Erschienen in:
01.12.2013 | Invited Commentary
Latest Developments in the Biology and Management of Uveal Melanoma
verfasst von:
Sapna P. Patel
Erschienen in:
Current Oncology Reports
|
Ausgabe 6/2013
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Excerpt
Uveal melanoma is the predominant intraocular tumor in adults and it represents approximately 3-5 % of all melanoma diagnoses, making it the most prevalent location for melanoma outside the skin [
1]. Approximately six people per million are diagnosed each year in the U.S., with similar incidences among Caucasian populations in other countries. The risk factors for this disease remain contradictory, with some reports suggesting ultraviolet radiation as a risk [
2], whereas others refute that claim [
3]. Still others suggest nonmodifiable risk factors such as race, skin color, eye color, and hair color are important, similar to the risk factors for cutaneous melanoma [
4]. Melanocytes of the epidermis and dermis as well as those of the uveal tract are embryologically derived from neural crest cells; however, the similarity between cutaneous and uveal melanomas ends there. Whereas cutaneous melanomas spread via lymphatic drainage, the uveal tract is not drained by lymphatics and spread occurs via hematogenous propagation [
5,
6]. Nearly half of all uveal melanomas will develop distant metastasis, with a predilection for the liver in virtually all cases. Primary treatment of the eye most commonly involves brachytherapy, but proton beam radiation therapy is making its mark on the treatment landscape as well [
7‐
10]. Enucleation, or surgical removal of the globe, is also an option used for large tumors or those with extraocular extension. Local recurrences in the eye are rare, but the development of distant disease in half of patients articulates the presence of micrometastasis or subclinical tumor cells at the time of diagnosis [
11]. These patients will develop metastatic disease most commonly within 10 years of diagnosis. The type of primary treatment (brachytherapy, enucleation, proton beam irradiation) plays no role in the incidence of metastatic disease, suggesting that microscopic metastases are present from the outset. In a Finnish series of nearly 300 patients with uveal melanoma followed for long-term survival, the incidence of metastatic disease at 15 years after diagnosis was 45 %, at 25 years was 49 %, and at 35 years was 52 % [
11]. Because of this, most experts agree surveillance for metastatic disease should continue for 15 years. Of the 145 patients in this series who developed metastatic disease, 62 % of metastases occurred within 5 years. …
