Eye tumors

Overview

Cancer of the eye is a cancer that begins in the eye and spreads throughout the body. A group of cancer cells is referred to as a malignancy. They're abnormal cells that develop rapidly and uncontrollably. They have the ability to spread to other parts of the body or infiltrate and damage tissues. Although there are many distinct types of eye malignancies, primary intraocular melanoma is the most frequent.

Cancer can sometimes move from another region of the body to the eye. Secondary eye cancer is the term for this type of cancer. Breast cancer is the most common cause in women, whereas lung cancer is the most common cause in males.

High-frequency ultrasound, fluorescein angiography, anterior and posterior segment optical coherence tomography, computed tomography (CT), and magnetic resonance imaging are all used to diagnose eye cancer (MRI)

Once a diagnosis has been made, treatment options are determined by the tumor's location, size, local extension, growth trends, and subsequent consequences. Observation, local excision, chemotherapy (topical, intravenous, intra-arterial, or intravitreal), and radiation are all possibilities for treatment (ophthalmic plaque or external beam). If these eye- and vision-saving therapies are not practicable, enucleation or exenteration is used.

Types of eye tumors

Ocular malignancies are the only diseases of the eye that pose a danger to both vision and life. In most situations, a thorough clinical history and specialist eye examination may be used to make the diagnosis. The different types of primary eye cancers include:

Eye or intraocular melanoma: conjunctival, choroidal
Squamous cell carcinoma
Lymphoma or Primary intraocular lymphoma: often associated with lymphoma in the brain (called primary central nervous system (CNS) lymphomas, usually bilateral)
Retinoblastoma – more commonly seen in children, especially in the 0-4 years age group. The most common secondaries, or metastatic tumors, in the eye are from breast and lung cancers.

Eye tumors symptoms

Eye malignancies are a broad group of diseases that manifest in a variety of ways. The majority of the symptoms appear benign, and as a result, patients overlook them in the early stages. The signs and symptoms described here might be caused by a variety of different benign illnesses, and cancer is generally identified only after a thorough eye examination by your eye specialist.

The commonly reported symptoms of eye cancer include:

Flashes of light, or floaters
Blurred vision, haloes, and shadows around images, especially of bright light
A dark nevus or mole on the white part of the eye that increases in size get “angry” looking blood vessels around it, or bleeds
The decrease in vision may be associated with pain
Bulging of one eye, or both, called proptosis
A lump or tumor on your eyelid or in your eye that’s increasing in size, getting blood vessels
Change in color of the iris
White reflex in the pupil

What is the treatment for eye tumors?

Surgery, radiation, and chemotherapy are the three main treatment options for eye malignancies. A multidisciplinary team decides on the operation, which is tailored to the patient's specific needs. It is dependent on a number of things. These are some of them:

The type of eye cancer, it’s grade, which determines how aggressive it is, and its malignant potential
The rate of tumor growth, and its size and stage at presentation, the extent of spread locally and at distant sites
The site of the tumor, whether it is on the eyelid, conjunctiva or within the eye or spread more extensively
General health and coexisting diseases in the patient, age at presentation and patient choice

The ophthalmologists taking care of these eye cancers work on the following fundamental principles:

Saving the life of the patient is paramount
Saving the sight, as much as possible, is the most important
Preserving the functional and structural integrity of the eye
Preserving the cosmetic appearance of the eyeball and surrounding areas

Based on this, the various options your eye surgeon can choose from, are listed below.

Laser therapy: It directs a laser beam to destroy the tumor cells locally.
Plaque therapy, also known as brachytherapy, is a treatment method in which a plate impregnated with the therapeutic substance is used to target the treatment to a single site, limiting harm to adjacent tissue.
Radiotherapy: Delivers radiation that kills tumor cells while sparing the surrounding delicate tissues, and several modern radiation treatments, such as proton therapy, have been shown to have a better track record in dose delivery accuracy, resulting in less collateral damage to the surrounding tissues.
Limited resection: This refers to the excision of the cancerous portion of the eye. Iridectomy, choroidectomy, eyelid excision, and other procedures are examples.
Enucleation is the removal of the eyeball without the removal of the eyelids or eye muscles. For improved cosmetic outcomes, the eye surgeon might later fit a prosthesis, or artificial eye.
Evisceration is the partial removal of the contents of the eye while leaving the sclera, or white component of the eye, intact. Enucleation and evisceration both necessitate the use of a prosthetic eye, which can only be a cosmetic one with no vision.
Exenteration: This is a major procedure that entails the removal of the eye and all orbital contents. A specially developed prosthesis can then be placed to restore the face's look.
Chemotherapy is the use of specific medications to kill cancer cells, which can be given orally or by injection.

