A meningioma is a tumor that develops from the meninges. The brain and spinal cord are surrounded by an envelope called the meninges. The outermost, thickest layer is called the dura mater. A second, thin, transparent membrane called the arachnoid doubles the dura mater. It is the origin of meningiomas. Between the arachnoid and the brain lies the cerebrospinal fluid (CSF).
Because meningiomas tend to grow towards the inside of the skull, they progressively compress the brain or spinal cord and nerves, and can cause neurological disorders.
The majority of meningiomas are non-cancerous, benign and slow-growing. However, some may be more aggressive and located in areas where resection is perilous.
Some people with meningiomas have no symptoms at all. The tumor may be discovered during a radiological examination (CT scan or MRI) carried out for another reason or for screening. This is called serendipity. This is an increasingly common method of discovery.
Symptoms usually appear gradually, and vary according to the location of the meningioma and the nerve structures it compresses.
Meningiomas can cause headaches, convulsions or epileptic seizures, weakness in the arm or leg, speech disorders, visual problems, abnormal sensations, personality changes, balance problems, dizziness, hearing loss, loss of smell…..
Meningiomas are classified into several grades. Treatment varies according to this grade, defined by analysis of a sample obtained at the time of surgery.
- Grade I: Benign meningiomas. They are slow-growing. If the meningioma doesn’t cause symptoms, it’s often more sensible to monitor it with a regular MRI or CT scan before treating it. Some meningiomas do not grow. They can also sometimes regress spontaneously when a hormonal treatment (progestogen therapy) that favored their growth is stopped.
- Grade II: Atypical meningiomas. They are more aggressive, with a higher risk of recurrence once the meningioma has been removed. Some grade II meningiomas require radiotherapy after surgery.
- Grade III: Malignant meningiomas. They are the most aggressive, but account for less than 1% of all meningiomas. It’s a rare, serious pathology, and surgery is always followed by radiotherapy. The risk of recurrence is high.
- Meningiomas are named according to their location (frontal meningioma, temporal meningioma, cavernous sinus meningioma ….) and can cause a wide variety of symptoms depending on their exact location.
Meningiomas of the skull base, located beneath the brain, are among the most complicated to treat, due to their deep location and the difficulty of accessing them. These meningiomas require specific advanced surgical techniques to access them, and are one of our department’s specialties.
The precise cause and mechanisms of meningioma development are not known, but a number of risk factors have been clearly identified. Certain factors can be considered as the cause of meningioma, even if the mechanisms are not clearly elucidated.
- One clearly established risk factor is exposure to ionizing radiation. Indeed, people who, for other reasons, have had cranial radiotherapy , particularly in childhood, are at greater risk of developing one or more meningiomas.
- The presence of certain genes inherited from ancestors
- Certain genetic diseases such as neurofibromatosis
- Hormonal factors. Meningiomas are more common in women. Their growth can accelerate during pregnancy, and they can sometimes decrease in size after delivery.
Among hormonal factors, it has now been proven that taking progestin-only drugs promotes meningiomas.
Progestins are drugs used in gynecological diseases (endometriosis, uterine fibroids, particularly long and/or heavy periods, cycle disorders), in hormone replacement therapy (including menopause), as oral contraceptives or in intrauterine devices, but also in obstetrics (infertility due to luteal insufficiency, recurrent miscarriages).
Certain progestogens, such as cyproterone acetate (Androcur*), Nomegestrol acetate (Lutenyl*), chlormadinone acetate (Luteran*) may be the cause. Many other progestogens play a role in the genesis and growth of meningiomas to varying degrees. It is possible that some patients are more likely than others to develop one or more meningiomas under the influence of these progestin treatments. This is currently the subject of a great deal of research.
Our team was the first to identify the risk of meningioma withcyproterone acetate.
- Does acetate promote meningiomatosis? 56th Congress of the French National Society of Internal Medicine
- Does cyproterone acetate promote multiple meningiomas?10th European Congress of Endocrinology
as well as the risk of meningioma withnormegestrol acetate (link to publication publication).
This has been one of our department’s major research topics since the arrival of Prof. Sébastien Froelich in 2011. This research has been the subject of numerous publications, and is carried out in close collaboration with the Epi-phare agency and theANSM.
The use of some of these progestin treatments is now subject to very strict recommendations from the ANSM (Link).
