Surgical placement of eye plaque brachytherapy (EPB) is the standard of care for the treatment of uveal melanomas, including iris/iridociliary melanomas. However, unique challenges exist in anterior EPB placement. Here, we describe a surgical technique for anterior EPB placement when placement requires plaque positioning onto the cornea. Blunt conjunctival peritomy exposes the sclera overlying the tumor. A \"dummy\" plaque is placed, with positioning confirmed by direct visualization. The amniotic membrane is draped across the cornea and anchored with the eyelet sutures, the plaque is placed overlying the membrane, the conjunctiva is closed over the plaque, and a temporary tarsorrhaphy is performed. One week later, the conjunctival incision is reopened for plaque/amniotic membrane removal. This technique was employed in the treatment of 12 iris/iridociliary melanomas at our institution, with no instances of corneal damage. In placing an anterior plaque, employing this technique allows appropriate cancer treatment while optimizing patient comfort and corneal integrity.

Von Recklinghausen disease or neurofibromatosis type I (NF1) is the most common phacomatosis. It is inherited in an autosomal dominant manner. Lisch nodules are the most common ophthalmic manifestation of NF1. They are reported in 73-95% of cases. We here report the case of a 49-year-old female patient referred for a consultation with an ophthalmologist for routine eye check-up after the onset of the following symptoms: coffee-with-milk colored spots and multiple neurofibromas (A). She reported similar family history. Visual acuity was 10/10 P2 in both eyes. Examination of the anterior segment showed several Lisch nodules in both irises. They were scattered over the entire iris surface and varied in size (B). Fundus examination was unremarkable. CT scan of the brain and the orbit as well as thoracoabdominal CT scan showed no associated lesion. The patient met NIH diagnostic criteria for NF1. Lisch nodules are small brown dome-shaped lesions developing on the surface of the iris, with well-defined edges, embedded in the stroma and lighter than iris pigmentation. Differential diagnosis includes iris mammillations, iris naevi, iris melanoma, iris granulomatous nodules. Unlike skin signs, the presence of multiple Lisch nodules are considered a specific indication of NF1. These nodules may occur in childhood and their prevalence and number increase with age. Thus, periodic eye examinations in subjects with suspected neurofibromatosis type I may help to improve early diagnosis.

To determine population-based incidence of intraocular tumours in Olmsted County, Minnesota.
Record review of the Rochester Epidemiology Project medical record linkage system from 1 January 2006 to 31 December 2015 for patient demographics, tumour type by clinical diagnosis and presence or absence of confirmation by histopathology. The incidence rate of any intraocular tumour and of each tumour type was calculated per million person-years. Poisson regression analysis was used to analyse changes in incidence over time.
There were 948 patients diagnosed with intraocular tumours resulting in an age-adjusted and sex-adjusted incidence rate of 727.5 per million (95% CI: 680.8 to 774.2, p<0.05). Most tumours were benign (953, 98%). Of the benign lesions, melanocytic lesions were the majority (942, 97%), with adjusted incidence rates of 646.9 (95% CI: 602.8 to 691.1) for choroidal nevus and 55.8 (95% CI: 43.2 to 64.8) for iris nevus. Malignant lesions were rare (16, 2%) with 13 cases of choroidal melanoma and 1 case each of iris melanoma, retinal leukaemic infiltration and metastasis. The adjusted incidence rate for choroidal melanoma was 7.1 (95% CI: 2.5 to 11.8).
In a population-based setting, most intraocular tumours are benign and melanocytic. Although malignant lesions are less common, it is important to remain vigilant with appropriate monitoring given the potential for vision loss and life-threatening malignancy.

