Üveal ve Oküler Malign Melanom
Özet
Oküler melanomlar, kutanöz tümörlerden sonra ikinci en sık görülen malign melanom türü olup yetişkinlerde birincil göz içi malignitelerin başında gelir. Bu tümörler büyük oranda üveal yapıdan (koroid, siliyer cisim, iris) köken alırken, daha az oranda konjonktivada ortaya çıkar. Üveal melanom genellikle 60 yaş civarında teşhis edilir ve açık ten ile göz rengi gibi genetik risk faktörleriyle ilişkilidir; ancak ultraviyole ışığın etkisi kanıtlanmamıştır. Klinik belirtiler tümörün boyutuna göre değişmekle birlikte görme kaybı ve fotopsi gibi semptomları içerir. Teşhiste oftalmoskopi ve B-Scan ultrasonografi gibi klinik yöntemler öne çıkarken, birincil tedavide plak radyoterapisi (brakiterapi) ve seçili vakalarda enükleasyon uygulanır. Üveal melanom, özellikle karaciğere yavaş ama erken metastaz yapma eğilimindedir. Metastatik hastalıkta HLA-A*02:01 pozitif hastalarda Tebentafusb ilk seçenektir. Diğer yandan, konjonktival melanomlar oküler melanomların %5'ini oluşturur ve moleküler açıdan üveal formdan ziyade kutanöz melanomlara benzer. Konjonktival melanomda BRAF ve KRAS mutasyonları sık görülürken, tedavisinde geniş lokal eksizyon, kriyoterapi ve immün kontrol noktası inhibitörleri (PD-1/CTLA4) tercih edilmektedir.
Ocular melanomas represent the second most common type of malignant melanoma after cutaneous tumors and constitute the most frequent primary intraocular malignancy in adults. While the vast majority originate from the uveal tract (choroid, ciliary body, iris), a smaller fraction arises from the conjunctiva. Uveal melanoma is typically diagnosed around the age of 60 and is strongly associated with risk factors such as fair skin and light eye color, whereas the role of ultraviolet light remains unproven. Clinical presentation depends on tumor size and location, often manifesting as vision loss or photopsia. Diagnosis primarily relies on ophthalmoscopy and B-Scan ultrasonography. Plaque radiotherapy (brachytherapy) serves as the primary treatment modality, while enucleation is reserved for advanced cases. Despite localized treatments, uveal melanoma carries a high risk of systemic metastasis, predominantly targeting the liver. In metastatic settings, Tebentafusp is the preferred frontline systemic therapy for HLA-A*02:01 positive patients. Conversely, conjunctival melanomas account for only 5% of ocular melanomas and exhibit genomic features closer to cutaneous melanomas. They frequently harbor BRAF and KRAS mutations, and their management combines wide local excision with cryotherapy and immune checkpoint inhibitors.
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