Visual symptoms and treatment of periorbital meningioma

Receiving a diagnosis that affects your vision often raises many questions: what exactly does it mean, could it progress, how might it affect your quality of life, and what treatment options are available today? A perioptique meningioma is a type of meningioma that develops very close to the optic nerve, the nerve that carries visual information from the eye to the brain. Although many meningiomas are benign and slow-growing, in this location the position of the tumor can make a difference, because even small lesions can affect structures that are very sensitive to vision.

Next, we will look at what peripapillary meningioma is, what visual symptoms it can cause, how it is diagnosed, and what treatment options are considered in each case.

What is a peripapillary meningioma?

A meningioma is a tumor that originates in the meninges, the membranes that cover and protect the brain and spinal cord. In general, it is one of the most common primary tumors of the central nervous system and, in most cases, it is slow-growing.

When we talk about perioptique meningioma, we are referring to meningiomas found in the area around the optic nerve and nearby structures (for example, in the orbit or optic canal). In some cases, the tumor may originate in the meninges directly surrounding the optic nerve, which is known as optic nerve sheath meningioma.

Although many meningiomas are benign, in this location the priority is not only to control the tumor, but also to preserve visual function and minimize sequelae.

Anatomy of the optic nerve and its relationship to meningioma

The optic nerve transmits visual information from the retina to the brain. It is surrounded by supporting structures and a sheath related to the meninges. Therefore, some meningiomas can develop in the orbit (behind the eye), along the optic canal, or in areas near the base of the skull, and affect vision by:

Direct compression of the optic nerve, which reduces its function.
Alteration of blood supply and visual signal transmission.
Effect on neighboring structures involved in eye movement.

In practice, this explains why a slow-growing tumor can cause progressive visual symptoms.

Why is it called “periophtalmic”?

Periophthalmic literally means “around the optic nerve.” It is used to describe a location that determines both the symptoms and how treatment is planned, prioritizing the balance between tumor control and vision preservation.

Causes and risk factors of peripapillary meningioma

In many patients, no single cause is identified. In general terms, some risk factors associated with meningiomas (not specific to peripapillary meningiomas) are described, although most diagnoses cannot be explained by a single reason.

In summary:

Previous radiation in the area: having received radiation therapy to the head (which is uncommon) may increase the risk of meningiomas.
Rare genetic factors: there are rare hereditary syndromes that increase the predisposition to tumors of the central nervous system.
Hormonal factors: meningiomas are more common in women, and in some cases, a hormonal influence has been observed.

Therefore, the most important thing is a correct diagnosis, an individualized plan, and close follow-up.

Most common symptoms

Symptoms depend on the exact location and how much the optic nerve or nearby structures are affected. In peripapillary meningioma, visual symptoms are usually the most common reason for consultation.

Common signs:

Progressive vision loss (in one eye, or asymmetrical).
Decreased visual acuity or difficulty seeing details.
Changes in the visual field (blurred areas or areas without vision).
Double vision (diplopia) if structures that control eye movement are affected.
Proptosis (bulging eye) in orbital cases.
In some cases, orbital pain or headache, although this does not always occur.

These symptoms can have many causes. If they are persistent or progressive, it is advisable to consult a specialist to assess the need for tests.

Diagnostic methods

The diagnosis is based on clinical evaluation, ophthalmological examination, and imaging tests, and is tailored to each patient.

In general, the process includes:

Medical history and neuro-ophthalmological examination: assessment of visual acuity, visual field, pupils, and fundus, as well as associated symptoms.
Imaging tests: Magnetic resonance imaging (MRI) is usually the test of choice for studying the path of the optic nerve, the orbit, and the relationship of the tumor to nearby structures.
Computed tomography (CT): This can be useful in selected situations (e.g., to assess the bone of the optic canal or other structures).
Histological confirmation (in specific cases): this is not always necessary at the outset if the imaging appearance and location are highly suggestive.

Once the diagnosis has been confirmed, it is usual to draw up an individualized plan based on symptoms, size, growth, and visual status.

Available treatment options

The treatment of optic nerve meningioma (or periorbital meningioma) must be individualized. There is no single strategy that is valid for all patients, because the objective is usually twofold: tumor control and preservation of vision.

In general, the following are considered:

Observation and follow-up: in small tumors with stable vision and slow growth, a surveillance strategy with clinical check-ups and serial MRIs may be considered. This option avoids unnecessary treatment when the risk of progression is low.
Surgery (in selected cases): Surgery may be considered when the tumor is accessible and the risk/benefit balance is favorable. However, in lesions very close to the optic nerve, it may pose a significant risk to vision, so it is not always the first option.
Radiotherapy (conventional or fractionated stereotactic): in meningiomas of the optic nerve sheath and periorbital locations, radiotherapy has been used as an alternative or complement when seeking tumor control while preserving visual function, especially if there is progression.

High-precision technologies in radiotherapy/radiosurgery

There are advanced techniques that allow radiation to be concentrated on the tumor and minimize the dose to nearby healthy structures. In periorbital tumors, this is especially important due to the sensitivity of the optic nerve. The indication and number of sessions (one or more) are decided on a case-by-case basis, prioritizing safety and visual preservation.

At IRCA, we have ZAP-X, a non-invasive stereotactic brain radiosurgery system that allows treatment to be administered with great precision, seeking to protect the surrounding healthy structures as much as possible. The aim is to treat with an approach that prioritizes quality of life, always after a complete and individualized medical assessment.

For further information, please consult: Optic nerve meningioma.

SOURCES

National Cancer Institute (NCI). Meningioma.

https://www.cancer.gov/rare-brain-spine-tumor/espanol/tumores/meningioma

SciELO (Oncology, PDF). Optic nerve sheath meningioma (ONSM).

https://scielo.isciii.es/pdf/onco/v29n1/05.pdf

PIPER-SERAM. Perioptic meningioma: a rare orbital tumor.

https://www.piper.espacio-seram.com/index.php/seram/article/view/5695

National Cancer Institute (NCI). PDQ®. Treatment of CNS Tumors in Adults (definitions of radiosurgery/radiotherapy).

https://www.cancer.gov/espanol/tipos/cerebro/paciente/tratamiento-cerebro-adultos-pdq

ISCIII (PDF). ZAP-X® Gyroscopic Radiosurgery (technical document in Spanish).

https://repisalud.isciii.es/rest/api/core/bitstreams/04686463-661a-41ea-ac3d-bf7a57d4c3fb/content