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Purpose: To report distinctive clinical and imaging features of iris freckles to differentiate them from iris nevi., Design: Retrospective observational study., Subjects: 53 patients (277 freckles) with incidental iris freckles and 102 patients (104 nevi) with iris nevi that are either clinically stable or pathologically confirmed., Methods: Patient data were collected from the Department of Ophthalmic Oncology, Cleveland Clinic, Cole Eye Institute database (2012-2023). Lesion characteristics were recorded from slit-lamp examination descriptions and review of colour photographs. Ancillary imaging features observed using anterior segment optical coherence tomography (AS-OCT) and ultrasound biomicroscopy (UBM) were assessed in patients (where available)., Main Outcome Measures: Comparison of clinical and imaging features of iris freckles and iris nevi., Results: A total of 277 iris freckles and 104 iris nevi were analysed. Iris freckles were more frequently bilateral (17%; nevi 0%) and multiple (69%; nevi 2%) and located centrally (89%; nevi 17%) compared with iris nevi (p<0.001). The median freckle largest basal diameter and thickness were 0.8 mm (nevi; 2.1 mm, p<0.001) and 0.04 mm (nevi 1.0 mm, p<0.001), respectively. All iris freckles had irregular margins without any secondary effects compared with iris nevi. Iris freckles appeared flat without effacement of iris folds compared with iris nevi on AS-OCT (p<0.001). Iris freckles were not detectable by UBM. Heat map revealed that freckles demonstrated several features with uniform or near uniform values, whereas nevi demonstrated more variability in values across features., Conclusions: Iris freckles exhibit specific clinical and imaging features reflective of their characteristic histological composition that support their classification as a distinct entity within the spectrum of iris pigmented lesions., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: To report the clinicopathological features and epidemiology of iris melanoma in Queensland, Australia., Methods: This was a retrospective study of 86 patients with iris melanoma treated between 2001 and 2022 at the Queensland Ocular Oncology Service, Brisbane, Australia. Main outcome measures included demographics, clinical and phenotypic features, age-adjusted incidence and relative survival., Results: Eighty-six patients (63% female) were included. Mean age was 54 years (range 17-82 years). The majority of patients (97%) were Caucasian, with blue eyes, fair skin and Fitzpatrick Skin Type I or II. Demographic features and clinical history showed a tendency for high ultraviolet radiation (UVR) exposure in the cohort. Histopathology was available in 69 cases (82%), and of these, 77% tumours were of spindle cell origin, with low-risk genetic profiles. Patients were followed for a mean of 8 years (median 7, range 1-21 years) after diagnosis, and only one case of metastasis was documented., Conclusions: The association of iris freckles, history of UVR exposure and dermatologic findings supports the role of UVR in iris melanoma. Occupation and avocation history, as well as evaluation of iris freckles may offer an easily accessible way of stratifying the risk of an individual for development of UVR-related uveal melanoma., (© 2024 The Author(s). Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

