Anterior plaque brachytherapy placement for treatment of iris and iridociliary melanomas - Surgical procedure and institutional experience.

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.

A distorted pupil could be caused by an iris melanoma.

Melanoma isolated to the iris is rare and can present with a distorted pupil. This is a case report of an 81-year-old asymptomatic man, who had a large pigmented element in his left iris through 30 years. Because of involvement of the angle the tumour was excised with the ciliary body, and histopathologic examination revealed an iris melanoma. The aim of this report is to underscore the clinical signs of an iris melanoma and when surgery is needed.

Focal choroidal excavation associated with nonmelanocytic iris tumor.

PURPOSE: To describe a unique unilateral association between an iris stromal tumor and a macular focal choroidal excavation. CASE DESCRIPTION: A 40-year old patient presented with a small iris tumor associated with a unilateral macular lesion disclosed during a routine ophthalmologic examination. The patient was asymptomatic and visual function was not affected. After clinical and instrumental evaluation, a diagnosis of nonmelanocytic undefined stromal tumor of the iris associated with macular focal choroidal excavation was made. The size and shape of the two lesions remained stable during a 7-year follow-up and the patient did not develop other signs. CONCLUSION: The concurrent presence of a stromal iris tumor associated with focal choroidal excavation has never been reported. Further reports of this association are required in order to understand its exact pathogenesis.

Iris melanocytic tumours in New Zealand/Aotearoa: presentation, management and outcome in a high UV exposure environment.

BACKGROUND/OBJECTIVES: 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. SUBJECTS/METHODS: 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. RESULTS: Cohort (N = 51) was predominantly NZ European (98.0%) with no indigenous Maori, 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%. CONCLUSION: 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.

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.

Glomus tumour of the iris: A case report.

INTRODUCTION: Glomus tumours are rare benign tumours formed by modified smooth muscle cells arising from the glomus body. Glomus tumours occurring in the iris have not been previously reported. CASE DESCRIPTION: A 32-year-old woman presented with a 9-day history of blurred vision in her right eye. Ultrasound, computed tomography and magnetic resonance imaging confirmed the presence of a mass lesion within the iris. Surgery of the iris was performed and the tumour was removed. Histopathological analysis confirmed a glomus tumour. The patient remains clinically stable 5 months following surgery and has experienced no tumour recurrence. CONCLUSION: The findings from this case suggest that the typical symptoms of a glomus tumour may be absent in some cases, and that imaging examinations can help in understanding the extent of the lesion and the involvement of adjacent structures. Moreover, pathology and immunohistochemistry are crucial to confirm the diagnosis.

Case Series: Different Presentations of Iris Melanoma-Potential Masquerade of Benign and Malignant.

SIGNIFICANCE: Iris melanoma and iris nevi can be challenging to distinguish clinically. This case series provides unique insight into the rare condition and variable clinical presentations of iris melanoma. PURPOSE: This study aimed to highlight the varying clinical presentations of iris melanoma and to demonstrate the overlapping features of melanoma and nevi. CASE REPORTS: This case series includes five patients of varying age and sex who presented to clinic with pigmented iris lesions. These five patients have differing timeline to presentation and very different clinical presentations of their lesions. Clinical evaluation was based around the established "ABDCEF" guide for the assessment of malignant risk in iris lesions. The presentation of each lesion is discussed in relation to this guide and the experienced clinician's clinical suspicion of malignancy. When comparing the clinical suspicion with histological analysis, after biopsy, the result may be unexpected. Notably, initially benign nevi may transform into melanoma over time. These five cases were managed on an individual basis because the management and prognosis of iris melanomas vary significantly. Importantly, iris melanotic lesions have variable metastatic risk based on cytology and genetic predisposition. Informed consent was obtained from all the patients, institutional approval was obtained, and no identifiable health information is included in this case series. CONCLUSIONS: When presented with a pigmented iris lesion, clinicians must be vigilant with regular monitoring and have a low threshold for biopsy in pigmented lesions of high clinical suspicion.

Ectopic thyroid tissue in the iris: a case report.

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. CASE PRESENTATION: 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.

Iris metastasis as the initial presentation of metastatic esophageal cancer diagnosed by fine needle aspiration biopsy: A case report.

RATIONALE: 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. PATIENT CONCERNS: A 70-year-old male complained of redness and discomfort in the right eye. DIAGNOSIS AND INTERVENTIONS: 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. OUTCOMES: 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. LESSONS: 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.

Anterior Segment OCTA of Melanocytic Lesions of the Conjunctiva and Iris.

PURPOSE: To study the feasibility and diagnostic value of vascular imaging using optical coherence tomography (OCT)-angiography (OCTA) of melanocytic lesions of the conjunctiva and iris. DESIGN: Cross-sectional study. METHODS: Twenty-five patients with an untreated conjunctival lesion (5 melanoma, 13 nevus, 7 primary acquired melanosis [PAM]) and 52 patients with an untreated iris lesion (10 melanoma, 42 nevus) were included. Patients were imaged using a commercially available OCTA device, with the addition of an anterior segment lens and manual focussing. Tumor vessel presence, vascular patterns and vascular density were assessed. RESULTS: Good OCTA images were obtained in 18 of 25 conjunctival lesions and 42 of 52 iris lesions. Failure was caused by lack of patient cooperation, an unfavorable location, or mydriasis. In all imaged conjunctival lesions and 77% of iris lesions, vascular structures were detected. Conjunctival melanoma and nevi demonstrated the same intralesional tortuous patterns, whereas vasculature in eyes with PAM was similar to normal conjunctiva. Both iris melanoma and nevi demonstrated tortuous patterns, distinct from the radially oriented normal iris vasculature. CONCLUSIONS: Optical coherence tomography angiography (OCTA) allows for noninvasive imaging of the vasculature in melanocytic lesions of the conjunctiva and iris. Good image quality depends highly on patient cooperation and lesion characteristics. Differentiation of benign and malignant lesions was not possible. New software is called for to improve image acquisition and analysis.

Clinical Spectrum of Uveal Metastasis in Korean Patients Based on Primary Tumor Origin.

PURPOSE: To describe the clinical features and prognosis of patients with uveal metastasis in Korea. DESIGN: Retrospective, observational case series. PARTICIPANTS: Patients diagnosed at 2 tertiary high-volume centers between November 2005 and November 2019. METHODS: Evaluation of multimodal imaging and electronic medical records. MAIN OUTCOME MEASURES: The clinical features and outcomes were assessed based on the primary cancer site. RESULTS: A total of 134 uveal metastases (128 choroidal, 3 iris, and 3 ciliary body tumors) were diagnosed in 95 eyes of 80 patients. Mean age at diagnosis was 56 years (median, 55 years; range, 24-86 years), with a minor preponderance of women (61%). Tumors were bilateral in 15 patients (19%) and the primary origin was established in 49 patients (61%) before ocular detection. The primary tumor originated in the lung (48%), breast (24%), gastrointestinal tract (10%), liver (3%), pancreas (3%), kidney (1%), cervix (1%), and nasopharynx (1%), with some remaining unknown (10%). The overall 5-year survival rate was 21%. Kaplan-Meier analysis revealed that the worst survival was found in pancreatic cancers (mean survival, 5.9 months; P = 0.045), and the best survival was found in gastrointestinal tract cancers (mean survival, 44.5 months). CONCLUSIONS: The primary tumor origins in Korean patients with uveal metastases differed from those reported in primarily population-based studies of White patients, with a higher prevalence of lung and gastrointestinal tract cancers.