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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.)

Objectives: Differentiation of iris and ciliary body lesions as benign or malignant and cystic or solid is important. The aim of this study was to compare anterior segment swept-source optical coherence tomography (AS SS-OCT) and ultrasound biomicroscopy (UBM) findings in iris and ciliary body tumors., Research Design and Methods: Forty-two eyes of 38 cases with iris and ciliary body tumors imaged with UBM and AS SS-OCT between September 2018 and September 2023 were evaluated retrospectively., Results: Of 42 eyes, 14 had melanoma, 14 iris pigment epithelial (IPE) cysts, 7 nevi, 3 Lisch nodules, 2 iris stromal cysts, 1 pars plana cysts, and 1 iris mammillations. An equivalent (100%) visualization of the anterior tumor margin was obtained with both techniques. Compared to AS SS-OCT, UBM was superior for posterior margin visualization in melanocytic tumors and IPE cysts. Bland-Altman plots demonstrated good agreement between UBM and AS SS-OCT for melanocytic tumors < 2.5 mm in base diameter and < 2 mm in thickness., Conclusions: Although, UBM is the gold standard for ciliary body and iridociliary tumors. AS SS-OCT should be considered as an excellent alternative to UBM, especially in minimally elevated iris lesions.

Introduction: Magnetic resonance imaging (MRI) has demonstrated high levels of tissue contrast, accuracy and reproducibility in evaluating posterior uveal melanoma. Owing to smaller size, the role of MRI in detecting and characterising iris melanoma has not yet been explored., Aims: To develop a protocol to image iris melanoma and describe the MRI characteristics of histopathological-confirmed iris melanoma., Materials and Methods: An optimised MRI protocol, using a 3T MRI scanner and a 32-channel head coil, was developed to image iris tumours. A prospective, single-centre, 12-month study was conducted on all patients with lesions suspicious for iris melanoma. All patients were offered an MRI scan in addition to the standardised clinical procedures. Image quality comparison was made with existing clinical investigations. Iris melanoma characteristics on MRI are described., Results: A successful optimised MRI scan protocol was developed that was able to detect and characterise iris melanoma. One normal participant and five patients with subsequent histopathological-confirmed iris melanoma (n = 6) were recruited. Four patients completed the full MRI sequence. All iris melanoma were detected on at least one T1- or T2-weighted images. When compared to the vitreous, all iris melanomas demonstrated hyper-intensity on T1-weighted images and hypo-intensity on T2-weighted images. On T1-mapping, T1-values of iris melanoma demonstrated an inverse relationship with the degree of tumour pigmentation., Conclusions: This study highlights an optimised, easily reproducible MRI scan protocol to image iris melanoma. Numerous MR imaging characteristics of iris melanoma are reported for the first time and a potential non-invasive tumour biomarker is described., (Copyright © 2024. Published by Elsevier Inc.)

Background: Gastric cancer has a wide spectrum of clinical features, imaging manifestations, and pathology. Punctate calcifications in gastric cancer are infrequent but are usually found in mucinous adenocarcinoma. However, there have only been a few autopsy case reports describing the correlation between the radiology and pathology findings of calcified mucinous adenocarcinoma of the stomach. We present an autopsy case of mucinous gastric adenocarcinoma with iris metastases as the initial symptom., Case Presentation: A 74-year-old Japanese woman presented with blurred vision. Her treating ophthalmologist diagnosed acute iritis with secondary glaucoma. The histopathological and immunohistochemical features of a trabeculectomy specimen favored metastatic carcinoma, most likely of gastrointestinal tract origin. Esophagogastroduodenoscopy revealed multiple irregularly shaped ulcerative lesions, multiple erosions, and thickened folds in the corpus of her stomach. Histologic examination of a gastric tissue specimen obtained by endoscopic biopsy revealed poorly differentiated carcinoma with signet ring cell features. Computed tomography revealed a tumor with multiple punctate calcifications in the thickened gastric wall with diffuse low attenuation and multiple lymph node metastases, including the para-aortic lymph nodes, and peritoneal dissemination. She was diagnosed with stage IV gastric cancer (T4N3M1) and underwent seven cycles of 5-weekly TS-1, a novel oral fluoropyrimidine derivative, plus cisplatin therapy. Serial follow-up computed tomography revealed successive increases in the gastric wall calcifications. Her disease stabilized, but she died of aspiration pneumonia 8 months after the first visit. Autopsy tissue specimens had miliary, punctate calcifications present in abundant extracellular mucin pools in the submucosa, corresponding to the thickened low-attenuating middle layer on computed tomography. The final diagnosis was mucinous gastric adenocarcinoma because mucinous adenocarcinoma is diagnosed when more than half of the tumor area contains extracellular mucin pools., Conclusions: We report the pathology and computed tomography imaging characteristics of a case of calcified mucinous adenocarcinoma of the stomach metastatic to the iris, including findings at autopsy. Metastatic carcinomas in the iris originating in the stomach are exceedingly rare. Multiple punctate calcifications were present in pools of extracellular mucin, a diagnostic clue for mucinous adenocarcinoma. Possible mechanisms underlying scattered punctuate calcifications in gastric mucinous adenocarcinoma warrant further investigation.

