By Jason Wasserman MD PhD FRCPC
April 27, 2026
A meningioma is a type of tumor that develops from cells in the meninges, the thin layers of tissue that cover and protect the brain and spinal cord. More specifically, meningiomas arise from meningothelial cells in the arachnoid mater, one of the protective layers. Most meningiomas are well-circumscribed, meaning the tumor has a clear border with the brain and is attached to the dura, the tough outer layer of the meninges that lines the inside of the skull. Because of this dural attachment, meningiomas are often described as “dural-based” or “extra-axial” tumors, meaning they sit on the surface of the brain rather than within it. This distinction has practical importance: meningiomas are usually separable from the brain at surgery and can often be completely removed, which is one of the main reasons most meningiomas have an excellent outlook.
Meningioma is the most common primary brain tumor in adults, accounting for more than one-third of all tumors that arise in the central nervous system. Most meningiomas grow slowly and are not cancerous, but a smaller number grow more quickly, are more likely to come back after treatment, or behave in ways that resemble cancer. The World Health Organization (WHO) classifies meningiomas as grade 1, grade 2, or grade 3 based on how the tumor looks under the microscope and on certain molecular findings. Each grade is covered in more detail below, and a dedicated article is available for atypical meningioma (WHO grade 2).
This article will help you understand the findings in your pathology report — what each term means and why it matters for your care.
The symptoms of meningioma depend on where the tumor is growing, its size, and how quickly it grows. Because most meningiomas grow slowly, symptoms often develop gradually over months or years. Many meningiomas are discovered incidentally when imaging is performed for an unrelated reason, such as after a head injury or for long-standing headaches.
Common symptoms include:
Meningiomas can develop anywhere along the meninges. The location often determines the symptoms and the complexity of surgery. Common locations include:
In rare cases, meningiomas can arise outside the central nervous system, such as in the lung. These extracranial meningiomas are unusual but recognized.
For most people diagnosed with meningioma, the exact cause is not known. The tumor develops through a series of genetic changes that accumulate in meningothelial cells over time. Several factors are known to increase the risk:
A small number of meningiomas develop in the setting of an inherited condition. Inherited conditions are caused by a genetic change that is present in every cell of the body from birth and can be passed from parent to child. Inherited conditions associated with meningioma include:
Most meningiomas are sporadic, meaning they have no clear inherited cause. Genetic counseling and germline (inherited) genetic testing may be recommended when a patient has multiple meningiomas, when a meningioma is diagnosed at an unusually young age, when a meningioma occurs alongside other tumors typical of an inherited syndrome, or when there is a strong family history.
Meningioma is the most common primary brain tumor in adults, accounting for more than one-third of all primary tumors of the central nervous system. The yearly incidence is approximately 9 cases per 100,000 people. Meningioma becomes more common with age, with a median age at diagnosis of about 66 years. Meningiomas are about twice as common in women as in men, particularly for grade 1 tumors. They are uncommon in children and young adults; when they do occur in younger patients, an inherited condition such as NF2 or SMARCE1-associated syndrome is more likely.
The diagnosis of meningioma usually begins when imaging of the brain or spine — most often magnetic resonance imaging (MRI) — reveals a mass. Meningiomas have several characteristic imaging features that often allow doctors to recognize them with high confidence even before surgery. They typically appear as well-defined, solid masses attached to the dura, with bright, uniform enhancement after intravenous contrast administration. A characteristic finding called the “dural tail” — where the contrast enhancement extends along the dura beyond the main tumor — is highly suggestive of meningioma. Calcifications (calcium deposits) within the tumor are common and are often easier to see on CT than on MRI. Some meningiomas, particularly certain subtypes, cause significant swelling (edema) in the surrounding brain. Although these imaging features can strongly suggest the diagnosis, a tissue sample is needed to confirm the diagnosis, identify the subtype, and assign the WHO grade.
