Malignant Orbital Tumors

What is Malignant Orbital Tumors?

The orbits are the areas in your face that hold and protect your eyes, including the muscles, nerves, blood vessels, tear systems, and fat tissues around your eyes. They are separated by your nose and sinuses and made up by parts of seven different bones. Tumors, or abnormal growths, can sometimes form in the orbit.

These tumors can be categorized based on where they originally formed. Primary tumors start growing in the actual structures inside the orbit, such as the blood vessels, nerves, muscles, bones, or the tear gland located here. Secondary tumors, on the other hand, originally start growing in nearby structures and then extend to the orbit. This could be from areas around the orbit like the sinuses, eyelids, eyes themselves, tear sac, brain, bones of the face, back of the throat, roof of the mouth, or salivary glands. Sometimes, the orbit can also be a place where cancer from a different part of the body spreads, although this is rare.

What Causes Malignant Orbital Tumors?

Every part of the eye’s orbit can potentially develop a cancerous tumor. These malignancies can be divided into different types based on where they come from and their cell composition.

Primary tumors:
1. Lymphoproliferative: The most common type of adult malignant eye tumors is ocular adnexal lymphomas. These tumors usually occur in older women and most commonly start in the conjunctiva, eyelids, tear glands, or the soft tissue of the eye’s orbit. This type of cancer usually consists of a type of low-grade B-cell lymphoma known as MALT lymphomas.

2. Lacrimal gland: The tumors from lacrimal (tear) gland can be classified into two major groups: epithelial (skin-like cells) and non-epithelial, with non-epithelial tumors making up 50% to 60% of these masses. Notable among non-epithelial tumors are lymphomas. Among epithelial tear gland tumors, there are many types including adenocarcinomas, cystic carcinomas, different types of adenocarcinomas, and melanomas. Tumors from connective tissues (mesenchymal tumors) are rare and form only 10% to 15% of all tear gland tumors.

3. Mesenchymal: These tumors are highly aggressive and can occur in children and adults. They include many types such as rhabdomyosarcomas (which is the most common type in children), liposarcomas, leiomyosarcomas, osteosarcomas, and chondrosarcomas.

4. Vasculogenic: These are very rare cancers that originate from the cells lining or surrounding the blood vessels, and include types such as hemangiopericytomas, angiosarcomas, and epitheloid hemangioendotheliomas.

5. Intraocular: These originate from the cells lining the eyeball and include retinoblastomas (common in children) and orbital melanomas. Primary orbital melanomas form the most common type of intraocular malignancy in adults.

6. Germ cell: These extremely rare variants derive from embryonic cells and include types such as teratomas, yolk sac tumors, and granulocytic sarcomas.

Secondary tumors: These originate from nearby structures like the nasal cavity (sinonasal malignancies), central nervous system, the globe (eyeball), conjunctiva (the thin clear tissue over the eyeball), tear ducts, and the skin.

Metastatic tumors: These are cancers that have spread to the eye’s orbit from other parts of the body, including breast, prostate, skin melanomas, and lungs. These form about 1% to 3% of all malignant orbital tumors.

Risk Factors and Frequency for Malignant Orbital Tumors

The majority of tumors found in the orbit, the cavity or socket of the skull in which the eye and its appendages are situated, can be categorized into primary, secondary, or metastatic malignancies. Primary orbital malignancies make up around 82% to 87% of all orbital masses, secondary orbital malignancies represent 9% to 11%, and orbital metastasis accounts for 4% to 8% of all orbital masses. Among these tumors, 68% are benign, meaning they are not harmful in effect, and 32% are malignant, signifying they are serious and may be life-threatening.

In adults over the age of 60, the most common primary orbital tumor comes from lymphoproliferative lesions, which are abnormal growths of lymphatic cells. In this case, orbital adnexal lymphomas, a type of cancer, are the most prevalent, making up 67% to 90% of all orbital lymphoproliferative tumors and 24% to 30% of all space-occupying orbital tumors.

• Metastatic cancers, which are cancers that have spread from one part of the body to another, make up only 1% to 3% of orbital tumors.
• The most common type of metastatic cancer found in the orbit comes from breast cancer, representing 48% to 53% of all orbital cases.
• This is then followed by metastatic prostate carcinoma, melanoma, and lung cancer.