- Androcur* and the risk of meningioma
- Lunenyl*, Luteran* and the risk of meningiomas
- Coprone*, depo-provera*, surgestone*
List of our publications :
- Prolonged use of nomegestrol acetate and risk of intracranial meningioma: a population-based cohort study Pierre Nguyen, Noémie Roland, Anke Neumann, Léa Hoisnard, Thibult Passeri, Lise Duranteau, Joël Coste, Sébastien Froelich, Mahmoud Zureik, AlainWeill
- Use of progestogens and the risk of intracranial meningioma: national case-control study. Roland N, Neumann A, Hoisnard L, Duranteau L, Froelich S, Zureik M, Weill A.BMJ. 2024 Mar 27;384:e078078.
- Opposed evolution of the osseous and soft parts of progestin-associated osteomeningioma after progestin intake discontinuation. Florea SM, Passeri T, Abbritti R, Bernat AL, Fontanel S, Yoldjian I, Funck-Brentano T, Weill A, Mandonnet E, Froelich S.J Neurosurg. 2023 Mar 3;139(4):944-952
- Risk of intracranial meningioma with three potent progestogens: A population-based case-control study. Hoisnard L, Laanani M, Passeri T, Duranteau L, Coste J, Zureik M, Froelich S, Weill A.Eur J Neurol. 2022 Sep;29(9):2801-2809.
- The tumours and the three bumps. Bousson V, Guichard JP, Froelich S, Orcel P.Lancet Oncol. 2022 Jan;23(1):e44.
- Atypical evolution of meningiomatosis after discontinuation of cyproterone acetate: clinical cases and histomolecular characterization. Passeri T, Giammattei L, Le Van T, Abbritti R, Perrier A, Wong J, Bourneix C, Polivka M, Adle-Biassette H, Bernat AL, Masliah-Planchon J, Mandonnet E, Froelich S.Acta Neurochir (Wien). 2022 Jan;164(1):265.
- Intracranial Meningiomas Decrease in Volume on Magnetic Resonance Imaging After Discontinuing Progestin. Voormolen EHJ, Champagne PO, Roca E, Giammattei L, Passeri T, di Russo P, Sanchez MM, Bernat AL, Yoldjian I, Fontanel S, Weill A, Mandonnet E, Froelich S.Neurosurgery. 2021 Jul 15;89(2):308-314.
- Use of high dose cyproterone acetate and risk of intracranial meningioma in women: cohort study. Weill A, Nguyen P, Labidi M, Cadier B, Passeri T, Duranteau L, Bernat AL, Yoldjian I, Fontanel S, Froelich S, Coste J.BMJ. 2021 Feb 3;372:n37.
- Cyproterone acetate and meningioma: a nationwide population-based study. Champeaux-Depond C, Weller J, Froelich S, Sartor A.J Neurooncol. 2021 Jan;151(2):331-338.
- Spontaneous regression of meningiomas after interruption of nomegestrol acetate: a series of three patients. Passeri T, Champagne PO, Bernat AL, Hanakita S, Salle H, Mandonnet E, Froelich S. Acta Neurochir (Wien). 2019 Apr;161(4):761-765.
- Combined hormonal influence of cyproterone acetate and nomegestrol acetate on meningioma: a case report. Champagne PO, Passeri T, Froelich S.Acta Neurochir (Wien). 2019 Mar;161(3):589-592.
- Regression of Giant Olfactory Groove Meningioma and Complete Visual Acuity Recovery after Discontinuation of Cyproterone Acetate. Bernat AL, Bonnin S, Labidi M, Aldahak N, Bresson D, Bouazza S, Froelich S.J Ophthalmic Vis Res. 2018 Jul-Sep;13(3):355-358.
- Growth stabilization and regression of meningiomas after discontinuation of cyproterone acetate: a case series of 12 patients. Bernat AL, Oyama K, Hamdi S, Mandonnet E, Vexiau D, Pocard M, George B, Froelich S.Acta Neurochir (Wien). 2015 Oct;157(10):1741-6.
- Regression of meningiomas after discontinuation of cyproterone acetate in a transsexual patient. Cebula H, Pham TQ, Boyer P, Froelich S.Acta Neurochir (Wien). 2010 Nov;152(11):1955-6.