The diagnosis is confirmed after a tissue sample is examined under the microscope by a pathologist. In most cases, the tissue is obtained during surgery to remove the tumor. A neurosurgeon opens the skull through an operation called a craniotomy and removes as much of the tumor as can be safely taken out, along with the affected dura when possible. When the tumor is in a location where surgery would be too risky — for example, deep at the skull base or near critical nerves and blood vessels — a smaller biopsy may be performed instead. Some meningiomas are followed with imaging without ever being biopsied or removed, particularly when they are small, asymptomatic, and incidentally found.
Under the microscope, meningiomas are tumors of meningothelial cells with round or oval nuclei and a moderate amount of cytoplasm. The cells are often arranged in characteristic whorls (circular swirls) and may contain psammoma bodies, which are small, layered calcifications. The microscopic appearance can vary considerably between subtypes, but most meningiomas are recognizable to a pathologist by their architecture and cell features.
To confirm the diagnosis, the pathologist uses immunohistochemistry, a laboratory test that uses antibodies to detect specific proteins in the tumor cells. Several markers are useful in confirming a meningioma and distinguishing it from other tumors that can occur in similar locations:
The tumor grade and histologic subtype are determined by combining microscopic and molecular findings, as described in the next sections.
Under the microscope, meningiomas can have different patterns of cell arrangement and appearance, called histologic subtypes. Most subtypes are considered WHO grade 1, but two subtypes — chordoid and clear cell — are automatically classified as at least WHO grade 2 because they are more likely to recur after treatment. The remaining subtypes are graded based on additional features described in the WHO grade section below.
Meningothelial meningioma is the most common subtype. The tumor cells are arranged in lobules, and the borders between cells are often hard to see, giving the tumor a flowing or syncytial appearance. The nuclei may have small clear areas (called intranuclear pseudoinclusions) caused by folds of cytoplasm extending into the nucleus. Whorls and psammoma bodies are less common in this subtype than in some others. Meningothelial meningiomas are usually grade 1 and have an excellent outlook when completely removed.
Fibrous meningioma is made up of spindle-shaped cells arranged in bundles within a collagen-rich background, giving the tumor a firm, fibrous texture. Whorls and psammoma bodies may be present. This subtype is usually grade 1 and has a low risk of coming back after complete removal.
Transitional meningioma shows features of both meningothelial and fibrous meningiomas — hence the name. Whorls and psammoma bodies are common. Most transitional meningiomas are grade 1 and behave like other benign meningiomas.
Psammomatous meningioma is composed of numerous psammoma bodies (rounded calcifications), with relatively few tumor cells between them. This subtype most often occurs in the spine, particularly in middle-aged or older women, and is almost always grade 1.
Angiomatous meningioma contains a large number of blood vessels, with meningioma cells squeezed between them. The blood vessels may be thick-walled. This subtype is grade 1 but is often associated with significant brain swelling around the tumor, which can produce more dramatic symptoms than the tumor’s grade alone would suggest.
Microcystic meningioma has many small fluid-filled spaces between tumor cells, giving a loose, spongy appearance. Like angiomatous meningioma, it is grade 1 but can be associated with prominent brain swelling.
Secretory meningioma contains small gland-like spaces filled with bright pink protein deposits called pseudopsammoma bodies. These tumors often cause significant brain swelling out of proportion to their grade. Despite alarming imaging findings, they are grade 1 and usually have an excellent outlook after complete removal.
Lymphoplasmacyte-rich meningioma contains a heavy infiltrate of inflammatory cells (lymphocytes and plasma cells) that can almost overshadow the tumor cells themselves. This rare subtype is grade 1 and can sometimes be confused with an inflammatory condition.
Metaplastic meningioma contains areas where tumor cells have changed to resemble other tissue types — bone, cartilage, fat, or foamy (xanthomatous) cells. These changes are not aggressive features, and the tumor is grade 1.
Chordoid meningioma has tumor cells arranged in cords within a mucin-rich (gelatinous) background. The pattern resembles another tumor type called chordoma, which is the source of the name. Chordoid meningiomas are automatically classified as at least WHO grade 2 because they are more likely to come back after treatment.