AimTo develop a predictive model for the diagnosis of iris melanoma., Methods: Retrospective consecutive case series that included 100 cases of pathologically confirmed iris melanoma and 112 cases of Iris naevus, either pathological confirmation or documented stability of >1 year. Patient demographic data, features of clinical presentation, tumour characteristics and follow-up were collected. Iris melanoma with ciliary body extension was excluded. Lasso logistic regression with 10-fold cross-validation was used to select the tuning parameter. Discrimination was assessed with the area under the curve (AUC) and calibration by a plot., Results: There was a significant asymmetry in the location of both nevi and melanoma with preference for inferior iris quadrants (83, 74%) and (79, 79%), respectively (p=0.50). Tumour seeding, glaucoma and hyphaema were present only in melanoma. The features that favoured the diagnosis of melanoma were size (increased height (OR 3.35); increased the largest basal diameter (OR 1.64)), pupillary distortion (ectropion uvea or corectopia (OR 2.55)), peripheral extension (angle or iris root involvement (OR 2.83)), secondary effects (pigment dispersion (OR 1.12)) and vascularity (OR 6.79). The optimism-corrected AUC was 0.865. The calibration plot indicated good calibration with most of the points falling near the identity line and the confidence band containing the identity line through most of the range of probabilities., Conclusions: The predictive model provides direct diagnostic prediction of the lesion being iris melanoma expressed as probability (%). Use of a prediction calculator (app) can enhance decision-making and patient counselling. Further refinements can be undertaken with additional datasets, forming the basis for automated diagnosis., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: With the advent of targeted therapies, the survival prognosis for metastatic tumors has extended, and it has become necessary to diagnose and consider treatment that takes into account Quality of Life for metastatic tumors of the eye. The reports of checking tumor marker in the aqueous humor for diagnosis of metastatic intraocular tumors are few. Here, we report a case of masquerade syndrome with secondary glaucoma in which a high carcinoembryonic antigen (CEA) level in the aqueous humor could assist diagnosis, and continuing targeted therapy and trabeculectomy were effective., Case Presentation: A 73-year-old man was referred to us for iritis and high intraocular pressure (IOP) with severe eye pain in the left eye. He had Stage IVB lung adenocarcinoma treated with a molecularly targeted drug, Osimertinib. His best corrected visual acuity was 0.15, and IOP was 52 mmHg in the left eye. Anterior chamber cells (+), numerous small nodules in the iris, and small masses in the inferior angle were observed. In the aqueous humor, the CEA level was higher than in the blood. Napsin A and Thyroid Transcription Factor-1 (TTF-1) positive cells showed in the resected tissue at iridectomy performed during trabeculectomy. The pathological diagnosis of metastatic iris tumor of the lung adenocarcinoma was made, and we injected bevacizumab intravitreally once and continued Osimertinib. His IOP lowered to 8-10 mmHg, and the iris masses disappeared. He lost vision by metastasis to the left optic nerve after termination of Osimertinib one and a half years later. The metastasis shrank after restarting the drug. He passed away from an exacerbation of his primary lung cancer two years and nine months after the first visit. Although he lost vision in his left eye, the metastatic tumor in his left eye and optic nerve had disappeared, and his quality of life was maintained without any pain in his eye., Conclusions: Checking tumor markers in the aqueous humor can aid in diagnosis, and aggressive treatment of metastatic iris tumors must help maintain patients' Quality of Life., (© 2024. The Author(s).)

The iris is a unique structure, with exquisite variations in colour and form. Pathological changes, specifically including iris cysts and tumours are relatively uncommon, difficult to diagnose, and yet potentially blinding or life-threatening. Based on a comprehensive literature review, with highly illustrated key case examples, this report aims to guide the clinician in filtering the differential diagnoses of iris cysts and tumours. Evaluation is in the context of key diagnostic clinical tools and management considerations. Diagnostic imaging techniques include serial anterior segment photography, ultrasound, anterior segment optical coherence tomography, and iris fluorescein angiography, however, the roles of computerised topography and magnetic resonance imaging are also considered in this review. Management includes categorisation in terms of solid iris tumours (melanocytic vs. non-melanocytic), or iris cysts (primary vs. secondary) that may be usefully differentiated by clinical assessment, avoiding more invasive interventions. Cystic lesions are generally benign, although implantation cysts in particular cause significant complications and surgical challenges. Most solid tumours are melanocytic and also typically benign. However, in larger lesions, rapid growth, symptoms and complications more likely indicate malignancy, requiring further investigation., (© 2024 The Authors. Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To examine complications, visual outcomes, photic patient-reported symptoms, corneal morphology, IOL tilt, and intraocular pressure after implantation of an intraocular lens (IOL) and iris prosthesis (IP) following iridocyclectomy., Methods: Patients with previous iridocyclectomy treated with an IOL and IP at the Copenhagen University Hospital Rigshospitalet between 2007 and 2018 were included in this national retrospective non-comparative case series. The assessment encompassed BCVA, PRO questionnaire, corneal topography, and anterior segment OCT., Results: 45 patients were included. Eight of 45 patients were previously treated with ruthenium-106 brachytherapy in conjunction with iridocyclectomy. Six of 45 patients developed endothelial dysfunction four of whom had received ruthenium-106 brachytherapy. Five of 45 patients had subluxation of the IOL/IP complex due to incomplete zonula apparatus. BCVA improved for all patients after lens surgery. 26 patients participated in the invited follow-up examination. 19 of 26 (73%) reported none or mild photic symptoms after IP instalment. Five (19%) reported ongoing severe photic symptoms. The corneal astigmatism significantly increased after iridocyclectomy but did not change after lens surgery., Conclusions: Implantation of an IOL and IP is a safe procedure, alleviating photic symptoms in most patients. It comes with higher risk of complications due to a more demanding procedure and larger surgical traumas from previous treatments. Ruthenium-106 brachytherapy increases the complication risk. Corneal astigmatism is induced by iridocyclectomy but does not change after lens surgery., (© 2024 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

