Fineman_Retina (Color Atlas and Synopsis of Clinical Ophthal

CHOROIDAL MELANOMA 275

Ultrasonographic measurements of tumor thickness are valuable in determination of doses for radiotherapy, as well as for serial evaluation following treatment. ● Fluorescein angiography of small choroidal tumors shows early mottled hyperfluores cence of the tumor with progressive staining in the late phases. Large tumors display filling of large intrinsic vessels in the venous phase, with diffuse late staining. ● Tumor biopsy, typically via fine-needle aspiration, can confirm diagnosis and assess metastatic risk via cytogenetic analysis. Prognosis and Management ● Prognosis for survival depends on a va riety of factors, including age at diagnosis, intraocular location, presence and extent of metastasis, tumor size in thickness and basal diameter, and genetic analysis. The 5-year mortality for patients with tumors with largest basal tumor diameter (LTD) greater than 15 mm is nearly 50%, and for those with LTD less than 10 mm, under 20%. The most important prognostic factor for metastatic death is internal genetic mutations (either by chromosomal analysis of genes 3, 6, and 8 or by gene expression profiling [GEP]). Tumors expressing monosomy or isodisomy of chromosome 3, GEP class 2, or mutation of the BAP1 gene are associated with higher metastatic rate. More recently, mutation of the PRAME gene has demonstrated effect on overall survival. ● Metastasis typically occurs to the liver. Re cent advances in liver-directed chemotherapy, immunotherapy, and radiation have improved overall survival. Multiple systemic immuno therapies are currently being investigated. The recent approval and clinical adoption of tebentafusp-tebn has increased survival in patients with hepatic or other systemic metastases. Nonetheless, most patients with

metastatic disease, especially those older than 60 years of age or with comorbidities will die within 5 years of the diagnosis of metastasis. There is no difference in mortality in patients treated initially with enucleation or radio therapy delivered by plaque brachytherapy or proton beam irradiation. Ocular ● The primary goal of treatment of choroidal melanoma is prevention of metastasis. The choice of a specific modality depends on tu mor size, location, and the patient’s systemic and psychological status. ● Treatment options include transpupil lary thermotherapy, radiotherapy (plaque brachytherapy or proton beam irradiation), and local resection with iridocyclectomy for anterior lesions and endoresection for those in the midperipheral fundus. Observation may be appropriate for small tumors with little evidence of growth. Enucleation is typi cally reserved for large tumors that cannot be covered completely with a radiation plaque or for blind, painful eyes. ● Patients should be evaluated regularly with careful funduscopy, wide-field serial fundus photographs ( Fig. 6-19 ), OCT, fundus auto fluorescence, and ultrasonography. Systemic ● The vast majority (~98%) of patients have no discernible metastasis at the time of diag nosis of their choroidal melanoma. ● Baseline systemic evaluation should be per formed in every patient, under the direction of a medical oncologist. Assessment typically includes complete blood count, liver enzyme panel, chest roentgenogram, and imaging (i.e., MRI of the liver or liver ultrasound) of the abdomen. ● Referral to a medical oncologist familiar with management of uveal or cutaneous melanoma

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