Clear cell meningioma is composed of cells with clear cytoplasm due to the accumulation of glycogen (a stored form of sugar) within the cells. These tumors most often occur in the cerebellopontine angle (a space at the back of the skull) or in the spinal canal, and tend to affect younger patients. Many clear cell meningiomas are caused by an inherited change in the SMARCE1 gene. They are automatically classified as at least WHO grade 2.
“Atypical” meningioma is the term used for any meningioma that meets the criteria for WHO grade 2 (other than chordoid or clear cell, which are grade 2 by subtype alone). “Anaplastic” meningioma was historically the term for meningiomas that met the criteria for WHO grade 3, although the term is now less commonly used, as molecular criteria can also assign grade 3. Both are described in detail in the WHO grade section below. Two additional rare patterns — rhabdoid and papillary meningioma — were previously automatically classified as grade 3 but are now graded according to the standard criteria. They can be grade 1, 2, or 3 depending on their other features.
The World Health Organization (WHO) assigns tumors of the central nervous system a grade from 1 to 4 that reflects how the tumor is expected to behave. Meningiomas are assigned grades 1, 2, or 3; there is no grade 4 meningioma. The grade is one of the most important pieces of information in the pathology report because it predicts how likely the tumor is to come back after treatment and influences whether radiation therapy is recommended in addition to surgery. It is important to understand that even a grade 1 meningioma is not necessarily harmless, because of the location of these tumors, even slow-growing meningiomas can compress vital brain structures and require treatment.
Grade 1 meningiomas are the most common (about 80% of meningiomas) and the least aggressive. Under the microscope, they have low mitotic activity (fewer than 2.5 mitotic figures per square millimeter, an updated standardized measurement introduced by the WHO in 2021) and do not show the aggressive features required for higher grades. Most histologic subtypes — meningothelial, fibrous, transitional, psammomatous, angiomatous, microcystic, secretory, lymphoplasmacyte-rich, and metaplastic — are usually grade 1. Grade 1 meningiomas have the lowest risk of recurrence, especially when completely removed. Ten-year survival is approximately 95%. Recurrence rates depend on the Simpson grade of resection: roughly 10% with a Simpson grade 1 resection, rising to 40% or higher with Simpson grade 4 (partial) resections.
Grade 2 meningiomas (about 15–30% of meningiomas) tend to grow more aggressively and have a higher risk of recurrence. A meningioma is classified as grade 2 if it meets any of the following criteria: increased mitotic activity (between 2.5 and 12.5 mitotic figures per square millimeter), brain invasion, or a combination of at least three out of five specific microscopic features (high cellularity, small cells with a high nucleus-to-cytoplasm ratio, prominent nucleoli, sheet-like growth without architecture, or spontaneous necrosis). Two histologic subtypes — chordoid and clear cell — are automatically classified as at least grade 2 even when they do not meet any of these criteria. Patients with grade 2 meningiomas often need closer follow-up after surgery, and radiation therapy may be recommended, particularly when the tumor cannot be completely removed. Ten-year survival is approximately 90%. A detailed article on atypical meningioma is available.
Grade 3 meningiomas are the rarest (1–3% of meningiomas) and the most aggressive. A meningioma is classified as grade 3 if it shows obvious malignant features under the microscope (high mitotic activity at 12.5 or more mitotic figures per square millimeter, marked nuclear atypia, and often brain invasion and necrosis). Importantly, the 2021 WHO classification also allows certain molecular changes to assign grade 3 even when the microscopic features would suggest a lower grade. The two molecular criteria for grade 3 are a TERT promoter mutation and homozygous deletion of CDKN2A and/or CDKN2B. These molecular findings are described in the biomarker section below. Grade 3 meningiomas have a high risk of coming back, may spread within the brain, and rarely metastasize outside the central nervous system. Treatment usually combines surgery with radiation therapy. Ten-year survival is approximately 30%, although outcomes vary significantly by molecular profile and extent of resection.