Extraocular infantile hemangiomas have been shown to respond to oral and topical beta blockers, while there is little literature regarding the management of intraocular infantile hemangiomas with beta blockers. This case report discusses the management of an iris hemangioma with topical timolol, a treatment previously unreported in the literature., (Copyright © 2018 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To report a case of solitary iris plasmacytoma successfully treated with ruthenium plaque radiotherapy., Methods: A 44-year-old white woman presented with pain in the right eye and raised intraocular pressure. Her medical history included breast cancer treated 11 years earlier with lumpectomy, lymph node clearance, chemotherapy, and radiotherapy. On examination, the right iris had a tan-colored mass with crystalline deposits visible on the mass surface and on the corneal endothelium. The fundus and left eye were normal. An anterior segment ultrasound scan showed a mass with mixed internal echogenicity and internal blood flow., Results: An iris biopsy showed an infiltrate of plasma cells. Immunohistochemistry for kappa and lambda light chains demonstrated lambda light chain restriction. Systemic hematologic investigations including complete blood count, serum paraproteins, bone marrow biopsy, and full-body magnetic resonance image were normal. The monoclonal plasma cell infiltrate was consistent with a solitary iris plasmacytoma. The mass was treated with ruthenium plaque radiotherapy. After 4 years of follow-up, the mass remained regressed, and no systemic myeloma has developed., Conclusions: Iris plasmacytoma is rare and should prompt systemic evaluation to rule out multiple myeloma. Solitary iris plasmacytoma can be successfully treated with plaque radiotherapy.

Ocular metastasis is relatively uncommon, with a reported incidence of approximately 8%, according to the results of autopsy evaluation. The majority of ocular metastases are located within the choroid, while metastatic tumors affecting the iris are rare. Metastatic tumors may manifest as stromal nodules or ill-defined iris thickening, or they may present with nonspecific features such as pain, iridocyclitis, and hyphema. Here, we describe three patients with iris metastasis and discuss the diagnostic challenges and unusual findings associated with these cases.

Purpose: To describe an unusual case of pediatric iris melanocytoma with pigment dispersion glaucoma that resolved after resection of the primary tumor., Methods: Retrospective case review of the clinical record, ultrasonographic images, and histopathology., Results: A 6-year-old Asian girl, with a dark iris tumor, pigment dispersion, and secondary glaucoma, was initially treated with topical antiglaucoma medication and observation. Rapid growth prompted biopsy, revealing melanocytoma. As the tumor continued to grow, excision of the primary tumor was performed. Surgery proved curative in that the pigment dispersion slowly reabsorbed and her glaucoma resolved., Conclusions: In this case, rapid growth did not indicate malignant transformation. Initial observation for growth and judiciously timed surgical intervention prevented progression, loss of vision, and potentially the loss of the eye.

Case Report: We present a case of plateau iris and glaucoma due to multiple unilateral iridociliary cysts. The patient was treated with iridotomy Nd: YAG laser and 360° iridoplasty, without achieving pressure control. Phacoemulsification improved the hypertension. Dynamic gonioscopy and OCT of the anterior chamber was also performed before and after treatment., Discussion: Iridociliary cysts are a benign condition that can cause iris plateau configuration, and can produce a difficult to treat ocular hypertension. Cystotomy, peripheral iridoplasty, and other treatments have been proposed., (Copyright © 2014 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Iris metastasis is one of the rare forms of ocular metastasis. Lung and breast cancers represent more than two thirds of the primary tumor sites in such patients. We here present the F-FDG PET/CT findings in a 60-year-old male patient with small cell lung cancer where metastasis to iris was incidentally discovered on PET/CT.

Purpose: A case of a teenage girl with tapioca melanoma of the iris is presented. This case is unusual, as the patient did not have heterochromia and did not present with elevated intraocular pressure., Case Report: A 14-year-old female patient presented with an amelanotic, multinodular, multifocal lesion of the right iris. Pathology confirmed a diagnosis of tapioca melanoma using immunohistologic staining. The patient underwent enucleation of her right eye and has been free of metastatic disease 3 years later., Conclusions: Tapioca melanoma of the iris must be included among the other differential diagnoses when examining patients with amelanotic iris lesions, even when iris heterochromia is not clearly evident.

