What is an Atypical Meningioma? (with pictures)

Greg Caramenico
Greg Caramenico
Headaches may occur in those with an atypical meningioma.
Headaches may occur in those with an atypical meningioma.

Meningiomas are tumors of the meninges, the membrane that covers the brain and spinal cord. Most commonly, these are benign meningiomas, or grade I meningiomas, which stay small and do not grow indefinitely like cancerous tumors. An atypical meningioma, also called a grade II meningioma, is a meningeal tumor that could be malignant and grow back after treatment or removal. A grade III meningioma grows aggressively, threatening to compress neighboring brain tissue and invade bone. Even a benign meningioma can be dangerous because of its closeness to the brain.

Meningiomas are one of the most common forms of brain tumors, and account for about 30 percent of all such cancers.
Meningiomas are one of the most common forms of brain tumors, and account for about 30 percent of all such cancers.

One of the most common forms of brain tumors, meningiomas account for about 30 percent of all such cancers. Diagnosis of meningiomas is most often made through magnetic resonance imaging (MRI). Patients who have symptoms will have undergone a neurological examination first, and then brain imaging to determine whether they have a brain tumor. In asymptomatic patients, diagnosis sometimes happens during brain imaging that was requested for another medical complaint.

Meningioma tumors are responsible for approximately one-third of all brain tumors and cancers.
Meningioma tumors are responsible for approximately one-third of all brain tumors and cancers.

Symptomatic patients often have the atypical or malignant varieties. They report neurological symptoms such as headaches, seizures, loss of sensation, nausea, blurred vision and sensitivity to light. They can develop almost anywhere in the nervous system, but most meningiomas occur in the head, often near the brain stem, base of the skull, venous sinus or optic nerve sheath.

The spinal cord is normally enclosed within membranes called meninges, which protect and cushion long nerves.
The spinal cord is normally enclosed within membranes called meninges, which protect and cushion long nerves.

An atypical meningioma is more common in women of middle age or above and is rare in children; spinal meningiomas are seldom found in men. They have a faster growth rate than grade I tumors, but they grow less rapid than clearly malignant ones. Grade II meningiomas comprise less than 10 percent of total incidence. Malignant grade II cases are roughly about 2 percent.

An atypical meningioma can result in blurred vision and other neurological problems.
An atypical meningioma can result in blurred vision and other neurological problems.

Scientists have linked the onset of maningiomas to a mutation in a gene that codes for a tumor-suppressor protein. Severe, congenital mutations of this gene cause a syndrome where patients develop multiple benign tumors. Other mutations lead to a greater risk of benign or atypical meningioma. A small percentage of cases have been linked to a hereditary form of the disease; most children who develop grade II meningiomas fall into this category. Grade II and grade III meningiomas in children are often quite serious and rapidly progressing.

Meningioma tumors are often diagnosed through brain imaging such as an MRI scan.
Meningioma tumors are often diagnosed through brain imaging such as an MRI scan.

An atypical meningioma might require treatment to prevent further growth. Any tumor causing symptoms in the patient is usually treated as though it were malignant. Treatment involves surgical removal and follow-up with radiation therapy to kill remaining tumor cells.

The decision to treat the tumor is weighed against its growth, present and future risks to brain tissue, age and health of the patient, and the possible consequences of the surgery such as infection or cerebral edema. Sometimes the shock of radiation and surgery might be greater problems than the risk imposed by a slow-growing tumor. For instance, in elderly patients who don't have fast-growing tumors, regular observation is often preferred as treatment, because surgery might present more life-threatening complications than the tumors themselves. A grade I tumor seldom returns if it has been surgically removed, but an atypical meningioma has a higher risk of recurrence.

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Discussion Comments

anon1004267

I have Atypical meningioma. It was discovered April 14, 2015. I had a craniotomy April 29, 2015. The surgeon removed tumor about size of ping-pong ball. He left remnant about 5mm because it was too close to right optic nerve. I had MRI's every 6 months. I had 5 radiation treatments with Cyberknife in Jan 2016. tumor didn't stop growing had 5 more treatments with Cyberknife in Jan 2017. Tumor turned into several. Had surgery again on July 30, 2020. Now I have 9 tumors. Most are behind my eyes close to the optic nerves, behind my nose, forehead and front of my brain. I have one tumor in each of my carotids. I have seen about seven doctors in my fight with these tumors, but not a single one told me the following words: If you don't remove the tumor pressing on your right optic nerve you will lose your sight. Sure enough, this past November, I lost my sight on my right eye. The Dr. that operated on me last July caused Ptosis on my left eyelid, it means that I can't open my left eye, so I am practically blind. So if any person is reading this be careful if your tumor is close or on your optic nerve find a neurosurgeon good enough to remove the tumor. I start chemo on Jan. 8, 2021

anon998453

At age 53, I was diagnosed with a small meningioma, told it was benign and not causing my symptoms. My symptoms were left facial seizures, left eye pain and some intermittent blurred vision. A second opinion was the same as above. I insisted we remove the tumor, Feb of 2017. The surgery is labeled a success, GTR, no radiation at this time. Symptoms have subsided about 90%. Note - the tumor was not benign; it was atypical. Glad I insisted we remove it. Very scary. Surgeon says prognosis very good with low probability of recurrence, but all you read states otherwise. Confusing indeed.

anon990360

My daughter was diagnosed with grade II atypical giant meningioma at age 2. She had surgery only. She has survived 11 years. I understand the mean recurrence is 10.9 years. The surgeon says she is 'cured''; he doesn't talk about statistics. We have sent her frozen sample to Germany for cytogenic analysis just in case we need to enroll her in a sympathetic ground clinical trial, should the statistics be correct and her tumor 1p36.

anon290330

My son was diagnosed age 11 with a grade II atypical meningioma, and he had 90 percent of it surgically removed. Two years later he had stereotactic radiation. We are unfortunate that two of his specialists have very different ideas on the extent of the tumor and prognosis. My son is 16 years old now.

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    • Headaches may occur in those with an atypical meningioma.
      Headaches may occur in those with an atypical meningioma.
    • Meningiomas are one of the most common forms of brain tumors, and account for about 30 percent of all such cancers.
      Meningiomas are one of the most common forms of brain tumors, and account for about 30 percent of all such cancers.
    • Meningioma tumors are responsible for approximately one-third of all brain tumors and cancers.
      Meningioma tumors are responsible for approximately one-third of all brain tumors and cancers.
    • The spinal cord is normally enclosed within membranes called meninges, which protect and cushion long nerves.
      The spinal cord is normally enclosed within membranes called meninges, which protect and cushion long nerves.
    • An atypical meningioma can result in blurred vision and other neurological problems.
      An atypical meningioma can result in blurred vision and other neurological problems.
    • Meningioma tumors are often diagnosed through brain imaging such as an MRI scan.
      Meningioma tumors are often diagnosed through brain imaging such as an MRI scan.
    • An atypical meningioma may be treated with surgery, depending on how big of a risk the tumor poses to a person's health.
      An atypical meningioma may be treated with surgery, depending on how big of a risk the tumor poses to a person's health.
    • Meningiomas are tumors on the membrane that covers the brain and spinal cord.
      Meningiomas are tumors on the membrane that covers the brain and spinal cord.