Contrastingly, in children, the most frequent metastatic orbital lesions occur from neuroblastomas, Ewing sarcoma, Wilms tumor, and leukemias. The most common primary malignancy in children, a cancer that originates in the orbit, is a cancer called rhabdomyosarcoma.

Signs and Symptoms of Malignant Orbital Tumors

When it comes to conditions of the eye and its surrounding areas, a complete history and physical examination are always required. Typically, masses in this region grow slowly, potentially causing symptoms such as dull eye pain, headache, or vision issues like decreased sight in one eye or double vision. These symptoms can occur before the appearance of more obvious signs such as bulging eyes.

To facilitate a thorough assessment, a checklist named ‘the 6 Ps’ has been developed. It stands for:

• Pain
• Progression
• Proptosis (bulging of the eye)
• Pulsation
• Palpation (physician examining by touch)
• Periocular changes (changes around the eye)

This checklist is used to evaluate the condition of the eye and its surroundings. Issues related to family history are usually not important as most eye tumors are not caused by inheritable genetic problems. By contrast, certain jobs or environmental exposures can increase the risk of developing specific tumors, so this information is crucial.

The examination should focus on detecting any ocular, orbital, or neurological symptoms, as well as examining the sinuses and neck if cancer is suspected. Tumors formed in different parts of the eye and its environs can cause various symptoms. Furthermore, specific symptoms can indicate the presence of an eye tumor resulting from a cancer in the sinuses.

A complete ophthalmic examination includes a range of assessments, including visual acuity, pupillary response, eye muscle function, exophthalmometry (measuring eye bulging), tonometry (detecting glaucoma), and fundoscopy (inspecting the retina). Other aspects to evaluate are skin, conjunctival, and corneal sensation, lid lag, abnormal conjunctival vessels, or choroidal-retinal folds. The position of the eyelid is measured using the margin-to-reflex distance.

A complete cranial nerve examination is required too, especially examining specific facial nerves and detecting any pain with ocular movements. Unilateral proptosis (one eye bulging more than the other) is often indicative of orbital pathology. A significant asymmetry between the two eyes is important. Upon palpation, if the eye globe is firm and with limited mobility, this could indicate an orbital malignancy.

If bulging eyes or suspicion of an internal eye mass is present, an intranasal examination is necessary. A thorough neck examination, including inspection of the parotid glands and cervical lymph nodes, should also be done.

Testing for Malignant Orbital Tumors

If your doctor thinks you might have cancer in the orbit, which is the area around your eye, they’ll probably need to do imaging tests. These tests are typically done using computed tomography (CT) scans or magnetic resonance imaging (MRI).

A CT scan is like a super-powered X-ray that can give detailed images of the inside of your body. If your doctor orders a CT scan of your orbit, plus your head and the areas around your nose (the paranasal sinuses), they’re doing this to see how far the suspected tumor has spread. This includes whether it has affected nearby bones. Changes in the bones or destruction of the bones can hint at how aggressive the tumor is.

If your doctor decides to do an MRI, they’ll probably want you to have one with intravenous (IV) contrast. This involves injecting a dye into your vein to make certain tissues show up more clearly in the images. MRI is especially good for showing soft tissues, so it can detail the extent of a tumor and whether it has spread to the tissues covering your brain (the dura) or the brain itself. It can also show if the tumor has spread to your nose or sinuses, as well as differentiate tumor tissues from blocked nasal secretions. Certain features on an MRI, like widespread lesions with irregular shapes and involvement of tissues around the orbit, can indicate a malignant (or cancerous) tumor rather than a benign (or non-cancerous) one. MRI also helps to distinguish between lymphomas in the orbit and inflammation or other low-grade malignancies. Ultrasonography, which uses sound waves to create images, is usually used for orbital masses that are pulsatile (or throbbing) or fluid-filled more than for malignant orbital masses.