For meningioma, the extent of surgical removal is one of the most important factors influencing the chance of cure and the risk of the tumor coming back. The pathology and operative reports often refer to the Simpson grade, a five-point scale developed in 1957 that is still widely used:
Simpson grades 1 and 2 are considered the most complete and are associated with the lowest risk of recurrence. Higher Simpson grades are associated with progressively higher recurrence risk. The pathology report will typically describe whether margins are clear of tumor, whether the dura is involved, and whether bone invasion is present. These findings, combined with the WHO grade, guide the decision about whether radiation therapy is needed after surgery.
Molecular testing is increasingly important for meningiomas, especially for grade 2 and grade 3 tumors and for any meningioma that recurs or behaves more aggressively than expected. Molecular results help refine the grade, predict the risk of recurrence, identify tumors associated with inherited syndromes, and identify potential targets for clinical trials.
The NF2 gene is a tumor suppressor gene located on chromosome 22 that helps control cell growth. Loss or mutation of NF2 is the most common genetic change in meningiomas, particularly in tumors arising over the cerebral convexities and in the spinal canal. NF2 alterations are an early event in tumor development. When multiple meningiomas are found, or when a meningioma occurs in a younger patient, germline testing for NF2 may be recommended to evaluate for neurofibromatosis type 2.
These genes are involved in cell growth and signaling pathways. Mutations in these genes are common in skull-base meningiomas, particularly in the meningothelial and secretory subtypes, and tend to occur in tumors without NF2 mutations. AKT1 p.E17K mutations are particularly common in meningothelial meningiomas of the skull base. SMO mutations are associated with olfactory groove meningiomas. KLF4 mutations are characteristic of secretory meningioma. These mutations are detected using next-generation sequencing. Their identification can help confirm the diagnosis and may identify patients eligible for targeted therapy clinical trials.
The TERT gene makes a protein that lengthens telomeres, the protective caps on the ends of chromosomes. Mutations in the TERT promoter (a regulatory region that controls whether the gene is turned on) increase telomerase activity and allow tumor cells to divide indefinitely. TERT promoter mutations are uncommon overall (about 6% of meningiomas) but are enriched in higher-grade tumors and are associated with significantly shorter time to recurrence and worse overall survival. The presence of a TERT promoter mutation automatically classifies the meningioma as WHO grade 3, even when the microscopic features would otherwise suggest a lower grade. Testing is performed using targeted DNA sequencing.
CDKN2A and CDKN2B are tumor suppressor genes that slow cell division. When both copies of CDKN2A and/or CDKN2B are lost — a change called homozygous deletion — tumor cells grow more easily and behave more aggressively. Homozygous deletion of CDKN2A and/or CDKN2B automatically classifies the meningioma as WHO grade 3, even when the microscopic features would otherwise suggest a lower grade. Testing is typically performed using next-generation sequencing with copy-number analysis or fluorescence in situ hybridization (FISH). Loss of the p16 protein on immunohistochemistry can serve as a surrogate marker for CDKN2A deletion in higher-grade tumors.
The SMARCE1 gene is involved in chromatin remodeling — the process by which DNA is packaged and how genes are turned on or off. Loss of SMARCE1 is the defining genetic feature of clear cell meningioma. Loss of nuclear SMARCE1 protein on immunohistochemistry supports the diagnosis. Many clear cell meningiomas are associated with an inherited change in the SMARCE1 gene, particularly in younger patients, so germline testing and genetic counseling are often recommended when this diagnosis is made.
The BAP1 gene is another tumor suppressor gene involved in chromatin regulation. Loss of BAP1 is associated with rhabdoid meningioma and a small number of high-grade meningiomas. Loss of nuclear BAP1 protein on immunohistochemistry supports the diagnosis. BAP1 alterations may indicate an underlying BAP1 tumor predisposition syndrome, which also increases the risk of mesothelioma, melanoma of the eye and skin, and kidney cancer. Germline testing is recommended when BAP1 loss is identified.