Ocular Melanoma definition

Melanoma is a cancerous overgrowth of melanocytes. After cutaneous melanoma, ocular melanoma is the most frequent kind of melanoma in adults, and it is the most common primary intraocular malignant tumor.

Light skin tone, atypical cutaneous nevi, light eye color, iris nevi, and freckles are all known risk factors for ocular melanoma. Ocular melanoma has also been linked to occupational sun exposure, tanning, and outdoor activities.

The reason is unknown, however it is more common in those with lighter complexion and blue eyes. In the Western world, uveal melanoma affects roughly 6 to 8 people per million each year. At the time of presentation, less than 2% of patients have signs of metastasis; yet, more than 40% of patients will die from extensive illness.

Uveal melanoma is the most frequent kind of ocular melanoma. Ocular melanoma has been shown to be 30 percent more common in women than in men. Non-Hispanic whites had a greater rate of association, with an incidence of 6.02 instances per million. African Americans and Asians had lower rates of ocular melanoma, with incidences of 0.31 per million and 0.39 per million, respectively.

One of the key processes by which ocular melanoma develops is oxidative damage to pigmented tissues, which is governed by the degree and type of iris pigmentation. A change in the underlying genomic sequence from aberrant adenine-to-cytosine mutations is another idea linked to the development of uveal melanoma. Adenine-to-thymine mutations, on the other hand, have been linked to ciliochoroidal melanoma. Melanomas can also develop from pre-existing lesions known as de-novo lesions.

Clinical presentation

The clinical appearance of ocular melanomas can range from mild to severe, depending on the location of the tumor. Blurred vision, visual field defects, flashing lights, redness, irritation, soreness, and a pressure-like sensation are all common symptoms. Because of the form of choroidal tumors, they can induce retinal detachment.

Conjunctival melanomas can appear as lumps or pigmented lumps, and they normally cause less discomfort and irritation than other types of melanomas. Ciliary body tumors can induce lens displacement and accommodation problems. Iris melanomas can develop from a prior lesion or a new area, causing pupil dilation, cataracts, and even glaucoma.

Evaluation

Fundoscopic examination, fundus photography, and ocular ultrasonography are all part of the fundamental assessment of an eye cancer. Various imaging techniques are utilized depending on the location of the cancer. If the orbit is implicated, the initial step is to do a CT or MRI scan. If lymphatic involvement is suspected, a PET scan, as well as a head and neck MRI and a fine-needle aspiration biopsy, are recommended.

More invasive approaches, such as fluorescein angiography (FA) and indocyanine green angiography (ICGA), have been utilized to help distinguish melanoma from underlying disease, check for subsequent neovascularization or ischemia, and further reveal lesions concealed by media opacity.

Optical coherence tomography and optical coherence tomography angiography have lately made it feasible to examine the superficial and deep structures of the retina and choroid non-invasively, providing greater detail than a basic clinical examination.

Management of ocular melanoma

Depending on the size of the melanoma, various treatment modalities are used. In uveal melanoma, options include the following:

Plaque radiation therapy
Particle beam radiotherapy
Transpupillary thermotherapy
Laser photocoagulation
Gamma knife stereotactic radiosurgery
Local surgical resection
Enucleation

Techniques like transretinal endoresection and transscleral resection have produced advances in vision and eye preservation. According to studies, chemotherapeutic drugs used in cutaneous melanoma can result in response rates ranging from 0% to 15%. Dacarbazine, temozolomide, cisplatin, treosulfan, and fotemustine have all been utilized as chemotherapy drugs.

Surgical resection is frequently used to treat iris melanomas. Plaque treatment or enucleation will be used to treat larger iris melanomas that cannot be surgically removed.

When choroidal tumors extend into the orbit, they are treated by enucleation or orbital exenteration. Local excision with adjuvant chemotherapy is an effective treatment plan for conjunctival melanoma (mitomycin)

Prognosis

Several clinical and histological factors related with metastatic mortality in primary uveal melanoma were discovered. Increased patient age, increased tumor size (tumor elevation and independently tumor diameter), ciliary body involvement, extraocular extension, epithelioid cell type, lymphocytic infiltration, presence of fibrovascular loops, and immunohistochemical methods to detect biomarkers such as human leukocyte antigen (HLA) molecules and others.