- Does cyproterone acetate promote meningiomatosis?S. Froelich, Q.T. Pham, N. Fontaine, P. Boyer, P. Kehrli, D. Maitrot, J. Auwerx, J.L. Schlienger 56th Congress of the French National Society of Internal Medicine
- Does cyproterone acetate promote multiple meningiomas?10th European Congress of Endocrinology. Sebastien Froelich , Nassim Dali-Youcef , Patrick Boyer , Pierre Kehrli , Daniel Maitrot , Johan Auwerx & ,10th European Congress of Endocrinology
Podcast on France culture – Androcur and meningiomas
https://www.radiofrance.fr/franceculture/podcasts/la-science-cqfd/androcur-4865893
Who’s concerned?
Meningiomas account for around 36% of all primary brain tumors. They most often occur between the ages of 40 and 70. They are rare in children.
How is the diagnosis made?
The lesion is discovered on CT scan or MRI , prescribed in the presence of symptoms suggesting a cerebral or spinal cord origin. As meningiomas are very characteristic, these examinations often provide a virtually certain diagnosis. MRI is the most important examination for determining the characteristics of the meningioma.
What treatments are available?
Because they grow slowly, they don’t necessarily need to be treated, and monitoring can be a temporary and sometimes permanent solution. This is the case for meningiomas caused by certain hormone treatments such as cyproterone acetate. When treatment is discontinued, the meningioma usually stabilizes or regresses, and no further treatment is required. Simple monitoring is enough.
If they need to be treated, the options are surgery and radiotherapy. The proposed treatment will depend on the type of disorder caused by the meningioma, the patient’s age and state of health, and the location and size of the meningioma.
Because meningiomas develop slowly, elderly patients with few or no symptoms can simply be monitored by regular MRI scans.
Surgery
Surgical removal is the most common treatment for meningiomas that cause symptoms.
The risks of surgery vary according to the location of the tumor. When deciding whether to operate, the risks of surgery should be considered lower than those associated with the natural evolution of the meningioma; it is therefore sometimes preferable to simply monitor the disease and let it evolve. Some operations are very complex, when the meningioma is located in more threatening, deeper areas such as the base of the skull.
Technological advances have improved the surgeon’s ability to locate the tumour precisely, define its boundaries and avoid touching vital, functional areas of the brain. They enable safer, less invasive interventions.
Radiotherapy
Radiotherapy is indicated when removal of the meningioma is incomplete and the tumor grows back, when there is a significant risk of regrowth after surgery (Grade II or III meningioma), when the meningioma is inoperable due to its location or the frailty of the patient’s state of health or age, or when the meningioma is small and lends itself well to radiosurgical treatment (single-dose stereotactic radiotherapy).
The effects of radiotherapy are not immediate, but occur over time. The tumor will stop growing and, in some cases, shrink in size. Exceptionally, it disappears.
There are two techniques for delivering radiation: a single high dose (stereotactic radiosurgery) or several low doses (fractionated radiotherapy).
Radiosurgery (Gamma Knife, Cyberknife, Novalis) delivers a high dose of radiation in one or more sessions. Although the word surgery is used in radiosurgery, no incisions are made. It’s because the precision of irradiation is similar to surgery that it’s been given this name.
For this treatment, the head must be completely immobilized either by a frame fixed to the head or by a custom-made face mask.
Fractional radiotherapy takes place over 5 to 6 weeks, with a session every day of the week, with a rest at the weekend. A mask is used to precisely locate the tumor. By delivering only a fraction of the total radiation dose each day, normal cells can repair themselves between treatments. This reduces side effects.
Find out more about radiotherapy
Monitoring
After treatment, whether by surgery or radiotherapy, the patient is monitored regularly, and must have regular MRI checks for many years. Although the risk of recurrence diminishes over time, it can still occur long after treatment, and it’s important not to miss out.
Search
- Clinical research: Data on patients treated in our department are studied to better understand these tumors and evaluate the results of the treatments we offer. Our department is particularly interested in the links between meningiomas and exogenous sex hormone treatments (contraceptive pills, hormone replacement therapy for menopause, hormone treatments for gynecological pathologies such as endometriosis).
- Basic research: Small fragments of all meningiomas operated on in our department are stored for research purposes. Preserving these small pieces of tumor is essential to understanding the origin of these tumors and developing new treatments. This storage of part of the meningioma removed at the time of the operation is only done after the patient has been informed and agreed. Consent is given to patients before each procedure. These samples are stored in the biobank at the Lariboisière Hospital’s CRB (Biological Resource Centre).