In addition to imaging, you’ll likely need a biopsy to get a tissue diagnosis. For a biopsy, your doctor will take a small bit of tissue from the suspected tumor so it can be looked at under a microscope. While taking the biopsy, your doctor will take care to avoid the back of your eye socket (the orbital apex), your optic nerve (which connects your eye to your brain), and the muscles around your eye. The specific approach your doctor uses for the biopsy will depend on where exactly the tumor is located and whether it has spread to other areas.

For tumors that are located only in the orbit or are just starting to spread to nearby areas, your doctor might be able to do a biopsy through an incision (cut) in your upper or lower eyelid or through the side of your eye socket. If the tumor has spread to your nose or sinuses, they might do an endoscopic biopsy, which involves putting a thin tube with a light and camera at the end into your nose. A transcranial route for biopsy (or going in through the skull) is generally avoided because it carries a high risk of complications. If there are also swollen lymph nodes in your neck, your doctor might prefer to do a fine needle aspiration of these. This involves using a thin needle to extract cells from these lymph nodes in order to make a diagnosis.

Treatment Options for Malignant Orbital Tumors

The best approach to treat cancers of the eye socket (orbital malignancies) depends on the size and location of the tumor, the type of cancer cells found, and how advanced the disease is.

Medical Treatment

In many cases of orbital cancers, such as orbital adnexal lymphomas (cancers of the structures around the eye socket), treatment often involves a combination of radiation therapy, chemotherapy, and/or immunotherapy. For tumors that are localized and in their early stages, doctors usually recommend radiation therapy as the primary treatment. If the disease is advanced or if the tumor cells are particularly aggressive, chemotherapy is typically given first, followed by radiation. The standard chemotherapy regimen usually includes a combination of drugs: cyclophosphamide, adriamycin, vincristine, and prednisone (collectively known as CHOP). In case of a specific type of lymphoma known as non-Hodgkin’s B-cell lymphoma, which includes diffuse large B-cell lymphoma, a medication named Rituximab is used. It works by targeting a specific receptor on B-cells. For these types of cancers, surgery usually isn’t the main treatment option.

Surgical Treatment

In contrast, for some other types of orbital tumors, surgery is the primary treatment, especially if the tumor can be completely removed. The extent of the surgery would depend on the stage of the cancer, type of cancer cells found, and its original location. There are different types of surgeries including local resection (removal of the tumor and some of the surrounding tissue), exenteration (removal of the entire eye and some of the surrounding structures), or radical exenteration (more extensive surgery removing additional surrounding structures).

For advanced-stage epithelial tumors (cancers that begin in the cells that line the eye socket) and certain other high-grade tumors, additional surgical procedures like elective neck dissection (surgery to remove lymph nodes in the neck) and ipsilateral parotidectomy (removal of the parotid gland on the same side) may be discussed.

After surgery, radiation therapy is often recommended in most cases to manage any remaining disease locally. For tumors that have not been completely removed, a type of radiation therapy called brachytherapy may be considered. In brachytherapy, radioactive material is placed inside the body near the cancer to kill any remaining cancer cells.

What else can Malignant Orbital Tumors be?

Patients experiencing symptoms like blurred or double vision, inability to move the eyes, swelling around the eyes, and bulging eyes may be concerned about orbital malignancy, a type of eye cancer. However, a number of other conditions can produce similar symptoms, so doctors will explore several possibilities, including:

• A nonspecific orbital inflammation, also known as orbital pseudotumor. This is a benign (non-cancerous) condition that can cause swelling around the eyes, making it seem like there might be a tumor present.
• IgG-4-related orbital disease affects the entire body, but often presents symptoms similar to an orbital tumor, like eyelid swelling and bulging eyes.
• ANCA-related vasculitis can mimic eye cancer due to its effect on the tissues around the eyes. The symptoms can differ depending on how much of the tissue is affected.
• Tolosa-Hunt syndrome causes inflammation in the eye area, resulting in severe discomfort and a difficulty moving the eyes. The absence of bulging eyes or a noticeable tumor can distinguish it from orbital malignancy.
• Langerhans cell histiocytosis can impact the eye, presenting symptoms similar to an orbital malignancy.
• Benign orbital tumors, while not cancerous, can still produce similar symptoms.

Seeing a doctor if you experience these symptoms is crucial. They can perform tests and make a diagnosis based on your symptoms and medical history.