By 10 to 15 years following diagnosis, almost half of individuals with ocular melanoma will have developed metastases. Even 20-25 years after their original diagnosis, a tiny fraction of patients will develop metastases. Metastatic illness is deadly in all cases. Despite advancements in treating the original eye tumor, the 50% death rate remains unchanged. To enhance patient outcomes, further research is urgently required.

Differential Diagnosis

Clinically, various diseases may present similarly to ocular melanoma.

Choroidal body: Hemorrhagic disorders such as age-related macular degeneration (AMD) and hemorrhagic choroidal detachment; choroidal nevus, choroidal hemangioma, osteoma; choroidal nevus, choroidal hemangioma, osteoma; choroidal nevus, choroidal hemangioma, osteoma; choroidal nevus, choroidal hemangioma
Iris: Iris nevus, iris foreign body, peripheral anterior synechia, iris pigment epithelial cyst, iris stromal cyst, melanocytoma, iris atrophy, and Cogan-Reese syndrome
Retina: Congenital retinal pigment epithelium hypertrophy and retinal pigment epithelium adenocarcinoma; and inflammatory lesions like posterior scleritis
Various: hemangiomas, sarcoid nodule, metastatic tumor, benign nevus, congenital hypertrophy, intraocular foreign body granuloma, Fuchs adenoma

How is the staging of eye tumors done?

Eye exams, radiographic testing (ultrasound, CT, and MRI scan), and pathology tests following a biopsy are used to establish the clinical stage. If the doctor suspects metastatic cancer, he or she may recommend a PET Scan to screen for secondary tumors or original cancer.

To plan treatment, provide patients with a prognosis, and collaborate with colleagues in various disciplines such as oncology, radiation medicine, and oncosurgery for patient care, doctors usually use standardized international staging systems such as the American Joint Committee on Cancer (AJCC) TNM system and the system used by the Collaborative Ocular Melanoma Study (COMS) group..

Lymphoma of the eye

Lymphoma is a cancer that starts in immune system cells called lymphocytes. The spleen, thymus, bone marrow, and other areas of the body contain these cells.

Lymphoma of the eye is divided into 2 groups:

primary intraocular lymphoma
ocular adnexal lymphoma

Primary intraocular lymphoma

These form within the eyes. Primary intraocular lymphoma is a rare malignancy that accounts for fewer than 1% of all eye malignancies. If your immune system is impaired, you're more prone to develop intraocular lymphoma. People who, for example:

have AIDS
have had organ transplants and need to take drugs to damp down their immune system
are elderly

Retinoblastoma

The soft tissue sarcoma rhabdomyosarcoma (RMS) is a form of rhabdomyosarcoma. Muscles, tendons, and nerves are examples of soft tissue. The delicate tissue around the eye is where orbital rhabdomyosarcoma begins. It mostly affects young children, although it can also afflict newborns and the elderly.

Symptoms include:

a droopy eyelid
bulging of the eye
a swollen eye

Other Eye Cancers

Other types of cancers can affect the eye. These include:

Ocular metastases are cancers that have progressed from other organs to the eye, such as the breasts or lungs. They are most typically seen in the choroid, which contains many blood veins. Cancer cells migrate from a distant organ to the eye via the circulation. In adults, it is the most frequent cancer discovered in the eye.

Orbital malignancies are tumors of the tissues that surround the eyeball (called the orbit). Muscles that move the eyeball around and nerves linked to the eyeball are examples of these tissues.

Adnexal malignancies are tumors that affect the eyeball's auxiliary structures (called adnexal structures). The eyelids and tear glands are examples of this.

Conclusion

Secondary tumors in the eye are frequently caused by malignancies that have migrated from other regions of the body, including the breast, lung, bowel, or prostate. Retinoblastoma in children and melanoma in adults are two forms of primary cancers that develop within the eye.

When ocular melanoma metastasizes, it is a dangerous and fatal clinical cancer with a dismal prognosis. When patients arrive with benign lesions in their uveal tract from the iris to the choroid, special care must be taken. For adequate assessment and treatment, an interprofessional team might be beneficial. The lesion may be initially seen by optometrists or primary care doctors. A complete ophthalmologic examination is required, followed by ophthalmology staging depending on size.