Purpose: To evaluate the outcomes of iris melanoma managed with plaque radiotherapy on the basis of the initial presence or absence of glaucoma., Design: Retrospective, comparative case series., Participants: A total of 144 patients., Intervention: Custom-designed iodine-125 plaque radiotherapy delivering planned 8000 cGy to melanoma apex using transcorneal application., Main Outcome Measures: Tumor control and treatment-related complications., Results: Of 144 patients with iris melanoma, glaucoma was present at the initial visit in 58 (40%). Causes of elevated intraocular pressure included angle infiltration by melanoma in 50 patients (86%), angle neovascularization in 4 patients (7%), and hyphema in 4 patients (7%). At presentation, the eyes displaying iris melanoma with glaucoma (vs. without glaucoma) were statistically more likely to display angle tumor (66% vs. 43%), with minimal thickness (1.9 vs. 2.9 mm), and melanoma seeding in iris stroma (7 vs. 3 clock hours) and angle (5 vs. 2 clock hours). Plaque radiotherapy was performed in all cases. Kaplan-Meier estimates at 7 years post-treatment revealed no statistical differences in outcomes of local recurrence (14% vs. 15%), enucleation (14% vs. 11%), or metastasis (2% vs. 0%) comparing eyes with and without glaucoma. Of the entire group, multivariate analysis for factors predictive of recurrence included partial (vs. complete) anterior segment irradiation and postradiotherapy glaucoma. Factors related to enucleation included diabetes mellitus, poor initial visual acuity, higher radiation dose to tumor apex, and tumor recurrence. There were no factors predictive of metastasis., Conclusions: Iodine-125 plaque radiotherapy provides adequate tumor control for iris melanoma with a low metastatic potential of 1% at 7 years. Iris melanoma with secondary glaucoma showed a statistically significant greater likelihood of flat tumor with iris and angle seeding and no difference in outcomes compared with eyes without glaucoma., Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article., (Copyright © 2013 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background: To describe the clinical and histopathological features of lymphomas involving the iris., Design: Retrospective, descriptive study., Participants: Fourteen eyes of 13 patients., Methods: Review of medical records., Main Outcome Measures: Clinical and histopathological findings., Results: Median patient age was 58 years (range, 25-76 years). Seven patients had known systemic lymphoma of which five were of large B-cell type. Symptoms included blurred vision (8/14), eye redness (3/14) and eye pain (2/14). Four eyes had secondary elevated intraocular pressure. Anterior segment findings included anterior chamber cells (14/14 eyes), keratic precipitates (11/14 eyes), congestion of conjunctival/episcleral blood vessels (9/14), hyphaema (7/14), conjunctival/anterior epibulbar lymphoma (6/14), tumour-induced pseudohypopyon (4/14) and corneal oedema (3/14). Of 12 eyes with adequate view of iris details, clinically detectable iris thickening or visible mass was noted in all (12/12). There was concomitant involvement of ciliary body (8/14), choroid (5/14) and orbit (2/14). All patients had biopsy of conjunctiva, episclera, iris or ciliary body, confirming the diagnosis of lymphoma. Histopathological or cytopathological evaluation of iris or ciliary body showed high-grade lymphoma in 9/11 eyes in which it was performed. Five patients did not have long-term follow up and, of the other eight patients, three died from complications of systemic lymphoma during follow up ranging from 1 to 44 months., Conclusion: Lymphomatous involvement of the iris should be considered in the differential diagnosis of corticosteroid-resistant uveitis in middle-aged and elderly patients. Iris lymphoma tends to be high grade and usually develops in patients with known aggressive systemic lymphoma., (© 2012 The Authors. Clinical and Experimental Ophthalmology © 2012 Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To determine the clinical and ultrasound biomicroscopy (UBM) features associated with growth in iris melanocytic lesions., Study Design: Retrospective case series analysis., Methods: We included all iris melanocytic lesions that were monitored between January 2005 and November 2009. At the end of the analysis, 44 eyes of 44 patients were included in the final analysis. The clinical features analyzed were: iris color, largest base diameter, radial location of the lesion epicenter, circumferential location of the lesion epicenter, lesion configuration, lesion pigmentation, intrinsic vascularity within the lesion, presence of associated pigmentation, the impact on the pupil, presence of iris atrophy, and lesion-induced localized cataracts. The UBM features included lesion thickness, presence of corneal touch, presence of surface plaque, internal structure, and internal reflectivity. Regression analysis was performed to define the features associated with growth., Results: Twenty-three percent of the lesions showed documented growth. Mean follow-up was 21.4 months (range: 10-48). Clinical features associated with growth were a large basal diameter at baseline (P = .004) and inferior location (P = .004). UBM features associated with growth were: a greater baseline thickness (P = .01), presence of corneal touch (P = .007), an irregular internal structure (P = .0001), and the presence of dots and linear streaks (P < .0001). Clinical features that were not associated with growth were the radial location of the lesion in the iris (P > .999), lesion configuration (P > .999), lesion pigmentation (P > .999), the presence of pigment dispersion (P = .70), iris freckles (P = .15), corectopia (P > .999), ectropion (P > .999), and intrinsic vascularity (P = .70). UBM features not associated with growth were the presence of a surface plaque (P = .07) and the internal reflectivity (P = .77)., Conclusion: Substantial growth in iris melanocytic lesions is associated with original larger basal diameter and inferior lesion location. On UBM growth is associated with greater original thickness, presence of corneal touch, and an irregular internal structure. Presence of these features could modify the frequency of observation of those lesions., (Copyright © 2012 Elsevier Inc. All rights reserved.)