Surgical Treatment of Malignant Orbital Tumors

Surgery is the main form of treatment for most orbital (eye area) tumors that can be removed. However, removing these tumors often requires removal of the eye itself. If the cancer spreads into areas that surgical tools can’t reach (like inside the brain or the back of the eye socket), radiation and chemotherapy can be used first to try to reduce the size of the tumor. After its size is reduced, the tumor may then be removed surgically.

Lateral orbitotomy, which is a surgery that involves making a cut on the outer side of the eye, is often a safe way to remove tumors on that side. Exactly how much surgery is needed will depend on the tumor’s size, nature and location. Medial and inferior orbitotomy (surgical incisions made on the inner and lower sides of the eye respectively) are done depending on where the tumor is. If the tumor is towards the inside, a scope put in through the nose (trans-nasal endoscopic route) can be used along with surgical cutting. The surgeon can also make incisions on the natural lines of the face to access tumors that have spread to the sinuses.

If the tumor is large or if the eye contents have to be removed, there can be quite a large hollow space left behind, which could expose vital tissues like the brain. Doctors use a variety of methods to fill in these spaces and rebuild the walls of the eye socket. This can involve using body tissue, animal tissue, or man-made materials. In some cases, a muscle from the temple area is used to reduce the size of the hollow space, making it easier to insert a fake eye. The main aim of any rebuilding surgery after tumor removal is making sure all of the tumor is out and that important structures are covered. After this, the doctor works on creating a space to put the fake eye, or just fills in the surgery area with tissue. Skin grafting (transplanting skin), using body tissue from different areas and transplanting tissue from other areas of the body are common ways to prepare the eye socket for a fake eye.

What to expect with Malignant Orbital Tumors

The potential outcomes for malignant tumors in the eye socket largely depend on their type and stage. In the case of specific kinds of tumors known as orbital adnexal lymphomas (OALs), researchers have created a scoring system to predict the outcomes of the disease. This system is based on the number of tumors and whether or not the cancer has spread.

Patients are divided into three groups: Group 1 includes patients with a single OAL tumor and no spread of cancer; Group 2 contains patients with either multiple tumors or signs that the cancer has spread; and Group 3 refers to patients with multiple OAL tumors and evidence that the cancer has spread. In a period of ten years, it was observed that 75%, 50%, and 0% of these groups, respectively, didn’t see their cancer spread to various parts of the body.

It’s been found that over 90% of patients who have a particular type of eye socket tumor called orbital rhabdomyosarcoma and receive a combination of surgical and medical treatment survive at least 5 years after their diagnosis. Unfortunately, the same can’t be said for malignant orbital melanomas, a type of cancer that affects the eye, where the long-term outlook is generally poor.

Possible Complications When Diagnosed with Malignant Orbital Tumors

Understanding the possible issues that can occur due to malignant tumours around the eye socket (orbits) is crucial for doctors. These conditions greatly impact patient well-being and their treatment plans. Problems may stem from the tumour itself, the surgeries used to remove it, or the radiotherapy given afterwards.

Tumour-related complications: Complete muscle paralysis around the eye, loss of vision, additional infections leading to cellulitis around the eye, spreading into nearby areas like the nose and paranasal sinuses. This could cause a blocked nose on one side or recurrent nosebleeds, or even spread to the brain cavity, causing symptoms akin to meningitis.

Surgery-related complications: Patients who are suited for surgery often go through wide local excision or removal of the entire orbital content (orbital exenteration). This may result in a non-aesthetic orbital cavity that needs reconstruction, which can further lead to complications associated with the flap. Complications during the surgery may be injury to the base of the skull or the outer layer of the brain causing a cerebrospinal fluid leak. Additionally, expected risks include excessive bleeding, hematoma, vision loss, injury to the tear-duct, cosmetic deformities, sinking of the eye into the socket, and drying of the cornea if globe preservation surgeries are done.

Radiotherapy-related complications: are delay to the sensitive lens, tear gland, and retina which are located near or within the target volume.