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., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Considering the similarity in clinical presentations of iris neoplasms of various origins, questions of their noninvasive diagnosis remain relevant. Optical coherence tomography angiography (OCT-A) is one of the imaging method that enables visualization of tumor vessels., Purpose: This article examines the features of angioarchitecture, vascular network density, and perfusion density of iris melanoma and progressive iris nevus using OCT-A., Material and Methods: A total of 67 patients with iris neoplasms were examined between 2021 and 2023. Group 1 included 29 patients with progressive nevus, while group 2 included 38 patients with iris melanoma, which was further divided into two subgroups (A - 32 patients with planar growth, B - six patients with nodular growth). All patients underwent OCT-A with analysis of qualitative and quantitative characteristics. Diagnoses were morphologically verified in all cases., Results: In one-third of cases, OCT-A of pigmented melanomas and progressive iris nevi revealed single hyperreflective vessels, while signal blocking was noted in other cases. In hypopigmented forms of progressive nevus, single hyperreflective vessels were observed, with a mean vascular network density of 5.1±2.9 mm - ¹ and perfusion density of 11.7±3.2%. Hypopigmented forms of iris melanoma showed multiple, chaotically arranged hyperreflective vessels, with a mean density of 11.1±2.6 mm - ¹ ( p <0.05) and perfusion density of 34.3±7.6% ( p =0.01), significantly exceeding these values in progressive nevi., Conclusion: OCT-A enables detection of tumor vessels primarily in hypopigmented forms of iris melanoma and progressive nevus. Increased vascular network density and perfusion, along with the presence of multiple chaotically arranged hyperreflective vessels, allows for differential diagnosis between melanoma and progressive iris nevus and facilitates appropriate organ-preserving treatment.

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.

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.

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 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%., 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., (© 2022. The Author(s).)

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., (© 2022. The Author(s).)

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.

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., (© 2021. The Author(s).)

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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

