Eye Cancer

21.03.2024

EYE CANCERS DIAGNOSIS AND TREATMENT

Eye cancer is a type of malignant tumor that begins in or in the tissues around the eye, causing uncontrolled cell growth. Eye cancer can include different subtypes, such as retinoblastoma and ocular melanoma. Retinoblastoma is a type of eye cancer that usually occurs in children and can cause vision loss if not diagnosed early. Ocular melanoma is more common in adults and can pose a serious threat. When eye cancer is diagnosed early and treated with appropriate treatment methods, patients' quality of life and the chance of preserving their eyes increases. Early signs of eye cancer often manifest with symptoms such as vision changes, eye pain or sensitivity to light. Therefore, it is important to consult an ophthalmologist in case of any eye disease or symptom and not to neglect regular eye examinations.

WHAT ARE THE CANCER RISK FACTORS?

Eye cancer risk factors may include:

Genetic predisposition: Family history may be a risk factor for some types of eye cancer. Genetic mutations may play a role, especially in childhood eye cancers such as retinoblastoma. The most important risk factor for retinoblastoma is mutations in the RB1 gene.

Age: Eye cancer, especially retinoblastoma, usually occurs in childhood. However, other types of eye cancer are more common in old age.

Skin Color: Light-skinned individuals may be more prone to types of eye cancer such as ocular melanoma.

Other Cancers: Individuals with certain types of cancer, especially cancers such as leukemia or neuroblastoma, may have an increased risk of eye cancer.

Exposure to UV Rays: Prolonged sun exposure or use of tanning beds may increase the risk of eye cancer.

Eye Injuries: It may especially increase the risk of retinoblastoma, and the risk of eye cancer may increase in children with eye injuries or trauma.

Other Factors: Eye cancer risk factors may also include certain genetic syndromes (for example, Gorlin syndrome), radiation exposure, chemical exposure, and other unknown factors.

Anyone can have these risk factors, and even if the risk of eye cancer is low, it's important to get regular eye exams and preventive measures.

Image 1: Eye cancers are rare and symptoms such as swelling around the eye, bleeding and change in eye color can be detected.

HOW DOES IT OCCUR?

Although the mechanisms of development of eye cancer are still not fully understood, some basic factors are believed to play a role in the development of this type of cancer. Childhood eye cancers, especially retinoblastoma, are associated with genetic mutations in the RB1 gene. These mutations lead to uncontrolled growth of normal retinoblast cells. Additionally, exposure to UV rays may increase the risk of types such as ocular melanoma, especially in fair-skinned individuals. Eye injuries or trauma can also increase the risk of eye cancer, particularly for retinoblastoma, which has been associated with previous eye injuries. However, more research is needed to understand exactly how eye cancers develop, and the mechanisms by which such cancers develop are still being studied in depth.

WHAT ARE THE SYMPTOMS?

Symptoms of eye cancer may vary depending on the type, location and size of the tumor. Symptoms of eye cancer may include:

Vision Changes: Eye cancer, especially tumors such as retinoblastoma, can cause changes in vision. These changes may include misalignment, double vision, blurred vision, or vision loss.

Intraocular Problems: When eye cancer starts inside the eye, it can cause symptoms such as pain, discomfort, pressure inside the eye, or redness.

Swelling or Deformity around the Eyes: Abnormal swelling around the eyes or changes in the shape of the eyelids may be noticed.

Eye Bleeding or Discharge: Eye cancer can cause bleeding or abnormal discharge from the eye.

Photosensitivity: Symptoms of eye cancer include sensitivity to light, especially in tumors such as retinoblastoma.

Lazy Eye (Amblyopia): When eye cancers such as retinoblastoma are more common in children, one may notice that one eye of the child is lazier than the other or that other symptoms are absent.

Eye Color Change: Types of eye cancer, such as ocular melanoma, can cause changes in the color of the eye.

These symptoms are signs that suggest that you may have eye cancer. If such symptoms are noticed, it is important to consult an ophthalmologist. Early diagnosis is critical for eye cancer treatment to be effective. However, it should not be forgotten that these symptoms are not limited to eye cancer and can also be a sign of other eye problems.

