Topography and clinical features of iris melanoma

Background To characterise the topographical and clinical features of primary iris melanoma and to visualise the patterns of tumour extent in the iris. Methods Clinical characteristics of iris melanomas were analysed, and data on their size, shape, and location were converted into a database of two-dimensional iris charts by means of computer-drawing software. The geometric centre of each tumour was entered into corresponding sectors of the chart. The extent of the melanomas was computationally visualised by merging the iris drawings and displaying the number of overlapping tumours on colour-coded iris maps. Results Twenty-nine patients (18 females and 11 males) with a mean age of 52 years met the inclusion criteria. The mean largest tumour diameter was 6.1 mm (range, 1.8–11.0 mm). Five tumours (17%) involved the pupillary margin, 10 (34%) involved the iris root, and 10 (34%) involved both sites. The hemispheric location of the tumour centroid was superior in 3 eyes (11%) and inferior in 25 (89%) (p < 0.0001), and the distribution between the temporal and nasal hemispheres was 17 (61%) and 11 (39%), respectively (p = 0.26). In females, the iris melanomas were located more temporally (p = 0.02) and had more often originated from a pre-existing naevus (p = 0.03), than in males. There was also shift towards more temporally located melanomas in younger patients. Conclusions The lower temporal iris quadrant is the preferential area of melanoma occurrence and growth. Iris melanoma tends to be more temporally located in females, who compared with males also have a higher proportion of melanomas arising from a pre-existing naevus.

Proton beam radiation for iris melanoma: case series and review of literature

ObjectivePurpose of this study is to analyse the visual outcomes, the complication and eye retention rate as well as tumour control data of patients treated with proton beam radiation therapy (PBRT) for iris melanoma.MethodsRetrospective case series and review based on patients’ records. All tumours were categorised according to the American Joint Committee of Cancer staging criteria for primary iris melanoma und underwent either sectorial or whole anterior segment PBRT.ResultsThirteen cases were identified of which five received PBRT of the whole anterior segment and eight received sectorial PBRT. Local tumour control after a mean follow-up of 25 months was 92%. Complications after PBRT included cataract (46%), secondary glaucoma (31%), superficial keratitis (15%) and madarosis (8%). Complications were more common in patients necessitating irradiation of the entire anterior segment than in patients which received sectorial irradiation. Eye retention was achieved in all cases. No statistically significant difference in the mean best corrected visual acuity (BCVA) and intraocular pressure (IOP) was found before and after treatment. Comparison of mean BCVA and IOP between different treatment groups (complete anterior segment vs sectorial irradiation) at the last follow-up visit were also not significantly different. No patient developed metastatic disease during follow-up.ConclusionPBRT is a safe and vision preserving therapeutic modality for iris melanoma. Complete irradiation of the anterior segment is associated with higher complication rates.

Iris Melanoma in a Patient with Retinitis Pigmentosa

This report describes the histopathological findings of an iris melanoma that developed in a patient with retinitis pigmentosa (RP). A 66-year-old man was referred to our hospital with a diagnosis of iris tumor. He had a medical history of RP for over 25 years. His best corrected visual acuity at presentation was 20/20 (OD) and 20/200 (OS). Slit lamp examination revealed an iris melanocytoma in his right eye and a brown iris mass involving the pupil in his left eye. Ocular fundus examination of his right eye showed diffuse chorioretinal atrophy with attenuated retinal vessels and scattered pigment. A diagnosis of iris melanoma was made and enucleation of his left eyeball was performed. Histopathological examination of the eyeball showed an iris tumor with proliferation of spindle cells positive for Melan-A, HMB-45, and S-100 protein by immunohistochemistry. Diffuse destruction of retinal architecture was observed, with loss of outer segment and replacement by glial cells. Accumulation of melanin pigment around retinal vessels was found in peripheral retina. These histopathological findings were compatible with advanced stage of RP. This case demonstrates that iris melanoma can occur in eye with RP.