We described a patient with bilateral iris metastases resulted from prostatic cancer. Slit lamp and ultrasonography examination of the both eye demonstrated tumor of the iris, as an amelanotic vascular mass located on the superior temporal quadrant. On open biopsy revealed undifferentiated tissue that stained strongly positive for prostate carcinoma, confirming the diagnosis of metastasis prostate adenocarcinoma. Early diagnostic procedures are essential for the causal therapy of prostate carcinoma as the primary neoplasm.

Objective: To collaborate with multiple centers to identify representative epidemiological, clinical, and pathologic characteristics of melanoma of the iris. This international, multicenter, Internet-assisted study in ophthalmic oncology demonstrates the collaboration among eye cancer specialists to stage and describe the clinical and pathologic characteristics of biopsy-proven melanoma of the iris., Methods: A computer program was created to allow for Internet-assisted multicenter, privacy-protected, online data entry. Eight eye cancer centers in 6 countries performed retrospective chart reviews. Statistical analysis included patient and tumor characteristics, ocular and angle abnormalities, management, histopathology, and outcomes., Results: A total of 131 patients with iris melanoma (mean age, 64 years [range, 20-100 years]) were found to have blue-gray (62.2%), green-hazel (29.1%), or brown (8.7%) irides. Iris melanoma color was brown (65.6%), amelanotic (9.9%), and multicolored (6.9%). A mean of 2.5 clock hours of iris was visibly involved with melanoma, typically centered at the 6-o'clock meridian. Presentations included iritis, glaucoma, hyphema, and sector cataract. High-frequency ultrasonography revealed a largest mean tumor diameter of 4.9 mm, a mean maximum tumor thickness of 1.9 mm, angle blunting (52%), iris root disinsertion (9%), and posterior iris pigment epithelium displacement (9%). Using the American Joint Commission on Cancer-International Union Against Cancer classification, we identified 56% of tumors as T1, 34% of tumors as T2, 2% of tumors as T3, and 1% of tumors as T4. Histopathologic grades were G1-spindle (54%), G2-mixed (28%), G3-epithelioid (5%), and undetermined (13%) cell types. Primary treatment involved radiation (26%) and surgery (64%). Kaplan-Meier analysis found a 10.7% risk of metastatic melanoma at 5 years., Conclusions: Iris melanomas were most likely to be brown and found in the inferior quadrants of patients with light irides. Typically small and unifocal, melanomas are commonly associated with angle blunting and spindle cell histopathology. This multicenter, Internet-based, international study successfully pooled data and extracted information on biopsy-proven melanoma of the iris.

Background: A 73-year-old patient presented in our clinic with multiple pigmented iris nodules in the left eye. Though asymptomatic, there was a reported increase in size and number of the nodules during the past year., Methods: Interventional case report describing a biopsy-proven multifocal iris melanoma treated with a palladium-103 plaque to include the entire anterior chamber within the targeted zone. Biopsy was performed utilizing a 25-gauge aspiration-cutter under viscoelastic. The cornea was protected during radiation therapy with the amniotic membrane buffer technique. Evaluation parameters included visual acuity and clinical assessments aided by slit-lamp photography as well as high-frequency ultrasound imaging., Results: Plaque radiation therapy was well tolerated. Her visual acuity has remained within five letters at 20/25 at 1-year follow-up. Clinical evaluation revealed no evidence of corneal epitheliopathy, iris neovascularization, or visually significant cataract. High-frequency ultrasound imaging revealed a 15% reduction of the tumor's height. Serial abdominal imaging revealed no metastatic uveal melanoma., Conclusions: We present a biopsy-proven iris diffuse melanoma treated with palladium-103 plaque radiation therapy. Though the entire anterior segment was irradiated, there were no significant side-effects. Local tumor control was excellent during the first postoperative year.