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., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the distribution of the PAX8 transcription factor protein in ocular tissues and to investigate if immunohistochemical stains for this biomarker are useful in the diagnosis of intraocular tumors., Design: Observational case series., Participants: Excision and cytologic analysis specimens of 6 ciliary body epithelial neoplasms, 2 iris epithelial neoplasms, 3 retinal pigment epithelial neoplasms, 3 intraocular medulloepitheliomas, 15 uveal melanomas, and 5 uveal melanocytomas., Methods: Hematoxylin-eosin and PAX8 immunohistochemical stains were performed on all specimens. In appropriate cases, bleached preparations and other immunohistochemical stains, including AE1/AE3 cytokeratin, Lin28A, and CD45, were performed., Main Outcome Measures: Distribution of PAX8 expression in normal and neoplastic tissue., Results: Strong nuclear PAX8 expression was observed in the normal corneal epithelium, iris sphincter pupillae muscle, iris pigment epithelium and dilator muscle complex, nonpigmented and pigmented epithelia of the ciliary body, lens epithelium, and a subset of retinal neurons. The normal retinal pigment epithelium and uveal melanocytes did not stain for PAX8. The ciliary body epithelial and neuroepithelial tumors (adenoma, adenocarcinoma, and medulloepithelioma) showed uniform strong nuclear PAX8 immunoreactivity. All melanocytic tumors (iris melanoma, ciliary-choroidal melanoma, and melanocytoma) and retinal pigment epithelial neoplasms showed negative results for PAX8. A subset of tumor-associated lymphocytes, most prominent in uveal melanoma, showed positive results for PAX8. The uniformity of the PAX8 staining was superior to the variable cytokeratin staining in the ciliary epithelial neoplasms and the variable Lin28A staining in malignant medulloepithelioma. The veracity of PAX8 staining was equally as robust on cytologic analysis and open-flap biopsy specimens of ciliary epithelial and iris epithelial neoplasms, melanocytoma, and melanoma., Conclusions: PAX8 has proven to be a very useful diagnostic marker in a select group of adult intraocular tumors, and we highly recommend its inclusion in diagnostic antibody panels of morphologically challenging intraocular neoplasms., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Iris lymphomas are rare malignant neoplasms arising either as primary tumors in the iris or as secondary tumors involving the iris. We summarize previously published data and make recommendations for work-up strategies for cases of suspected iris lymphoma. Our objective is to provide a structured overview of the typical clinical symptoms and signs, the pathologic, ophthalmic, as well as hematologic work-up for diagnosis, treatment, and follow-up of iris lymphomas and offer a flowchart on how to diagnose and treat these tumors., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Significance: Iris tumors are rare conditions, and there is a relative paucity of recent published data on its broad clinical spectrum. Tapioca iris melanoma is a rarer yet devastating form with wide and challenging differential diagnoses because of its amelanotic nodular appearance., Purpose: This study aimed to report the challenging presentation of an uncommon iris melanoma, describing the clinical and histological findings and comparing them with the existing published data., Case Report: An uncommon clinicopathological report on the tumor unusual localization, patient age, absence of elevated IOP and heterochromia, and negative S-100 stain that caused diagnostic uncertainty is presented. The patient remains free of metastatic disease 7 years after a complete tumor full-thickness excision., Conclusions: Tapioca iris melanomas are uncommon tumors with a presentation/surgical management that differs from other malignant tumors. Ophthalmologists should consider it among the vast differential diagnoses when observing amelanotic lesions, even without the hallmark signs being evident.

Introduction: In cases of suspected intraocular malignancy, vitreous may be the preferred pathologic sample; however, cellularity may be insufficient for definitive cytopathological diagnosis. Ancillary methodology to study vitreous fluid aspiration for mutational analysis may assist in treatment decisions., Materials and Methods: Three individual patient vitreous humor samples were received in the laboratory for mutation testing. The samples were collected during standard of care and analyzed for routine cytopathology. In each case, cytopathology was inconclusive and mutational analyses to support diagnostic suspicions were clinically requested. Based on the clinically and pathologically suspected diagnoses, an appropriate massively parallel sequencing assay previously validated for clinical use was performed using DNA extracted from vitreous samples that had previously undergone various processing. Nucleic acid yield was assessed by fluorometric or spectrophotometric methods, with yield ranging from 2.7 to 86.5 ng. Library preparations were performed using standard laboratory protocols., Results: Two of the cases were suspicious for melanoma and a 50-gene solid tumor panel was performed. The third case was worrisome for vitreoretinal lymphoma and a 49-gene myeloid panel was performed., Conclusions: In all cases, the molecular profiling assisted with the clinical assessment and/or management of each patient., (Copyright © 2020 American Society of Cytopathology. Published by Elsevier Inc. All rights reserved.)