To identify factors predictive of post-management secondary glaucoma in eyes with iris melanoma.
Patients with iris melanoma who were conservatively managed on the Ocular Oncology Service, Wills Eye Hospital from 1970 to 2016 were included. Charts were retrospectively reviewed, and binary logistic regression analysis was performed. Main outcome measures were factors predictive of post-management glaucoma, defined as intraocular pressure (IOP) > 22 mmHg following melanoma treatment.
Of 271 patients with iris melanoma, melanoma-related glaucoma was identified in 40 (15%) at presentation and post-management glaucoma developed in 75 (28%) at a mean of 103.7 months (range:1.0-120.0). Comparison (post-management glaucoma vs. no glaucoma) revealed patients with post-management glaucoma presented with worse visual acuity (20/50-20/150) (17% vs. 5%, p = 0.001), increased mean tumour basal diameter (5.1 mm vs. 4.3 mm, p = 0.004), greater melanoma-related increased IOP on presentation (24.1 mmHg vs. 16.2 mmHg, p < 0.001), diffuse tumour shape (9% vs. 4%, p = 0.01), American Joint Committee on Cancer (AJCC) T4 category (7% vs. 2%, p = 0.03), and extraocular tumour extension (7% vs. 2%, p = 0.03). Risk factors for post-management glaucoma identified by multivariate analysis included melanoma-related increased IOP at presentation (OR:1.1, [1.08-1.22] per 1-mmHg increase, p < 0.001), increased mean tumour basal diameter (OR:1.17, [1.02-1.33] per 1-millimetre increments, p = 0.03), advanced AJCC clinical T subcategory (OR:1.23, [1.04-1.46] per 1-subcategory increments, p = 0.02) and plaque radiotherapy treatment (OR:2.32, [1.13-4.75], p = 0.02).
Features of iris melanoma that predicted post-management glaucoma included melanoma-related increased IOP on presentation, advanced AJCC clinical T subcategory, increased mean tumour basal diameter, and plaque radiotherapy treatment.

Iris melanoma, a rare intraocular malignancy, represents the smallest subgroup of uveal melanoma. This first, comprehensive study of iris melanocytic lesions in the high ultraviolet environment in New Zealand/ Aotearoa (NZ) examines diagnosis, management and outcomes.
Retrospective study of iris melanocytic tumours referred to tertiary referral centres in Auckland, NZ, over 20 years (1999-2018). Data analysed include demographics, tumour characteristics, histology, genetic analyses, treatment modalities, recurrence, metastasis, 5-year and overall survival.
Cohort (N = 51) was predominantly NZ European (98.0%) with no indigenous Māori, or Pasifika. Median age at presentation was 58 years. Tumours involved a median of two clock hours of iris. The posterior tumour margin extended to the anterior chamber angle in 22 patients (45.8%). Management included initial observation 54.9%, iridectomy/excision biopsy 29.4%, irido-cyclectomy 7.8%, plaque radiotherapy 7.8%, proton beam radiotherapy 7.8%, and ultimately enucleation 17.6%. Histology was performed in 19 cases (37%) with 16 confirmed melanomas (84%). Mean follow-up 4.2 years with median visual acuity of 6/7.5 two years post intervention. Melanoma-related metastasis and mortality occurred in two cases with five-year melanoma-related mortality of 2.0%.
In a climate with high ultraviolet exposure iris melanocytic tumours occurred almost exclusively in NZ Europeans, however, the majority of cases were category T1, possibly reflecting early diagnosis in the NZ health system. Nonetheless, >50% underwent surgery or radiotherapy, often utilising more than one modality. A high index of suspicion and early referral of iris melanocytic lesions should be considered in regions with high UV exposure.

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.

