the "ETV6-NTRK3" fusion gene while larotrectinib and entrectinib have been useful in treating various cancers (e.g. a metastatic sarcoma, papillary thyroid cancer, non-small-cell lung carcinoma, gastrointestinal stromal tumor, mammary analog secretory carcinoma, and colorectal cancer) that are driven by mutated, overly active tyrosine kinases. Relevant to this issue, a 16-month-old girl with infantile fibrosarcoma harboring the "ETV6–NTRK3" fusion gene was successfully trated with larotrectinib. The success of these drugs, howwever, will likely depend on the relative malignancy-promoting roles of ETV6-NTRK3 protein's tyrosine kinase activity, the lose of ETV6-related transcription activity accompanying formation of ETV6-NTRK3 protein, and the various trisomy chromosomes that populate mesoblastic nephroma.

In 2015 the first consensus guidelines for the diagnosis and treatment of chordoma were published in the Lancet Oncology.

In one study, the 10-year tumor free survival rate for sacral chordoma was 46%. Chondroid chordomas appear to have a more indolent clinical course.

In most cases, complete surgical resection followed by radiation therapy offers the best chance of long-term control. Incomplete resection of the primary tumor makes controlling the disease more difficult and increases the odds of recurrence. The decision whether complete or incomplete surgery should be performed primarily depends on the anatomical location of the tumor and its proximity to vital parts of the central nervous system.

Chordomas are relatively radioresistant, requiring high doses of radiation to be controlled. The proximity of chordomas to vital neurological structures such as the brain stem and nerves limits the dose of radiation that can safely be delivered. Therefore, highly focused radiation such as proton therapy and carbon ion therapy are more effective than conventional x-ray radiation.

There are no drugs currently approved to treat chordoma, however a clinical trial conducted in Italy using the PDGFR inhibitor Imatinib demonstrated a modest response in some chordoma patients. The same group in Italy found that the combination of imatinib and sirolimus caused a response in several patients whose tumors progressed on imatinib alone.

Brainstem glioma is an aggressive and dangerous cancer. Without treatment, the life expectancy is typically a few months from the time of diagnosis. With appropriate treatment, 37% survive more than one year, 20% survive 2 years. and 13% survive 3 years.This is not for all brainstem glioma, this statistic reflects DIPG. There are other brainstem gliomas.

The outcome for hemangioblastoma is very good, if surgical extraction of the tumor can be achieved; excision is possible in most cases and permanent neurologic deficit is uncommon and can be avoided altogether if the tumor is diagnosed and treated early. Persons with VHL syndrome have a bleaker prognosis than those who have sporadic tumors since those with VHL syndrome usually have more than one lesion.

TSC typically affects multiple organ systems and manifests differently in each patient and in different stages of the life course. Drug therapy, surgery, and other interventions can be effective in managing some of the manifestations and symptoms of TSC.

In the United States, the Food and Drug Administration has approved several drugs for managing some of the major manifestations of TSC. The antiepileptic medication vigabatrin was approved in 2009 for treatment of infantile spasms and was recommended as first-line therapy for infantile spasms in children with TSC by the 2012 International TSC Consensus Conference. Adrenocorticotropic hormone was approved in 2010 to treat infantile spasms. Everolimus was approved for treatment of TSC-related tumors in the brain (subependymal giant cell astrocytoma) in 2010 and in the kidneys (renal angiomyolipoma) in 2012. Everolimus also showed evidence of effectiveness at treating epilepsy in some people with TSC. In 2017, the European Commission approved everolimus for treatment of refractory partial-onset seizures associated with TSC.

Neurosurgical intervention may reduce the severity and frequency of seizures in TSC patients. Embolization and other surgical interventions can be used to treat renal angiomyolipoma with acute hemorrhage. Surgical treatments for symptoms of lymphangioleiomyomatosis (LAM) in adult TSC patients include pleurodesis to prevent pneumothorax and lung transplantation in the case of irreversible lung failure.

Other treatments that have been used to treat TSC manifestations and symptoms include a ketogenic diet for intractable epilepsy and pulmonary rehabilitation for LAM.