Complications include:

• Tumor-related complications: complete eye muscle paralysis, vision loss, infection causing cellulitis around the eye, spreading into nearby nasal and sinus regions, spreading into brain cavity causing meningitis-like symptoms.
• Surgery-related complications: non-aesthetic orbital cavity, complications with the reconstructive flap, brain injury causing a cerebrospinal fluid leak, bleeding, hematoma, vision loss, tear-duct injury, cosmetic deformities, a sunken eye, and drying of the cornea due to globe preservation surgeries.
• Radiotherapy-related complications: damage to the lens, tear gland, and retina.

Recovery from Malignant Orbital Tumors

The recovery process after surgery for malignant orbital tumors, which are harmful growths around the eye, usually involves multiple types of treatments. The aim is to restore normal functioning, manage any complications, and make sure the patient’s quality of life is as high as possible. The exact recovery plan can vary depending on things like the type and size of the tumor, the type of surgery done, and the patient’s individual needs. However, there are some common aspects to the recovery process after surgery for malignant orbital tumors.

Wound care is critical because it helps prevent infection and encourages healing after surgery. This usually involves regularly cleaning the wound, applying medicine directly to the wound, and watching for any signs that the wound might be infected.

Potentially, a patient might choose to receive a prosthetic eye to improve their appearance and self-image if they have had to have an eye removed or had surgery that affects the look of the eye. The surgeon might have to do some additional work to make sure the prosthetic will fit well. Alternatively, the patient might choose to wear eye patches or glasses with opaque lenses.

Pain and discomfort after surgery can be managed with medicine prescribed by the health care team. Another option to help manage pain could be relaxation exercises and ice packs.

Patient who have had surgery to cut out a large tumor or remove the eye entirely might need further surgery to adjust the look of the eye area, along with visual aids. If the tumor resulted in significant disfigurement or loss of an eye, a custom-made prosthetic eye can be considered to restore appearance and self-esteem.

Patient who have had surgery for a tumor that started in the throat or nasal passages and spread to the eye might need therapy to help with speech and swallowing. This is because removing tumors in this area can affect the sound of the voice, the movement of the roof of the mouth, and the senses of smell and taste. A team of speech and language therapy professionals would typically be involved in this part of the recovery process.

As with any serious illness, dealing with a malignant orbital tumor and the treatments for it can be emotionally challenging. Support from professionals like counselors and psychologists, or from support groups, can help patients and their families deal with feelings of anxiety and depression, and other emotional challenges, that might arise because of the illness and treatments.

Finally, regular follow-up appointments with the healthcare team are essential to keep an eye on recovery, spot any complications early, and make any needed changes to the recovery plan.

Preventing Malignant Orbital Tumors

The best way to prevent and deal with harmful eye tumors is by ensuring early detection, treating any conditions that might lead to these tumors, and minimizing exposure to factors that increase the risk of acquiring these tumors. Regular eye check-ups are particularly crucial for individuals who have a family history of eye tumors or certain genetic conditions that might predisposing them to these tumors. These can help to catch the tumor early.

If you start to experience symptoms like changes in your vision (like differences in clarity or seeing double), pain in the eye that doesn’t go away, or noticeable differences in the appearance of the eye, it’s important not to ignore them and visit your main healthcare provider as soon as possible. These signs might suggest you have a potentially harmful eye tumor. Healthcare professionals might consider these as a red flag and want to investigate further, possibly using imaging techniques. Some particular symptoms are protruding eyes, inability to move the eyes, headaches that only affect one side, and blockage or repeated nosebleeds – these should never be ignored, as they might prevent severe complications from developing.

Making healthy lifestyle choices can also play a role in reducing the risk of certain forms of eye tumors. Actions such as avoiding tobacco use and excessive exposure to the sun may help. Healthcare professionals often stress the importance of using protective eyewear when working in high-risk professions or doing activities that have a risk of damaging the eyes, as this could prevent injuries that might lead to the development of eye tumors. It’s also helpful to raise awareness about the signs and symptoms of eye tumors through education campaigns. These could target both healthcare professionals and everyone else, motivating them to quickly seek medical evaluation and treatment. By maintaining a proactive approach to your health and taking preventive measures, you can help to minimize the number and severity of harmful eye tumors.