Purpose: To demonstrate the correlation of ultrasound biomicroscopy (UBM) features of iris melanoma with histopathology., Design: Retrospective analysis of medical records., Methods: The medical records of patients that underwent surgery for iris melanoma at the Princess Margaret Hospital, University of Toronto, from June 1990 to October 1998 were reviewed. The clinical features, as well as the UBM findings prior to surgical intervention, were evaluated. The anatomic features noted on UBM were correlated with histopathologic features seen in the surgical specimens., Results: Fourteen cases met the inclusion criteria and were included in the final analysis. The ultrasound acoustic characteristics showed a broad spectrum of findings among iris melanomas. Tumor acoustic parameters correlated well with histologic features, including tumor vascularity, surface plaque, extrascleral extension, ciliary body involvement, and integrity of iris pigment epithelium., Conclusions: UBM is a useful imaging technique for the in vivo assessment of primary iris melanoma and can provide detailed imaging of the tumor's interface with the angle structures. The preoperative assessment of these tumors by UBM may aid the surgeon in choosing the most appropriate technique to ensure total removal., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: To report the experience of the Princess Margaret Hospital/University Health Network with the treatment of iris melanoma (IM) with (125)Iodine plaque brachytherapy., Design: Retrospective noncomparative case series., Methods: All cases of IM submitted to (125)Iodine plaque radiotherapy were included. Patients' demographic, clinical, management, and follow-up data were reviewed. Outcome measures included rates of tumor control, eye preservation, systemic metastases, and brachytherapy-related complications., Results: Fourteen IMs were included in the study. All patients had blue/green irises. Mean largest basal dimension and thickness were 7.1 +/- 2.1 mm (range, 4.0 to 11.5 mm) and 2.2 +/- 0.8 mm (range, 1.0 to 3.5 mm), respectively. Ten patients (71%) had seeding and 2 (14%) had glaucoma at presentation. Median follow-up was 26.6 +/- 19.5 months (range, 6 to 72 months). Tumor control was achieved in 100% of the cases and no eye was enucleated because of radiation-induced complications. At last visit, all patients were alive and free of metastasis. Final visual acuity was the same as or better than before treatment in 9 patients (75%). Cataract was the most common complication (8; 75%), followed by persistent glaucoma (2; 17%) and anterior uveitis (1; 8%). No other significant complication was seen during the follow-up period., Conclusions: Plaque radiotherapy is a safe and effective conservative treatment option for IM, although cataract is a common, yet treatable, complication. This treatment scheme circumvents an intraocular procedure and may avoid the dissemination of malignant cells, and provides a margin of safety in the treatment of clinically undetectable disease., (Copyright 2010 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the utility of anterior segment optical coherence tomography (OCT) in the imaging of anterior segment tumors and compare the images to ultrasound biomicroscopy (UBM)., Design: Prospective observational case series., Methods: Eighteen eyes of 18 patients with anterior segment tumors were evaluated at Princess Margaret Hospital. The evaluation included clinical examination, clinical photography, anterior segment OCT, and UBM. Comparison of images obtained by both methods was done., Results: Anterior segment OCT imaged small hypopigmented tumors with complete penetration. Cysts were incompletely imaged behind the iris pigment epithelium. Highly pigmented tumors, large tumors, and ciliary body tumors were incompletely penetrated. Even without complete penetration it was possible to differentiate cystic lesions from solid lesions. UBM penetrated all tumors completely., Conclusions: Anterior segment OCT can penetrate small hypopigmented tumors and supply some information on internal characteristics of other tumors. UBM is preferable for clinical anterior tumor assessment and follow-up because of its superior ability to penetrate large tumors, highly pigmented tumors, and ciliary body tumors.

The clinical and ultrasound biomicroscopic features of two cases of iris melanocytoma are evaluated. On ultrasound biomicroscopy examination, iris melanocytoma appears as a highly reflective nodular mass with a smooth or irregular surface and sharp and well-defined edges. Ultrasound biomicroscopy also allows clear visualization and measurement of the tumors. These findings were compared with clinical and histopathologic findings.

A 50-year-old white man with oculo(dermal) melanocytosis and longstanding iris nevus was found to have growth of the iris mass. Excision and histopathologic examination revealed a mixed cell type malignant melanoma. Benign nevus cells were present at the periphery of the tumor surrounding the entire melanoma. White patients with oculo(dermal) melanocytosis have a predisposition to uveal melanoma, which is usually choroidal in origin. Literature review showed only three confirmed cases of iris melanoma in this setting. Two additional cases initially published as spindle A melanoma have been reclassified as iris nevi based on the modified Callender classification of uveal melanomas. It is recommended that patients with oculo(dermal) melanocytosis be followed for the occurrence of uveal melanoma.

Background: In the presence of pigmented iris lesions evocative of malignant melanoma and implying oncological treatment, a foregoing biopsy to exclude a benign lesion may seem a reasonable approach. After examining patient files, the utility of such a diagnostic approach was explored., Material and Methods: Retrospective, consecutive histopathologic case series of 10 pigmented iris tumor specimens excised since 1993. Histopathologic diagnosis was compared with final diagnosis and outcome in the patient's medical chart., Results: Five biopsies had only nevus cells, whereas ulterior clinical data or histopathologic examinations were compatible with the diagnosis of malignant melanoma. One biopsy contained insufficient sample tissue. Four biopsies confirmed clinical suspicion of iris melanoma., Conclusion: In the current case series, 6 out of 10 biopsies provided a falsely reassuring negative or an inconclusive result. Modern management techniques such as ultrasound biomicroscopy and proton therapy of the whole anterior segment have equally diminished indications for a biopsy. In cases clinically evocative of iris melanoma, a biopsy has only a relative value., (Copyright 2008 S. Karger AG, Basel.)