HOW IS IT DIAGNOSED?

Diagnosis of eye cancer is usually made by an ophthalmologist or an oncologist. The diagnostic process may include the following steps in the case of suspected eye cancer: First, patient complaints and eye examination are evaluated. Next, imaging tests (for example, ultrasound, MRI, or CT scans) and procedures such as ophthalmoscopy or biopsy may be performed to examine lesions inside the eye. While childhood eye cancers, such as retinoblastoma, are usually diagnosed during an eye exam, eye cancers that are more common in adults, such as ocular melanoma, may require further imaging and examination. Diagnosis is important to determine the type, size, stage and treatment options of the tumor. Therefore, it is important to consult a specialist whenever you experience any eye discomfort or symptoms.

WHAT ARE THE PATHOLOGICAL TYPES?

Eye cancer is a type of tumor that can have different pathological types, and pathological types are classified according to the cellular and histological characteristics of the tumor. Some pathological types of eye cancer may include:

Retinoblastoma: This type is a type of eye cancer that occurs mostly in childhood and causes uncontrolled growth of cells in the retina.

Ocular Melanoma: This type of cancer, which originates from cells called melanocytes, involves the uncontrolled growth of pigment cells in the eye. It is more common in adults.

Eye Sarcomas: Sarcomas, which originate in the tissues of the eye, are a rare type of eye cancer. These sarcomas may arise from the connective tissue or muscles in the eye.

Carcinomas: Carcinomas refer to types of cancer that arise from eye surface cells. Conjunctival carcinomas fall into this category.

Lymphoma: Eye lymphomas arise from cells called lymphocytes in or around the eye and represent a rare type of eye cancer.

Pathological types of eye cancer may differ depending on the type and location of the tumor, and the treatment approach and prognosis of each type may be different. When eye cancer is diagnosed, it is important to determine the pathological types and evaluate the characteristics of the tumor because this can affect the treatment plan and the patient's prognosis. In the treatment of eye cancer, certain targeted drugs or treatment methods can be used to control the growth and spread of tumor cells. To achieve these goals, some receptors or genetic mutations are taken into consideration. Childhood eye cancers, especially retinoblastoma, are associated with genetic mutations in the RB1 gene, and these mutations are taken into consideration in treatment. Types of eye cancer such as ocular melanoma are linked to mutations in the BRAF gene, and these mutations play an important role in targeted therapies. For immunotherapy treatments, the presence of protective mechanisms developed by tumor cells against immune cells such as PD-L1 is evaluated and these mechanisms are aimed to be blocked. Additionally, VEGF receptors are targeted in cases where eye cancers promote the growth of blood vessels. These receptors and genetic characteristics play an important role in determining the treatment plan depending on the type of tumor and the medical condition of the patient. Treatment of eye cancer requires a multidisciplinary approach and should be determined by taking into account the patient's specific medical condition.

Image 2: Different types of cancer can develop in the eye depending on the tissue of origin.

HOW IS TUMOR STAGING DONE?

Eye cancer staging is a system used to determine the size, location and spread of the tumor. Eye cancer tumor staging is usually based on the following factors:

Tumor Size (T): It describes how large the tumor is in or around the eye. This is usually classified as T0 (no tumor) to T4 (tumor is large and has spread to peripheral tissues).

Regional Lymph Nodes (N): Determines whether the tumor has spread to nearby lymph nodes. It is classified between N0 (no lymph node involvement) and N1 (spread to lymph nodes).

Distant Metastases (M): It determines whether the tumor has spread to other parts of the body. It is classified as M0 (no distant metastasis) and M1 (distant metastasis).

Eye cancer staging may vary depending on the type and location of the tumor. For example, childhood eye cancers such as retinoblastoma often undergo a different staging system. Staging is important in determining tumor treatment options and prognosis. This staging information is used to plan eye cancer treatment and follow up the patient. Staging is performed by an ophthalmologist or oncologist using appropriate imaging tests and examinations.

