Uveal melanoma: physiopathology and new in situ-specific therapies

• Published in: Cancer chemotherapy and pharmacology Vol. 84; no. 1; pp. 15 - 32
• Main Authors: Souto, Eliana Maria Barbosa, Zielinska, A., Luis, M., Carbone, C., Martins-Gomes, C., Souto, S. B., Silva, A. M.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Nature 01.07.2019; Springer Berlin Heidelberg; Springer Nature B.V
• Subjects: Adult; Brachytherapy; Cancer Research; Charged particles; Chemotherapy; Chromosome Aberrations; Clinical trials; Enucleation; GTP-Binding Protein alpha Subunits - genetics; GTP-Binding Protein alpha Subunits, Gq-G11 - genetics; Humans; Immunotherapy; Liver; Liver metastases; Liver Neoplasms - secondary; Liver Neoplasms - therapy; Medical research; Medicine; Medicine & Public Health; Melanocytes; Melanoma; Melanoma - genetics; Melanoma - pathology; Melanoma - therapy; Metastases; Metastasis; Mutation; Oncology; Pathogenesis; Perfusion; Pharmacology/Toxicology; Photocoagulation; Photodynamic therapy; Phototherapy; Radiation therapy; Review Article; Science & Technology; Surgery; Transpupillary thermal therapy; Tumor Suppressor Proteins - genetics; Ubiquitin Thiolesterase - genetics; Uveal melanoma; Uveal Neoplasms - genetics; Uveal Neoplasms - pathology; Uveal Neoplasms - therapy; Photocoagulation; Uveal melanoma; Liver metastases; Photodynamic therapy; Transpupillary thermal therapy; Liver-directed therapies
• ISSN: 0344-5704; 1432-0843
• DOI: 10.1007/s00280-019-03860-z

Uveal melanoma is the most common primary intraocular tumor in adults. It can arise from melanocytes in the anterior (iris) or posterior uveal tract (choroid and ciliary body). Uveal melanoma has a particular molecular pathogenesis, being characterized by specific chromosome alterations and gene mutations (e.g., GNAQ/GNA11; BAP1), which are considered promising targets for molecular therapy. Primary treatment of uveal melanoma includes radiotherapy (brachytherapy and charged-particle therapy), phototherapy (photocoagulation, transpupillary thermal therapy, and photodynamic therapy) and surgery (local resection, enucleation and exenteration). Approximately half of patients with uveal melanoma will, however, develop metastasis, especially in the liver. The treatment of metastatic uveal melanoma includes systemic chemotherapy, immunotherapy and molecular targeted therapy. Liver-directed therapies, such as resection, chemoembolization, immunoembolization, radioembolization, isolated hepatic perfusion and percutaneous hepatic perfusion, are also available to treat metastatic uveal melanoma. Several clinical trials are being developed to study new therapeutic options to treat uveal melanoma, mainly for those with identified liver metastases. The present work discusses the physiopathology and new in situ-specific therapies for the treatment of uveal melanoma. The authors wish to acknowledge the financial support received from Portuguese Science and Technology Founda-tion (FCT/MCT) and from European Funds (PRODER/COMPETE) under the projects M-ERA-NET-0004/2015-PAIRED and UID/AGR/04033/2019 (CITAB), co-financed by FEDER, under the Partnership Agreement PT2020. The authors wish to acknowledge the contribution of the Master Student Ms. Irina Pereira in the reading of the manuscript.