Objective: To evaluate size, characteristics, and regression of iris and iridociliary melanomas on high-frequency ultrasound images before and after plaque brachytherapy., Methods: Retrospective review of high-frequency ultrasound characteristics of 24 consecutive iris and iridociliary melanomas before and after radiation therapy., Results: The median tumor thickness before radiation therapy was 2.3 mm (range, 1.4-4.3 mm). Nineteen iris melanomas (79%) involved the ciliary body, 18 (75%) involved the iris pigment epithelium, 11 (46%) were club shaped, and 4 (17%) caused disinsertion of the iris root. At a median follow-up of 30 months after plaque brachytherapy, the mean tumor thickness had diminished to 1.2 mm (median, 1.2 mm; range, 0.9-1.9 mm). While all tumors exhibited a reduction in thickness, no tumors showed additional regression after 30 months past treatment. Fourteen tumors (58%) were noted to have increases in internal reflectivity. There was 1 failure of local control (at 6 years), successfully treated by a second application of plaque brachytherapy., Conclusion: High-frequency ultrasonography revealed unique tumor characteristics, quantified tumor size, and demonstrated tumor response to radiation therapy.

Purpose: To document the relationship of iris cavernous hemangiomas with multiorgan hemangiomas in a child., Methods: A 7-year-old girl with kidney hemangiomas, brain hemangiomas, and skin hemangiomas developed blurred vision and darkening of the right iris. She had undergone 6 previous brain surgeries and multiple revisions of a ventriculoperitoneal shunt for hydrocephalus beginning at age 3 months., Results: Examination revealed visual acuity of 20/30 in the right eye and 20/20 in the left eye. There was a reddish-purple lobulated cystic mass along the superior iris pupillary margin measuring 2 mm in greatest diameter confirmed by ultrasound biomicroscopy. The remainder of the ocular examination was unremarkable. The diagnosis was iris cavernous hemangioma in a patient with multiorgan cavernous hemangiomatosis. By 1-year follow-up, the iris hemangioma deflated, leaving a fibrotic, involuted scar., Conclusions: Iris cavernous hemangioma can be associated with multiorgan hemangiomas that can be life threatening.

We report three cases of renal cell carcinoma metastatic to the eye and orbit and review the relevant literature. The case reports of a 67-year-old man, a 58-year-old man, and a 23-year-old woman with metastatic renal cell carcinoma are described. The iris mass occurred in a 67-year-old man, a known case of renal cell carcinoma. Whereas the orbital metastasis in the 58-year-old man was the initial presenting sign in a hitherto undiagnosed patient, the orbital metastasis in the 23-year-old female patient was detected following nephrectomy for renal cell carcinoma. Renal cell carcinoma metastasizing to the eye and orbit are very rare, with only 68 cases reported previously. In patients presenting with atypical orbital or ocular masses, the possibility of renal cell carcinoma metastasis should be considered, especially if there is a history of previous renal disorder. Incisional biopsy with histopathological evaluation may be an important means to diagnose this condition and facilitate appropriate therapy.

Purpose: To report the ultrasound biomicroscopy (UBM) findings of anterior segment tumors and simulating conditions., Methods: Thirty-five patients underwent UBM. Of those, 16 had histopathologically or cytopathologically diagnosed tumors, and 19 had clinically diagnosed lesions., Results: The study material comprised 13 iris pigment epithelial (IPE) cysts, 7 ciliary body melanomas, 4 iris melanomas, 4 iris nevi, 3 intraocular invasions of conjunctival squamous cell carcinoma, 2 ring melanomas of the anterior chamber angle, 1 medulloepithelioma and 1 pars plana cyst. On UBM, all IPE cysts presented as cystic lesions with a thin cyst wall and no solid components. All ciliary body melanomas showed low to medium reflectivity, with cavitation in one case and extraocular extension in another. Iris melanomas presented as anterior (stromal) iris lesions with medium to high internal reflectivity. There was irregularity and convex bowing of the posterior iris plane in iris melanomas, a feature not seen in iris nevi. Intraocular invasion of conjunctival squamous cell carcinoma was evidenced as areas of medium to high reflectivity in the ciliary body and iris, loss of the acute angle shape and highly reflective spots in the anterior chamber., Conclusions: UBM was particularly useful in the diagnosis of IPE cysts, in the visualization of small ciliary body melanomas, in the differentiation of iris melanomas from iris nevi and in the demonstration of intraocular invasion from conjunctival squamous cell carcinoma., ((c) 2007 S. Karger AG, Basel.)