HOW IS TREATMENT DONE ACCORDING TO STAGES?

Eye cancer treatment may vary depending on the stage and type of tumor and the patient's overall health. A general summary of treatment approaches according to eye cancer stages:

Stage 0 (Carcinoma In Situ): At this stage, the tumor is only found on the surface of the eye or at its starting point. Treatment usually begins with complete removal of the tumor. For small tumors, local surgery (or laser treatment) may be performed.

Stage I and II (Local Tumors): In these stages, the tumor may have grown in or around the eye, but has not yet spread to lymph nodes or distant areas. Treatment may include surgical removal or radiation therapy, depending on the size and location of the tumor.

Stage III: The tumor has still not spread to distant organs. Treatment may focus on the lymph nodes as well as the eye tumor. In this case, surgical removal of the lymph nodes and/or radiation therapy may be considered.

Stage IV (Distant Metastases): The tumor has spread to other parts of the body (for example, the liver or lung). Treatment may include treatments that target distant metastases, such as systemic chemotherapy, targeted drugs, or immunotherapy. It may also be necessary to treat the tumor in the eye and the lymph nodes.

Eye cancer treatment requires a multidisciplinary approach and should be determined based on the patient's specific situation. The treatment plan is created jointly by an ophthalmologist, oncologist and other oncology specialties. Early diagnosis and appropriate treatment can significantly improve the prognosis and quality of life of eye cancer patients.

Image 3: Diagnosis and initial treatment of eye cancers are carried out by ophthalmologists. In advanced tumors, radiotherapy, chemotherapy and smart drug treatments may be required.

WHAT ARE THE SYSTEMIC DRUGS USED IN TREATMENT?

Some chemotherapies, hormonal drugs, targeted therapies and immunotherapies used to treat eye cancer include:

Chemotherapy drugs:

Carboplatin: It is a platinum-based chemotherapy drug used in the treatment of eye cancer.

Etoposide: This drug, which is a topoisomerase II inhibitor, can be used in the treatment of retinoblastoma.

Targeted Therapies:

BRAF Inhibitors: Mutations in the BRAF gene can be targeted, especially in ocular melanoma. Drugs such as dabrafenib and trametinib can be used.

VEGF Inhibitors: Drugs that target vascular endothelial growth factor (VEGF) can control tumor growth by inhibiting the growth of blood vessels.

Immunotherapies:

Checkpoint Inhibitors: Drugs that target receptors that regulate the immune system, such as programmed cell death ligand-1 (PD-L1) or cytotoxic T lymphocyte antigen-4 (CTLA-4), can help destroy tumor cells by increasing the immune system response. Drugs such as pembrolizumab and nivolumab can be used.

Eye cancer treatment is determined depending on the type and stage of the tumor and the patient's general health condition. The treatment plan should be created by a multidisciplinary oncology team and tailored to the patient's specific conditions. Therefore, the treatment approach may differ for each patient.

HOW SHOULD FOLLOW-UP BE CARRIED OUT AFTER RECOVERY?

Follow-up after eye cancer treatment is critical for monitoring the patient's health and assessing the risk of tumor recurrence. The follow-up plan may vary depending on the stage of the tumor, response to treatment, and the patient's medical condition. Generally, follow-up includes the following components: Monitoring the tumor site and its surroundings with regular physical examinations. Postoperative function and mobility are also evaluated. Imaging tests (for example, x-rays, MRIs, or CT scans) monitor whether the tumor has returned or if new lesions appear. Blood tests are used to monitor the patient's general health and evaluate response to treatment. If any suspicious signs or symptoms occur, a biopsy may be performed and tumor recurrence evaluated. The frequency and duration of post-treatment follow-up may vary depending on the patient's specific condition, but is generally performed more frequently during the first year after treatment and at more regular intervals thereafter. Follow-up allows early recurrence of the tumor or another problem to be quickly identified and helps preserve the patient's long-term health and quality of life. Therefore, it is important to keep regular follow-up appointments after treatment.