Background/aim: Metastatic iris tumors are relatively rare entities that are often treated with radiation therapy; however, it remains difficult to evaluate the effectiveness of treatment. Anterior segment optical coherence tomography (AS-OCT) provides high-resolution cross-sectional images with no dye injection, and may thus be useful for evaluating iris tumors. Herein, we report a case of metastatic iris tumor examined with AS-OCT before and after radiation therapy., Case Report: A 56-year-old Japanese woman complained of ocular hyperemia and pain in her right eye, and was referred to our hospital because of uveitis and an iris mass. One year before the initial visit, the patient was diagnosed with lung adenocarcinoma, and treated with chemotherapy. She also underwent radiation therapy 4 months ago due to multiple bone metastases. Slit-lamp microscopy revealed a 2×2 mm elevated lesion with partial depigmentation on the lower iris. AS-OCT examination revealed that the mass was located in the iris stroma with an irregular anterior surface. Based on fine needle aspiration cytology, she was diagnosed with metastatic iris tumor derived from lung adenocarcinoma, leading to local radiotherapy. Seven months later, AS-OCT showed shrinkage and atrophy of the iris tumor with peripheral anterior synechia formation., Conclusion: We report a case of cytology-proven metastatic iris tumor observed with AS-OCT before and after radiation therapy. This study highlights the potential usefulness of AS-OCT for monitoring changes in tumor size and configuration following radiotherapy., (Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background/purpose: To report a unique case of a pulmonary carcinoid tumor unilaterally metastatic to the iris and ciliary body and bilaterally to the choroid that was conservatively followed., Methods: A 46-year-old woman presented with bilateral choroidal lesions and a left iris tumor. Ultrasound biomicroscopy disclosed a ciliary body component. A diagnosis of metastatic carcinoid tumor was made based on the clinical features. Rather than an excision, photodynamic therapy, or radiation treatment, as has been reported in all previous cases of carcinoid tumor metastatic to the iris, the patient was observed., Results: Excellent vision was maintained for 8 years. The iris tumor gradually enlarged, but the choroidal lesions remained unchanged. The iris with the carcinoid tumor gradually acquired a brown pigmentation; this is the first reported case of acquired iris heterochromia in the setting of carcinoid tumor., Conclusion: We conclude, in cases of metastatic carcinoid in which visual acuity is excellent and the patient is asymptomatic, that observation of the ocular lesions is an acceptable course of action. The iris heterochromia is believed to have been caused by secretory factors produced by the tumor.

Objective: To evaluate the benefit of iris biopsy in cats with iris hyperpigmentation to differentiate melanosis from early feline diffuse iris melanoma (FDIM)., Methods: The medical records of cats with unilateral iris hyperpigmentation that had undergone iris biopsy between February 2013 and September 2016 at Willows Veterinary Centre & Referral Service were reviewed., Results: Seven cats with unilateral iris hyperpigmentation were included in this retrospective study. The biopsy procedure was performed under general anesthesia (n = 7) with neuromuscular blockade (n = 6) following pre-operative topical miotic therapy (n = 5). One to six biopsy samples per eye were harvested from areas of hyperpigmentation. The samples were partial thickness (n = 4 eyes) and full thickness (n = 3 eyes). Complications were minor: mild intra-operative hemorrhage (n = 4), fibrin clot (n = 2), corneal ulcer (n = 1), post-operative ocular hypertension (n = 1), dyscoria (n = 1), and pseudopolycoria (n = 2). The first biopsy was diagnostic in six cats; a repeat biopsy was necessary in one cat. Histopathology was consistent with melanosis in five cats and with early FDIM in two cats. Screening for signs of metastatic disease (thoracic computed tomography and abdominal ultrasonography) was negative in the two cats with a preliminary diagnosis of early FDIM. Subsequent enucleation and histopathology confirmed the initial diagnosis in both cases., Conclusions: Iris biopsy in cats with iris hyperpigmentation can be beneficial to differentiate melanosis from early FDIM and thereby help to justify the decision for early enucleation., (© 2019 American College of Veterinary Ophthalmologists.)

Purpose: The diagnosis of iris melanoma can be difficult, with no established diagnostic criteria currently available. Careful monitoring of patients with suspicious iris lesions is one approach to managing these tumors. We determined the risk of malignant transformation and melanoma-related mortality in patients under observation to evaluate the validity of this management approach., Methods: This was a retrospective chart review of patients with suspicious iris lesions diagnosed at Massachusetts Eye and Ear Infirmary (MEE) between 1975 and 2014. All patients with an initial diagnosis of suspicious iris lesion followed and/or treated after malignant transformation at the MEE in this 39-year period were included in the cohort. Rates of malignant transformation and melanoma-related mortality were calculated. Treatment outcomes after proton beam irradiation were evaluated in patients who developed iris melanomas during observation., Results: Two hundred thirty-four patients had a diagnosis of suspicious iris lesion (median follow-up, 5.8 years). Malignant transformation occurred in 16 (6.8%) patients with suspicious lesions during the observation period (median follow-up, 9.9 years). All patients diagnosed with iris melanomas were treated with proton beam irradiation (PBI). Complications after treatment included cataract (18.8%), secondary glaucoma (6.3%), and neovascular glaucoma (12.5%). Two of 16 patients (12.5%) who developed iris melanomas died of metastatic melanoma 32.6 months and 10 years after treatment with PBI. Both cases had been followed regularly to monitor for malignant transformation of their suspicious lesions (8.2 years and 3.2 years before melanoma diagnosis, respectively)., Conclusions: These data suggest that suspicious iris lesions have low malignant potential, and a conservative approach to the management of these lesions is appropriate. Survival does not appear to be compromised with an observational approach, and there is potential for preservation of good visual function because vision-threatening treatments can be avoided.