DNA methylation refers to small chemical tags attached to DNA that help control which genes are turned on or off. Different tumor types have distinct methylation patterns, almost like a fingerprint. DNA methylation profiling compares a tumor’s pattern to a large reference database. For meningiomas, methylation profiling can not only confirm the diagnosis but also identify three molecular classes (often called methylation classes or “molecular grades”) that correlate with risk of recurrence — sometimes more accurately than WHO grade alone. This testing is increasingly used in specialized centers for cases where the WHO grade is borderline or where molecular risk stratification would change management.
For more information about biomarkers and molecular testing across all cancer types, visit the Biomarkers and Genetic Testing section.
The prognosis for meningioma depends on multiple factors, including the WHO grade, the histologic subtype, the tumor’s location, the extent of surgical removal, and the molecular features of the tumor. Most meningiomas have an excellent prognosis, particularly when they are grade 1 and can be completely removed.
Typical 10-year relative survival figures by grade are:
Several features influence the outlook:
Meningioma is managed by a multidisciplinary team that typically includes a neurosurgeon, a neuro-oncologist (or, for grade 1 tumors, often the neurosurgeon alone), a radiation oncologist, a neuropathologist, and a neuroradiologist. Other members of the team may include a neurologist for seizure management, a neuro-ophthalmologist for tumors near the optic nerves, an endocrinologist for tumors near the pituitary gland, and a geneticist or genetic counselor when an inherited condition is suspected.
Treatment depends on the tumor grade, its size and location, the patient’s symptoms, and the extent of surgical removal achieved.
Many small, asymptomatic meningiomas — particularly in older adults — do not require immediate treatment. These are followed with regular MRI scans, typically every 6–12 months at first and less often if the tumor remains stable. Observation is appropriate when the tumor is small, the patient has no symptoms, and the tumor is not in a location where growth would quickly cause problems. Many meningiomas grow very slowly or not at all, and some patients are followed safely for years or decades without ever needing treatment.
Surgery is the main treatment for symptomatic meningiomas, growing meningiomas, and meningiomas in locations where growth is likely to cause neurological symptoms. The goal is maximal safe removal, ideally a Simpson grade 1 or 2 resection, but this is not always possible, particularly for skull-base tumors that involve critical nerves and blood vessels. The pathology report will typically describe the tumor type, the WHO grade, the extent of resection, and any high-risk features.
Radiation therapy is used in several situations:
Radiation can be delivered in several ways. Stereotactic radiosurgery (using systems such as Gamma Knife or CyberKnife) delivers a single high dose to a precisely targeted area and is often used for small to medium-sized tumors. Fractionated radiation delivers smaller doses over multiple sessions and is often used for larger tumors or for tumors near critical structures.
Unlike many other cancers, meningiomas have no widely approved systemic therapy. Hormone therapy and standard chemotherapy have not been shown to be effective. For recurrent meningiomas that cannot be treated with further surgery or radiation, options sometimes include bevacizumab (a drug that blocks the formation of new blood vessels) and targeted therapies based on specific mutations identified in the tumor (for example, SMO inhibitors for SMO-mutant tumors). Clinical trials are an important option for patients with recurrent or aggressive meningiomas, and tumor-treating fields (TTFields) are being studied in clinical trials for grade 2 and grade 3 disease.
Long-term follow-up is essential for all meningiomas, even after complete removal. Follow-up typically includes regular MRI scans, with the interval determined by the WHO grade, the extent of resection, and the time since treatment. Long-term effects of the tumor and its treatment — including seizures, hormone problems (for tumors near the pituitary gland), vision changes (for tumors near the optic nerves), cognitive changes, and the effects of radiation — are managed by the multidisciplinary team. Neuropsychological testing, rehabilitation, mental health support, and seizure management are important parts of survivorship care for many patients.
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