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Glioblastoma

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Glioblastoma

Glioblastoma, previously known as glioblastoma multiforme (GBM), is the most aggressive and most common type of cancer that originates in the brain, and has a very poor prognosis for survival. Initial signs and symptoms of glioblastoma are nonspecific. They may include headaches, personality changes, nausea, and symptoms similar to those of a stroke. Symptoms often worsen rapidly and may progress to unconsciousness. The cause of most cases of glioblastoma is not known. Uncommon risk factors include genetic disorders, such as neurofibromatosis and Li–Fraumeni syndrome, and previous radiation therapy. Glioblastomas represent 15% of all brain tumors. They are thought to arise from astrocytes. The diagnosis typically is made by a combination of a CT scan, MRI scan, and tissue biopsy. There is no known method of preventing the cancer. Treatment usually involves surgery, after which chemotherapy and radiation therapy are used. The medication temozolomide has been generally advised as part of chemotherapy. However, clinical trial participation, if available, is preferred by current NCCN guidelines. High-dose steroids may be used to help reduce swelling and decrease symptoms. Surgical removal (decompression) of the tumor is linked to increased survival, but only by some months. Despite maximum treatment, the cancer almost always recurs. The typical duration of survival following diagnosis is 10–13 months, with fewer than 5–10% of people surviving longer than five years. Without treatment, survival is typically three months. It is the most common cancer that begins within the brain and the second-most common brain tumor, after meningioma, which is benign in most cases. About 3 in 100,000 people develop the disease per year. The average age at diagnosis is 64.

Infobox

Other names
Glioblastoma multiforme
Specialty
Neuro-oncology, neurosurgery
Symptoms
Initially nonspecific, headaches, personality changes, nausea, symptoms similar to a stroke
Usual onset
64 years old
Causes
Usually unclear
Risk factors
Genetic disorders (neurofibromatosis, Li–Fraumeni syndrome), previous radiation therapy
Diagnostic method
CT scan, MRI scan, tissue biopsy
Prevention
Unknown
Treatment
Surgery, chemotherapy, radiation
Medication
Temozolomide, corticosteroids
Prognosis
Life expectancy 12 months with treatment (5 year survival <10%)
Frequency
3 per 100,000 per year

Tables

Features of glioblastoma, IDH wildtype, WHO 4 · Pathogenesis › Glioblastoma classification
Cell of origin
Cell of origin
Synonyms
Cell of origin
Glioblastoma, GBM
Astrocyte
Median age at diagnosis
Median age at diagnosis
Synonyms
Median age at diagnosis
Glioblastoma, GBM
~62 years
Male:Female ratio
Male:Female ratio
Synonyms
Male:Female ratio
Glioblastoma, GBM
1 :1
Median length of clinical history at diagnosis
Median length of clinical history at diagnosis
Synonyms
Median length of clinical history at diagnosis
Glioblastoma, GBM
4 months
Median overall survival
Median overall survival
Synonyms
Median overall survival
Surgery + radiotherapy
Surgery + radiotherapy
Synonyms
Surgery + radiotherapy
Glioblastoma, GBM
9 months
Surgery + radiotherapy + chemotherapy
Surgery + radiotherapy + chemotherapy
Synonyms
Surgery + radiotherapy + chemotherapy
Glioblastoma, GBM
15 months
Location
Location
Synonyms
Location
Glioblastoma, GBM
Usually supratentorial, rarely cerebellum or spine
Necrosis and microvascular proliferation
Necrosis and microvascular proliferation
Synonyms
Necrosis and microvascular proliferation
Glioblastoma, GBM
Extensive
Associated molecular/genetic mutations
Associated molecular/genetic mutations
Synonyms
Associated molecular/genetic mutations
Glioblastoma, GBM
TERT promoter mutation, combined gain of chromosome 7 and loss of chromosome 10; EGFR amplification
Synonyms
Glioblastoma, GBM
Cell of origin
Astrocyte
Median age at diagnosis
~62 years
Male:Female ratio
1 :1
Median length of clinical history at diagnosis
4 months
Median overall survival
Surgery + radiotherapy
9 months
Surgery + radiotherapy + chemotherapy
15 months
Location
Usually supratentorial, rarely cerebellum or spine
Necrosis and microvascular proliferation
Extensive
Associated molecular/genetic mutations
TERT promoter mutation, combined gain of chromosome 7 and loss of chromosome 10; EGFR amplification
· Prognosis
III
III
Recursive partitioning analysis (RPA) class
III
Definition
Age < 50, KPS ≥ 90
Historical Median Survival Time
17 months
Historical 1-Year Survival
70%
Historical 3-Year Survival
20%
Historical 5-Year Survival
14%
IV
IV
Recursive partitioning analysis (RPA) class
IV
Definition
Age < 50, KPS < 90
Historical Median Survival Time
11 months
Historical 1-Year Survival
46%
Historical 3-Year Survival
7%
Historical 5-Year Survival
4%
Age ≥ 50, KPS ≥ 70, surgical removal with good neurologic function
Age ≥ 50, KPS ≥ 70, surgical removal with good neurologic function
Recursive partitioning analysis (RPA) class
Age ≥ 50, KPS ≥ 70, surgical removal with good neurologic function
V + VI
V + VI
Recursive partitioning analysis (RPA) class
V + VI
Definition
Age ≥ 50, KPS ≥ 70, surgical removal with poor neurologic function
Historical Median Survival Time
7 months
Historical 1-Year Survival
28%
Historical 3-Year Survival
1%
Historical 5-Year Survival
0%
Age ≥ 50, KPS ≥ 70, no surgical removal
Age ≥ 50, KPS ≥ 70, no surgical removal
Recursive partitioning analysis (RPA) class
Age ≥ 50, KPS ≥ 70, no surgical removal
Age ≥ 50, KPS < 70
Age ≥ 50, KPS < 70
Recursive partitioning analysis (RPA) class
Age ≥ 50, KPS < 70
Recursive partitioning analysis (RPA) class
Definition
Historical Median Survival Time
Historical 1-Year Survival
Historical 3-Year Survival
Historical 5-Year Survival
III
Age < 50, KPS ≥ 90
17 months
70%
20%
14%
IV
Age < 50, KPS < 90
11 months
46%
7%
4%
Age ≥ 50, KPS ≥ 70, surgical removal with good neurologic function
V + VI
Age ≥ 50, KPS ≥ 70, surgical removal with poor neurologic function
7 months
28%
1%
0%
Age ≥ 50, KPS ≥ 70, no surgical removal
Age ≥ 50, KPS < 70
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