Background/aim: Ultrasound biomicroscopy (UBM) is an important tool for assessing anterior segment pathology. This study sought to evaluate UBM in the management of anterior segment tumours., Methods: Retrospective analysis of medical records of consecutive patients referred to the ocular oncology unit, University of California San Francisco (UCSF), for suspected anterior segment tumours from 1999 to 2004., Results: 132 eyes from 130 patients were evaluated, including 55 uveal melanomas (UM), 21 iris naevi, 30 iris cysts, and 26 remaining lesions. Of the melanomas, 45 were also evaluated with conventional A/B-scan. There was 29% correspondence between the anatomical structures invaded by melanoma as identified by B-scan v disease extent defined by UBM. Ciliary body and peripheral iris involvement by melanomas was significantly more frequently observed by UBM than B-scan. Seven of 30 benign cysts were diagnosed as cystic before UBM evaluation. In three cases, neuroepithelial cysts were associated with intercurrent pathology including iris naevus (n = 2) and ciliary body melanoma (n = 1). Two ciliary body melanomas showed cavitation, including one patient with a pseudocyst. Histopathological correlation was possible in six cases., Conclusion: UBM is an indispensable tool for the management of anterior segment tumours. This study demonstrates the superiority of UBM v conventional B-scan for the precise localisation of uveal melanoma, especially involving the ciliary body and peripheral iris.

Background and Objective: To determine ultrasound biomicroscopy (UBM) features of iris and ciliary body melanomas before and after brachytherapy., Patients and Methods: Four uveal anterior melanoma cases undergoing brachytherapy were retrospectively studied. All cases were examined by UBM prior to treatment and repeatedly after treatment., Results: Before brachytherapy, UBM examination showed a solid mass in the iris, ciliary body, or both in all four cases and allowed its characterization, sizing, and positioning. Two cases had a pigmented scleral lesion corresponding to the tumor location, but UBM did not detect any scleral infiltration at those sites. After brachytherapy, all lesions showed progressive decrease in size and progressive attenuation of their limits. Internal reflectivity was variable. Complications related to brachytherapy were demonstrated, including cataract, peripheral anterior synechiae at tumor location, and secondary scleral thinning., Conclusion: UBM played an important role as a complementary diagnostic method for anterior uveal melanomas, particularly ciliary body melanomas, but also allowed therapeutic planning (brachytherapy or surgery) and follow-up after treatment.

The ultrasound biomicroscope (UBM) has a major role in detecting and following different types of intra-ocular masses in the anterior part of the eye. This equipment may provide the possibility to detect the inner structure of the masses, to differentiate between cysts and solid tumours, and to follow their progression and spreading into the surrounding tissue. In the last six years 30 patients with iris and ciliary body tumour were found in our laboratory. The examination were performed by Zeiss Humphrey Ultrasound Biomicroscope, Model 840, 50 MHz probe. We followed closely 22 patients. Surgical removal and histological examination were performed in 3 cases. Melanocytoma, retinoblastoma and ring melanoma were revealed. Although the symptoms of the anterior uveal tumours are uncommon, and these tumours show very slow progression, early detection and regular follow-up is needed. In the case of tumour progression the UBM has an important role in planning and timing of surgery or radiotherapy, which can have a favourable effect on the outcome of the disease.

Purpose: Posterior pole imaging with OCT3 provides macular scans with a very high resolution. Use of OCT1 has already been described for anterior segment imaging. This work presents the advantages of OCT3 in anterior segment imaging compared to UBM (50 MHz) and 20-MHz ultrasound imaging with long focal distance (patent pending PCT/FR98/02788)., Material: and methods: We used an OCT3 unit dedicated to the posterior pole. By defocusing the laser beam we obtained high-resolution scans of the cornea, angle, lens and IOLs. Fifty-seven eyes (35 patients) were imaged by OCT3 and high-resolution ultrasound in cases of corneal dystrophy, corneal transplant, LASIK, narrow angle, iris bowing, etc., Results: In all cases OCT3 provided high-resolution information with a limit of penetration at the posterior part of the iris. This limitation was not observed with ultrasound imaging. Nevertheless, OCT3 is a useful tool for corneal imaging in cases of opacities limiting biomicroscope imaging. OCT3 corneal scans at high resolution make it possible to identify the corneal interface in lamellar transplants or LASIK. With a narrow angle, a dynamic test of angle closure can be done. With iris bowing, OCT3 can differentiate iris cysts and iris tumors. Anterior chamber IOLs can be imaged with their relationships to anterior segment structures., Conclusion: OCT3 provides non-contact anterior segment imaging with high resolution but with a limit of penetration given by posterior part of the iris.

Background: Cutaneous melanoma is a primary tumour that rarely produces intraocular metastases. We report on a 47-year-old woman who was referred to our outpatient department because of anisocoria., Methods: A cutaneous melanoma of the upper left arm had been excised 21 months ago with subsequent polychemotherapy for lymph node involvement. Visual acuity and intraocular tension were normal. The left iris showed a brownish, parapupillary, prominent nodule. In both eyes, multiple circumscribed whitish choroidal sub-pigment epithelial tumours were observed., Results: General metastasis to lymph nodes, pleura, lungs, mucosa and skin was diagnosed. The patient died 2 months later., Conclusion: Metastases of cutaneous melanoma to the iris are extremely rare. The ophthalmologist may be helpful in monitoring treatment by quantitative documentation at follow-up.