BACKGROUND: Ectopic thyroid tissue in the iris, also known as a thyroid glandular epithelial choristoma of the iris, has only been described twice in the literature. In both cases it remained asymptomatic.
METHODS: A 67-year-old female patient presented for the first time in mid-2017 with corneal endothelial decompensation, with a history of complicated cataract surgery and IStent® implantation. Slit lamp microscopy showed endothelial decompensation, pseudophakia, anterior synechiae and a whitish iris tumour adhering to the endothelium. The latter had existed since childhood. Given these findings, reduced visual acuity of hand movement perception and an intraocular pressure of 23 mmHg, we performed a keratoplasty combined with an en bloc resection of the iris tumour at 9 o\'clock and sector iridectomy at the end of 2019. Histological and immunohistological examination of the iris tumour unexpectedly revealed thyroid tissue. After the procedure described above, the patient had an increase in visual acuity while the graft stayed clear and the eye showed no evidence of tumour recurrence or other complications.
CONCLUSIONS: We report a third case of ectopic thyroid tissue in the iris. Both previous cases remained asymptomatic, whereas in our case, size and location of the ectopic thyroid tissue contributed to a more complex cataract surgery resulting in endothelial decompensation. Therefore, in such cases appropriate patient information should be provided prior to cataract surgery. Furthermore, careful histological examination and examination of the thyroid is important to exclude malignant diagnoses such as a metastasis of a follicular thyroid carcinoma.

OBJECTIVE: To describe a patient with an unusual presentation of iris metastasis from breast cancer and her response to systemic therapy.
METHODS: Retrospective chart review of one patient.
RESULTS: A 57-year-old woman presented with a superonasal translucent vascularized iris stromal mass with fish egg-like structures budding from the surface. High-frequency anterior segment ultrasonography demonstrated a solid iris stromal mass measuring 6.0 mm × 3.3 mm × 1.9 mm. On optical coherence tomography, the egglike structures appeared as hyperreflective spheres, some of which were detached from the main iris stromal tumor. Oncologic evaluation revealed metastatic breast cancer involving the brain and lung. She was treated with oral abemaciclib and letrozole, as well as external beam radiotherapy to the brain. The iris mass had completely regressed within 4 months and remained undetectable through the 8-month follow-up. The other metastatic lesions responded well to therapy.
CONCLUSIONS: A case of iris metastasis was reported as the presenting sign of cancer dissemination that was successfully treated with targeted systemic therapy without ocular radiotherapy.

BACKGROUND: Metastasis of neoplasms to the eye is quite uncommon. In this case report, we describe a patient where primary esophageal cancer was diagnosed by fine needle aspiration biopsy (FNAB) of an iris tumor.
METHODS: A 70-year-old male complained of redness and discomfort in the right eye.
METHODS: The patient\'s right eye was diagnosed as idiopathic uveitis, and a topical steroid was administered. As vitreous opacities were observed even after topical therapy, oral prednisolone was administered. On slit-lamp examination of the right eye, an iris mass with neovascularization was seen in the anterior chamber. A metastatic tumor was suspected, and FNAB was performed. Histology revealed squamous cell carcinoma. Systemic workup revealed esophageal cancer with several metastases. Best-corrected visual acuity decreased to 20/400, and intraocular pressure was 40 mmHg in the right eye. Two iris tumors with neovascularization were present extending into the anterior chamber with posterior iris synechiae and 360 degree peripheral anterior synechiae. Intraocular pressure in the right eye was medically managed with hypotensive eye drops and oral acetazolamide. Iris metastases were treated with 40 Gray of radiation therapy and concurrent chemotherapy.
RESULTS: The tumor regressed, but intraocular pressure was refractory to treatment because of 360 degree goniosynechial closure. The right eye lost light perception six months after treatment commenced, and the patient died 9 months after the onset of therapy due to multiple systemic metastases.
CONCLUSIONS: This is a rare case of masquerade syndrome without systemic symptoms in which FNAB of an iris tumor led to a diagnosis of metastatic esophageal squamous cell carcinoma. Although the patient lost his sight due to uncontrollable ocular hypertension, systemic chemotherapy, and radiation therapy were initially effective in the treatment of the metastatic iris tumor. As the prognosis of patients with metastatic iris tumors is poor, it is important for ophthalmologists to consider such diagnoses and conduct systemic investigations when necessary.

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