Uveal melanoma, also referred to as choroidal melanoma when arising from the choroid, most commonly occurs in middle-aged and older adults, with peak incidence between approximately 50 and 70 years of age. Although rare overall, it is the most common primary malignant intraocular tumor in adults. Uveal melanoma develops from pigment-producing uveal melanocytes, which normally synthesize melanin.
The choroid is the highly vascular layer situated between the retina and the white outer coat of the eye, the sclera. Its dense vascular network supplies the ocular tissues with oxygen and nutrients.
Genetic alterations can cause uveal melanocytes to proliferate in an uncontrolled manner and form a tumor. The precise causes of these alterations are not always known; spontaneous mutations and changes in specific genes may be involved. Loss of one copy of chromosome 3 (monosomy 3) is particularly characteristic and is associated with a more aggressive clinical course. Other recurrent abnormalities include gain of the long arm of chromosome 8 (8q gain) and alterations involving chromosome 6, including 6p gain. Light skin and light eye color are recognized risk factors. The annual incidence is approximately 7 cases per million population, and the risk increases with age.
Choroidal melanoma frequently develops in the posterior choroid, particularly near the macula - the region responsible for high-acuity central vision - or in the vicinity of the optic disc. These areas contain numerous melanocytes from which the tumor may arise. Less commonly, uveal melanoma originates in the ciliary body or iris. Although there is no single characteristic site within the choroid, the posterior segment is affected most often.
Early-stage disease is often entirely asymptomatic and may therefore be detected incidentally during a routine ophthalmologic examination. When symptoms occur, they may include blurred vision, visual-field defects, photopsia (flashes of light), shadows or dark areas in the visual field, metamorphopsia (visual distortion), or sudden deterioration in visual acuity. Pain is uncommon and generally occurs only in more advanced disease, for example when intraocular pressure is elevated.
In many patients, the diagnosis can be established on the basis of the characteristic funduscopic appearance in combination with ocular ultrasonography.
Assessment of visual acuity and visual-field function.
Evaluation of the anterior segment of the eye.
Following pharmacologic pupillary dilation, the retina and choroid are examined directly. This is often the most important initial diagnostic examination.
Determination of tumor size, thickness, and location.
High-resolution assessment of retinal changes and detection of subretinal or intraretinal fluid overlying the tumor.
Used selectively when there is a specific clinical question regarding extraocular extension.
Particular attention is given to the liver, typically using ultrasonography, MRI, or CT, together with appropriate laboratory investigations.
Primarily appropriate for very small or indeterminate pigmented choroidal lesions in which a definitive distinction between an early melanoma and a benign choroidal nevus cannot yet be made. Close follow-up typically includes fundus examination, serial photography, ultrasonography, and often OCT. Particular attention is paid to documented growth, increasing thickness, development of subretinal fluid, or new symptoms. Active treatment is generally recommended once concerning risk features emerge.
A well-established standard treatment for many small to medium-sized tumors. A radioactive plaque is temporarily sutured to the outer surface of the eye overlying the tumor. Local tumor-control rates are high, and the eye can often be preserved.
A highly precise form of external-beam radiotherapy that may be suitable depending on tumor size and location. Its physical dose characteristics allow substantial sparing of surrounding tissues.
Surgical resection of the tumor may be considered in selected patients at highly specialized centers.
Removal of the eye may be necessary for very large tumors, severe tumor-related pain, or situations in which useful vision cannot be preserved. An ocular prosthesis can subsequently be fitted.
A non-invasive, high-precision treatment using robot-assisted radiosurgical technology such as CyberKnife or ZAP-X. In appropriately selected patients, radiosurgery offers an eye-preserving alternative, particularly when other radiation techniques are less favorable. A major advantage is the delivery of a highly focused radiation dose, generally in a single treatment session, without surgical tumor removal.
CyberKnife or ZAP-X radiosurgery is performed on an outpatient basis and does not require conventional open surgery. The procedure is painless and delivers radiation with submillimeter precision, enabling optimal protection of adjacent healthy tissue. General anesthesia is not required. In more than 90% of cases, a single radiosurgical session is sufficient. Treatment typically lasts approximately 10-20 minutes.
ERCM has more than 20 years of experience in radiosurgical treatment and has treated approximately 1,250 patients with choroidal melanoma. Published clinical data support robotic radiosurgery as an effective eye-preserving treatment option in appropriately selected patients. Compared with enucleation, preservation of the eye may provide important benefits for physical and emotional quality of life.
Regular post-treatment surveillance is essential to assess therapeutic response and identify complications or recurrence at an early stage. Initially, follow-up examinations are generally performed at intervals of approximately three months.
ERCM performs CyberKnife treatment for choroidal melanoma in cooperation with the Department of Ophthalmology at LMU University Hospital Munich. More than 1,250 patients have been treated using this approach.
Choroidal melanomas are malignant tumors. Prognosis depends on several factors, particularly tumor size, location, extent, and underlying biological risk features. Early diagnosis and prompt, effective treatment by an experienced multidisciplinary specialist team are important determinants of outcome.
The need for immediate treatment depends on the individual clinical situation. Very small, slowly changing lesions that cannot yet be definitively classified as melanoma may be monitored closely. In contrast, established choroidal melanomas, particularly larger or demonstrably growing tumors, generally require timely active treatment.
The risk profile depends primarily on tumor size and location and on the proximity of radiosensitive ocular structures. Radiosurgery is generally well tolerated. Potential adverse effects may include temporary or persistent visual impairment and radiation-related effects in adjacent ocular tissues. Individual risks should be discussed in detail before treatment.
Yes. Local recurrence can occur after apparently successful treatment, although it is uncommon. Depending on the individual situation, repeat radiosurgical treatment may be feasible. Management is determined on a case-by-case basis.
Possible symptoms include blurred or distorted vision, reduced visual acuity in one eye, shadows or dark spots within the visual field, flashes of light, and floaters. These symptoms are nonspecific and should be assessed promptly by an ophthalmologist.
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