Background: Metastatic cancer of the iris is rare. We report a case of breast carcinoma which metastasized to the iris and a review of the literature., Method: A 60-year-old woman with a history of breast carcinoma presented with lesions of the iris 5 years after diagnosis and tumor excision. In addition to slit-lamp examination including gonioscopy, ultrasound biomicroscopy, positron-emission tomography and fluorescein angiography of the iris were used for diagnosis. Furthermore, the literature was searched using the medline database., Results: The iris metastasis exhibited multiple whitish to pink nodules on the inferior half of the iris and infiltration of the chamber angle. The main tumor mass was prominent and highly vascularized. Best corrected visual acuity was 20/40 and the intraocular pressure was 7 mmHg. Ultrasound biomicroscopy showed lobular masses characterized by mid to low reflectivity and there was no distinct border to the surrounding tissue. Fluorescein angiography of the iris showed tumor vessels with dye leakage. The positron-emission tomography displayed metastatic lesions to the liver, lung, bones and lymph nodules in addition to the lesion of the iris. The review of the literature revealed that breast carcinoma leads to choroidal metastases in 5% of cases and iris metastases are even rarer. In twothirds of these cases the lesions form unilateral whitish to red clumps at the horizontal meridian and in the inferior quadrants of the iris. The preferred treatment is chemotherapy and/or radiation therapy., Conclusions: Breast carcinoma is rarely associated with iris metastasis. Positron-emission tomography is a sensitive diagnostic tool to identify metastatic lesions and is a useful method for planning therapeutic approaches. Radiation therapy, argon laser treatment and block excision are therapeutic options with an isolated metastasis of the iris.

Purpose: To report on plaque radiation therapy for malignant melanomas involving the iris and ciliary body., Methods: Twenty-two eyes (22 patients) with anterior uveal melanomas were treated with (103)Pd ophthalmic plaque radiation therapy. Transillumination and ultrasonography were used to evaluate ciliary body involvement and posterior iris extension. Plaques were placed onto the cornea to treat the anterior tumor margins. The targeted-zone included the tumor and a 2 to 3 mm tumor-free margin. After plaque removal, patients were examined at 1 day, 7 days, 4 weeks, and then every 3 to 6 months thereafter. Systemic evaluations for possible metastatic disease were performed every 6 months., Results: After plaque radiation therapy, the melanomas decreased in thickness (mean 47%) in all 22 eyes, and no secondary enucleation was performed. One patient died of metastatic melanoma 5 years after radiation therapy. Despite anterior plaque placement that covered portions of the cornea, no epiphora, eyelash loss, or visually significant corneal opacities were noted. Whereas 15 of 21 phakic eyes (71%) developed secondary cataract, no eyes developed ischemic or neovascular radiation maculopathy. Four eyes were noted to have glaucoma before treatment, and two developed it after irradiation. Twenty of 22 eyes (91%) were within 2 lines of their pretreatment visual acuity. After radiation, the mean +/- SD follow-up was 56 +/- 34.4 months (range, 9 to 117 months)., Conclusions: Plaque radiotherapy of melanomas involving the iris and ciliary body resulted in excellent local control with preservation of vision. Although there was high incidence of secondary cataracts, (103)Pd plaque radiation therapy resulted in no visually significant corneal opacity or radiation retinopathy.

The aim of this study was to evaluate high-frequency ultrasound imaging (HFUI) as an aid in localizing anterior margins of tumours of the eye for proton therapy. Proton irradiation of ocular melanoma requires an accurate assessment of all tumour margins. The tumour is marked surgically by suturing to the sclera four or five tantalum rings on the borders of the tumour defined by transillumination. In order to evaluate the clinical usefulness of high-frequency ultrasound imaging, four and five rings were surgically placed in a patient with an iris/ciliary body melanoma and in a patient with ciliochoroidal melanoma using transillumination to localize the tumour margins. Subsequently margins were verified by HFUI. In the first patient, the distances between the rings and the limbus were measured using calipers during surgery and were compared with HFUI measurements and measurements from planning software. The distances were comparable within 0.5 mm. In the second patient the treatment was planned in two different ways using EYEPLAN software. In the first scenario the shape of the tumour and its relation to the rings were obtained from the surgeon's mapping, the fundus drawing using a transilluminating point light source, and the HFUI. In the second scenario, the shape of the tumour was deduced from the ring positions only. It was observed that the maximum difference between the tumour edge as seen on high-frequency ultrasound images and the rings was 2.6 mm. The tumour volume was underestimated by 39% when tumour shape was obtained from ring positions only. During the past year we have utilized HFUI in 18 patients having tumours involving the anterior segment of the eye, among which four were treated with proton therapy. In conclusion, we believe that high-frequency ultrasound imaging provides additional information with respect to the location of tumour margins in ciliary body and anterior uveal melanoma. Occult extension of the tumour within the ciliary body or posterior iris may not be appreciated by transillumination alone.