uveal melanoma

What is Uveal Melanoma?

Uveal melanoma, also known as ocular melanoma, is a rare and serious form of cancer that affects the middle layer of the eye, called the uvea. This layer contains blood vessels, pigment cells (melanocytes), and provides nutrients and oxygen to the retina.

Types of Uveal Melanoma

Uveal melanoma can occur in different parts of the uvea, including:

• The iris: a small tumor that grows slowly and rarely spreads to other parts of the body
• The ciliary body: usually larger tumors and are more likely to spread to other parts of the body
• The choroid: the most common type of eye cancer in adults

Symptoms

Symptoms may include:

• Decreased or blurry vision
• Floaters, or spots that drift in your vision
• Flashes of light
• A dark spot on the iris
• Changes in the shape and size of the pupil

Causes and Risk Factors

While the exact cause of uveal melanoma is unknown, it's believed to be related to genetic mutations. People with a family history of melanoma or those who have had previous skin cancers are at higher risk.

Treatment and Prognosis

Diagnosing eye melanoma begins with a dilated eye exam by an ophthalmologist. Treatment options may include surgery, radiation therapy, or chemotherapy, depending on the size and location of the tumor. The prognosis varies depending on the stage and type of uveal melanoma, but early detection and treatment can improve outcomes.

References

• [1] Uveal melanoma most often affects the middle layer of your eye (uvea). Parts of your eye's uvea that can develop melanoma include the colored portion of your eye (iris), the muscle fibers around your eye's lens (ciliary body), and the choroid. [Source: 1]
• [3] Symptoms include blurred vision, loss of vision, and photopsia, but there may be no symptoms. [Source: 11]
• [4] Uveal melanoma is a type of eye cancer in the uvea of the eye. [Source: 11]

Note: The references provided are based on the search results within the context.

Common Signs and Symptoms of Uveal Melanoma

Uveal melanoma, a rare type of eye cancer, may not cause any symptoms in its early stages. However, as the tumor grows, it can lead to various signs and symptoms, including:

• Blurred or changed vision: This is one of the most common symptoms of uveal melanoma, where patients experience a change in their visual acuity or perception.
• Floaters or spots in the vision: Small specks, wavy lines, or "floaters" can appear in the vision, which can be a sign of a tumor growing on the retina or other parts of the eye.
• Flashes of light: Some patients may experience sudden flashes of light in their vision, which can be a symptom of uveal melanoma.
• A dark spot on the iris: A dark spot or patch can appear on

Treatment Options for Uveal Melanoma

Uveal melanoma, also known as ocular melanoma, is a rare and aggressive form of eye cancer that affects the middle layer of the eye, called the uvea. While it's relatively rare, when it spreads to other parts of the body, treatment options have historically been limited.

New Treatment Options

However, recent advancements in medical research have led to the development of new treatment options for uveal melanoma. One such option is tebentafusp, a first-in-class oral drug that has been approved by the FDA for the treatment of unresectable or metastatic uveal melanoma.

How Tebentafusp Works

Tebentafusp works by mobilizing and activating T cells to fight tumor cells. It's designed specifically for patients with human leukocyte antigen (HLA)-A*02:01-positive adults, which is a specific genetic marker that helps identify eligible patients.

Clinical Trials and Studies

Several clinical trials and studies have been conducted to evaluate the efficacy of tebentafusp in treating uveal melanoma. One notable study, known as the FOCUS study, demonstrated promising results in patients with unresectable hepatic metastases.

Other Treatment Options

While tebentafusp is a significant advancement in treatment options for uveal melanoma, other treatments are also being explored. For example, darovasertib, a first-in-class oral inhibitor of protein kinase C (PKC), has shown promise in treating this type of cancer.

Availability and Accessibility

Tebentafusp is now available on the NHS in England for hundreds of people with uveal melanoma, marking a significant milestone in the treatment of this rare form of eye cancer.

References:

• [5] Tebentafusp can be used to treat uveal melanoma that has spread or that can't be treated with surgery, in people whose cells have a certain HLA ...
• [10] Last January, the FDA approved tebentafusp (Kimmtrak, Im­munocore) for the treatment of unresectable or metastatic uveal melanoma in human leukocyte antigen (HLA)-A*02:01–positive adults.
• [12] 'Pioneering' drug for rare eye cancer to be rolled out on NHS in England.
• [13] Tebentafusp new treatment for melanoma patients.

Differential Diagnosis of Uveal Melanoma

Uveal melanoma, a type of cancer that affects the eye, can be challenging to diagnose due to its similarity in appearance to other conditions. The differential diagnosis of uveal melanoma includes various tumors and non-neoplastic entities that must be considered when making a diagnosis.

Common Differential Diagnoses:

• Staphyloma: A condition characterized by a thinning or weakening of the sclera, which can mimic the appearance of uveal melanoma.
• Medulloepithelioma: A rare tumor that arises from the ciliary body and can be mistaken for uveal melanoma.
• Leiomyoma: A benign tumor that can occur in the iris or ciliary body and may resemble uveal melanoma.
• Choroidal nevi: Benign growths on the choroid layer of the eye that can be confused with uveal melanoma.
• Retinoblastoma: A type of cancer that affects the retina, which can be mistaken for uveal melanoma in some cases.

Other Differential Diagnoses:

• Uveal metastases: Cancer cells from other parts of the body that have spread to the eye and can mimic the appearance of uveal melanoma.
• Choroidal detachment: A condition where the choroid layer separates from the sclera, which can be mistaken for uveal melanoma.
• Choroidal hemangioma: A benign tumor that arises from the choroid layer and may resemble uveal melanoma.

Importance of Accurate Diagnosis:

Accurate diagnosis of uveal melanoma is crucial to ensure proper treatment and management of the condition. The differential diagnoses listed above must be considered when making a diagnosis, as they can have significant implications for patient care.

References:

• [2] by PV Foti · 2021 · Cited by 51 — The diagnosis of uveal melanoma