Robotic assisted CyberKnife radiosurgery for the treatment of iris melanoma

Iris melanoma is a rare form of uveal melanoma with potential metastic spread. Treatment options include surgical resection, enucleation or irradiation. We analysed visual outcome, complication appearance and management in eight patients with iris melanoma following robotic-assisted CyberKnife treatment. Consecutive patients from the Department of Ophthalmology at University of Munich were included in the study if they had an iris melanoma that was treated with CyberKnife and had a minimum follow-up of 12 months. We evaluated tumor thickness, largest diameter, visual acuity and complications. 8 patients were included in this report. The median age was 74 years (range: 53–86 years). The median follow-up was 23 months (range 12–48 months). Tumor thickness decreased from 2.1 to 1.4 mm on average. Four out of eight patients showed stable or increased visual acuity compared to visual acuity at first visit. We did not find a correlation of applied radiation volume or radiation dose on visual outcome. Radiation keratopathy was the most common complication in five patients. No recurrences were noted. Robotic-assisted radiosurgery following CyberKnife is a promising non-invasive, single session treatment option for iris melanoma with comparable results regarding recurrence rate or complications to brachytherapy and proton beam therapy. All included patients showed good visual outcome.

Genetics of Ocular Melanoma: Insights into Genetics, Inheritance and Testing

Ocular melanoma consists of posterior uveal melanoma, iris melanoma and conjunctival melanoma. These malignancies derive from melanocytes in the uveal tract or conjunctiva. The genetic profiles of these different entities differ from each other. In uveal melanoma, GNAQ and GNA11 gene mutations are frequently found and prognosis is based on mutation status of BAP1, SF3B1 and EIF1AX genes. Iris melanoma, also originating from the uvea, has similarities to the genetic makeups of both posterior uveal melanoma (UM) and conjunctival melanoma since mutations in GNAQ and GNA11 are less common and genes involved in conjunctival melanoma such as BRAF have been described. The genetic spectrum of conjunctival melanoma, however, includes frequent mutations in the BRAF, NRAS and TERT promoter genes, which are found in cutaneous melanoma as well. The BRAF status of the tumor is not correlated to prognosis, whereas the TERT promoter gene mutations are. Clinical presentation, histopathological characteristics and copy number alterations are associated with survival in ocular melanoma. Tissue material is needed to classify ocular melanoma in the different subgroups, which creates a need for the use of noninvasive techniques to prognosticate patients who underwent eye preserving treatment.

Iris Vascular Malformation with 360-Degree Iridocorneal Angle Affectation

Vascular iris lesions are rare and can sometimes be associated to systemic vascular lesions. They usually cause spontaneous recurrent hyphema. The differential diagnosis should be considered primarily with iris rubeosis and with highly vascular tumors as iris melanoma. Generally, vascular lesions of hemangioma type are located in the iris without extension to iridocorneal angle. We present a case of a 77-year-old male with an iris vascular lesion suggestive of racemose hemangioma, who is asymptomatic, and with 360-degree iridocorneal angle affectation showing no lesions at any other location.

Palladium-103 plaque therapy for multifocal iris melanoma: Radiation of the entire anterior segment of the eye

Purpose: To investigate the safety and tolerability of total anterior segment palladium-103 (103Pd) eye plaque brachytherapy for multifocal iris melanoma. Methods: Interventional case series of 11 patients with multifocal iris melanomas. Anterior segment ultrasound revealed tumor size, location, and intraocular margins. Epicorneal amniotic membrane grafts protected the cornea and decreased pain during total anterior segment 103-Pd ophthalmic plaque brachytherapy. Results: Eleven diffuse iris melanomas were American Joint Committee on Cancer 8th edition–classified as T1 (n = 5, 45.5%) and T2 (n = 6, 54.5%). Plaque radiation was completed to a minimum mean tumor dose of 85 Gy (mean dose rate, 58.1 cGy/h). Ultrasonographic tumor thickness regression was 41% (follow up mean 58.7, median 50, range: 8–139 months). Despite 100% local control and 100% eye retention, one patient (9.1%) developed metastatic disease. Four eyes required cataract surgery. There was no corneal stem-cell deficiency, corneal opacity, radiation maculopathy, or optic neuropathy. While visual acuity prior to treatment was 20/40 or better in 10 (91%), 9 were 20/40 or better (81.9%) at last follow-up. Four (36%) had glaucoma prior to treatment and three eyes developed glaucoma after treatment for a total of 63%. Conclusion: Total anterior segment (103Pd) plaque brachytherapy resulted in local control, good visual acuity, eye and life preservation in the treatment of multifocal iris melanoma.