Competing Interests: The authors declare that they have no conflict of interest.

Objectives: Feline diffuse iris melanoma (FDIM) is the most common malignant primary intraocular tumour in cats, with reported metastases rates between 19% and 63%. Currently, the only available diagnostic tool for a tentative diagnosis is histopathological examination of the enucleated eye. Therefore, the veterinary ophthalmologist is often faced with the dilemma of whether to enucleate an oftentimes visual eye or to continue monitoring, with the risk of metastases developing. In the past, cell-free DNA (cfDNA) gained more attention in human medicine, especially in the field of oncology. Prior studies have shown the use of cfDNA as diagnostic or prognostic markers in canine and human cancer patients. Therefore, the aim of this study was to investigate cfDNA concentration and integrity in cats with FDIMs compared with cats with benign iris naevi and without ocular abnormalities., Methods: cfDNA from plasma of cats with iris melanoma (n = 34), iris naevus (n = 30) and without ocular abnormalities (n = 32) were extracted. Primer and probes for feline amyloid beta precursor protein ( APP) and beta actin ( ACTB) were designed for amplicons of various lengths and quantitative PCRs of extracted cfDNA were performed to measure cfDNA concentration and integrity of the plasma samples. Differences of cfDNA concentrations and integrity levels between the three groups (iris melanoma, iris naevi and controls) were analysed using the Mann-Whitney U-test., Results: cfDNA concentration and integrity analysis revealed no significant differences between the cats with iris melanoma, iris naevus or the control group ( P >0.01). Cats with metastases showed similar cfDNA concentration and integrity to cats without metastases., Conclusions and Relevance: cfDNA concentration and integrity seem to be insufficient as a diagnostic or prognostic marker in cats with FDIMs.

We report an unusual case of an amelanotic melanoma in a 7 year old hispanic child with subclinical globe perforation. Uveal melanoma rarely occurs in children. Young affected patients are mostly light-colored eye Caucasian adolescents. Since they are not common, these tumors are usually not recognized and misdiagnosed. Differential diagnoses and therapeutic options are outlined., (Copyright © 2018 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objective: To investigate the validity and safety of tele-ophthalmology evaluations as a clinical assessment tool when performed by an ophthalmologist to detect lesion growth in patients with low-, medium-, and high-risk choroidal and iris nevi., Design: Retrospective observational pilot study., Methods: Consecutive patients with low-/medium-/high-risk choroidal or iris nevi who underwent tele-oncology examinations over 5 months. All patients had a dilated fundus or anterior segment photography, A- and B-scan ultrasonography or ultrasound biomicroscopy (UBM), and spectral domain optical coherence tomography (SD-OCT) depending on the nature of their lesion. Patients who followed up with in-person examinations had an additional ophthalmoscopic examination., Results: Seventy-one eyes of 71 patients were included. The diagnoses were 47 low-risk choroidal nevi, 10 medium-risk choroidal nevi, 5 high-risk choroidal nevi, and 9 iris nevi. The tele-ophthalmology examinations found a sensitivity of 100%, specificity of 92%, positive predictive value of 57%, and negative predictive value of 100% to detect growth of a lesion., Conclusions: Tele-ophthalmology assessment for choroidal and iris nevi is a sensitive clinical tool to evaluate growth with 100% sensitivity and negative predictive value when performed by trained ultrasound technicians and reviewed by an ophthalmologist with expertise in ocular oncology. It has the potential to alleviate patient- and physician-related treatment burden., (Copyright © 2017 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)