ENUCLEATION TO TREAT UVEAL MELANOMA STILL A REALITY

ENUCLEATION TO TREAT UVEAL MELANOMA: STILL A REALITY IN DEVELOPING COUNTRIES? Clemente, R. S. M. ; Lacerda, M. C. ; Cabral, R. G. C. ; Negreiros, B. ; Miranda, J. F. O. ; Moreira-Neto, C. A. ; Moreira Junior, C. A. ; Veroneis, L. S. HOSPITAL DE OLHOS DO PARANÁ BACKGROUND Uveal melanomas (UM) are the most common form of primary intraocular malignancy and ordinarily present as a pigmented mass. UM have no gender predilection but are 10 times more common in caucasian than on noncaucasian. Ciliary body melanomas are extremely rare as the incidence of UM in general is only 6 per milion per year, and they represents only 6% of this group. Usually asymptomatic, their position makes them extremely difficult to notice through an undilated pupil so they can be easily ignored in a routine ophthalmic examination. The diagnosis is based primarily on clinical examination and nonivasive ancillary exams, which helps enforcing clinical diagnosis and allows discrimination from other differencial diagnosis. Ocular treatment is aimed at conserving the eye and useful vision, preventing metastatic disease if possible. Therapeutic modalities include various forms of radiotherapy, laser therapy, tumor resection and enucleation. Today, most patients are treated with radiotherapy. CASE REPORT 71 year-old female patient, referred to our hospital due to the presence of a mass behind the iris observed after cataract surgery. Upon examination, the patient presented pseudophakic in the right eye, phakic in the left eye with visual acuity of 20/50 and 20/25. A dark mass (Fig. 1 and 2) could be seen in biomicroscopic examination which showed the presence of a sentinel episcleral vessel and a brown lesion in superior temporal cameral angle (Fig. 4). Retinal exam without changes in both eyes (Fig. 5). Ultrasound showing anterior mass of high external reflectivity and medium internal reflectivity with kappa angle measuring approximately 9. 57 x 13. 67 mm in length and width respectively and 9. 20 mm in height. Tomography of abdomen and chest without alterations. Due to the absence of radiotherapy center in patient’s hometown, the large lesion size and invasion of structures in the anterior chamber, enucleation was the treatment of choice. Biopsy report resulted in uveal mixed melanoma (Epithelioid and Fusiform). Patient still under regular follow-up. IMAGES Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6: Ultrasonography of uveal melanoma / Kappa Angle CONCLUSION Although radiotherapy is now the first choice in cases of uveal melanomas, enucleation still is required in about 20‒ 40% of patients. Determining factors for going through this surgery include large tumor size and extensive involvement of the ciliary body. The Collaborative Ocular Melanoma Study (COMS) reported no significant survival difference between local radiotherapy and enucleation in medium sized tumors. Although this conclusion is probably correct, the study lacked statistical power because many patients developed metastases so soon after treatment that systemic disease must already have been present at the time of ocular treatment. Despite successful eradication of the ocular tumor, up to 50% of patients develop metastatic disease, which typically involves the liver and is usually fatal within a year of onset of symptoms, as no effective therapies targeting metastases yet exists long-term postoperative surveillance is necessary to identify and treat local tumor recurrence and any other complications. Factors predicting metastasis include older age, large tumor size, ciliary body involvement, extraocular spread, epithelioid cytomorphology, chromosome 3 loss, chromosome 8 q gain, class 2 gene expression profile, loss of BRCA 1 -associated protein-1 (BAP 1), and the presence of inflammation. Many of them were present in our case. We agree that preservation of an anatomical and functional globe must be the main objectives , and so, radiotherapy should always be tried when possible, however, due to the difficulty in assisting and monitoring patients who live in poorly developed distant cities and lack of radiotherapy centers, enucleation becomes the only therapeutic option and unfortunately a reality in some cases. BIBLIOGRAPHICAL REFERENCES 1. Damato D. Progress in the management of patients with uveal melanoma. The 2012 Ashton Lecture. Eye 2012; 26: 1157– 1172. 2. Carvajal RD, Schwartz GK, Tezel T, Marr B, Francis JH, Nathan PD. Metastatic disease from uveal melanoma: treatment options and future prospects. Br J Ophthalmol 2017; 101: 3. 38– 44 Singh AD, De Potter P, Fijal BA, Shields CL, Elston RC. Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis. Ophthalmology. 1998 Jan; 105(1): 195– 8. http: //dx. doi. org/10. 1016/S 0161 -6420(98)92205 -9 4. Shah CP, Weis E, Lajous M, Shields JA, Shields CL. Intermittent and chronic ultraviolet light exposure and uveal melanoma. A meta-analysis. Ophthalmology. 2005 Sep; 112(9): 1599– 607. http: //dx. doi. org/10. 1016/j. ophtha. 2005. 04. 020