Influence of Post-radiation Ocular Surface Disorder on Ocular Surgery: A Case Report and Review of the Literature.

BACKGROUND: Ocular surface disorder after ocular radiation therapy, even though commonly reported, is often overlooked. Any delay in diagnosis may lead to complications that threaten vision. The presented case highlights the clinical outcome of a severe post-radiation disorder of the ocular surface, the importance of intensive therapy, and the limitations of further surgical interventions. CASE PRESENTATION: A 34-year-old woman was referred for a second opinion due to a years-long history of pain and redness in her right eye (OD) after proton beam therapy for recurrent iris melanoma. The patient then developed post-radiation retinopathy with macula edema, secondary glaucoma, cataract, as well as a severe ocular surface disorder with corneal decompensation and band keratopathy. Several surgical treatments have been attempted, including phacoemulsification with IOL implantation and trabeculectomy with mitomycin C. Due to refractory glaucoma, Baerveldt glaucoma drainage was then necessary. Given the worsening clinical presentation of post-radiation ocular surface disorder with progressing band keratopathy, the possibility of penetrating keratoplasty (PKP) was discussed. CONCLUSION: The continuous worsening of clinical symptoms of the disorder of the ocular surface after proton beam radiotherapy can be the result of a post-radiation syndrome. Gradual expansion of ischemia, vasculitis, and inflammatory mediators compresses the retinal tissue, leading to recurrent macular edema as well as to secondary glaucoma and corneal decompensation. Band keratopathy is occasionally noted and seems to result from severe post-radiation disorder of the ocular surface. However, PKP would typically be indicated in cases of corneal perforation, uncontrolled infectious keratitis, or for improving vision in the presence of corneal opacification, none of which applied to our patient. Furthermore, post-radiation keratopathy implies compromised corneal stromal lymphogenesis and angiogenesis, both of which are now considered essential conditions for allograft rejection. Moreover, a previously performed Baerveldt glaucoma drainage surgery can affect the survival rate of the endothelial cells of the recipient cornea. Therefore, a penetrating or endothelial keratoplasty should be viewed as a high-risk procedure. In this instance, the rigorous treatment of the severe ocular surface disorder was crucial. We managed our patient's complex situation by following the latest guidelines set by the Tear Film & Ocular Surface Society and aimed to alleviate the symptoms as effectively as possible. In conclusion, careful decision-making regarding surgical treatment options should be considered, taking into account the complexities and potential risks involved.

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.

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.

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.

Clinical outcomes of Modified Collaborative Ocular Melanoma Study IRIS plaques for treatment of iris, iridociliary, and ciliary body melanoma.

BACKGROUND/OBJECTIVES: To report clinical outcomes of modified Collaborative Ocular Melanoma Study IRIS (COMS IRIS) plaques for treatment of iris, iridociliary, and ciliary body melanoma. SUBJECTS/METHODS: Retrospective, single-centre cohort study of iris melanoma treated with COMS IRIS plaque radiotherapy from July 26, 2010 to October 15, 2018. Medical records were reviewed for demographics, tumour features, treatment parameters, and clinical outcomes. RESULTS: There were 22 cases, diagnosed at mean age of 59 years (median 65, range 21-85 years) with female sex in 14 (64%). Presenting tumour features included Snellen visual acuity (VA) ≥ 20/40 in 18 (82%) cases, mean largest tumour basal diameter 4.7 mm (median 3.9, range 2.3-13.8 mm) and thickness 1.7 mm (median 1.6 mm, range 0.8-3.9 mm), iris stromal seeding in 3 (14%) cases, angle seeding in 16 (73%), and ciliary body involvement in 13 (59%). After mean follow-up of 51 months (median 44, range 4-113 months), Snellen VA was ≥20/40 in 14 (64%) cases, with local tumour recurrence in 2 (9%), and enucleation in 2 (9%). The 3-year Kaplan-Meier estimated risk of local tumour recurrence was 7%. The most common radiation side effects were cataract in 17 (77%) patients and dry eye in 5 (23%). Systemic metastasis occurred in no cases, and 1 (5%) non-melanoma-related death due to natural causes was observed at last follow-up. CONCLUSIONS: COMS IRIS plaques are effective for treatment of iris, iridociliary, and ciliary body melanoma with modest VA outcomes and low frequency of local tumour recurrence, enucleation, radiation side effects, and systemic metastasis.

Iris melanocytoma and secondary glaucoma.

Iris melanocytoma (IM) is a rare variant of iris nevus with distinctive clinical and histopathological features. A 66-year-old woman, with a history of right eye pigmented iris nevus, presented to us with a recent onset of visual acuity decrease in that eye. She had a melanocytic iris lesion with iridocorneal angle invasion, peripheral corneal adhesion, pupil corectopia, sectorial cataract and high intraocular pressure. Ultrasound biomicroscopy did not exclude malignant transformation, so excisional biopsy was performed revealing the presence of IM without signs of atypia. Subsequently, the patient underwent cataract surgery combined with iridoplasty and later an ab externo trabeculectomy. Most cases of IM remain stable and require no intervention, but in cases of unusual clinical course, with rapid growth or secondary glaucoma, surgical treatment is indicated as a diagnostic and therapeutic measure. This case report highlights the importance of a timely and multidisciplinary ophthalmological approach for a better visual outcome.

Case Report: Tapioca Iris Melanoma: An Uncommon Presentation of an Uncommon Tumor.

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.

Surgical excision of iridociliary tumors using a postero-anterior cyclo-iridectomy and thermocautery in two dogs.

OBJECTIVE: To report the surgical excision of an iridociliary adenoma and iridal melanocytoma using a postero-anterior cyclo-iridectomy in two dogs. PROCEDURE: A 7 year old neutered male English springer spaniel (case 1) and a 7 year old neutered male Labrador mix (case 2) were presented for evaluation of an intrairidal mass OS. RESULTS: Complete ophthalmic examination revealed a large, dorsonasal, well-demarcated, intrairidal mass OS. A tan to pink intrairidal mass extending into the iridocorneal angle (case 1) and a pigmented intrairidal mass (case 2) were present. B-mode ultrasonography showed a focal, soft tissue, homogenous mass within the uvea adjacent to and contacting the lens. Neither pars plana involvement nor vitreal extension was present. A postero-anterior cyclo-iridectomy was performed through a polyhedral scleral flap. Thermocautery was used to complete the cyclo-iridectomy (case 1) and partial iridectomy (case 2) to excise the mass en bloc. Histopathology revealed a completely excised iridociliary adenoma (case 1) and iris melanocytoma (case 2). The surgery sites healed without complication. Mild uveitis (cases 1 and 2), scant vitreal hemorrhage (case 1), and mild hyphema (case 2) were present three days postoperatively but had resolved ten days postoperatively. The patients remain visual twenty-two months (case 1) and seven months (case 2) postoperatively with a normal intraocular examination other than an iridal defect and mild dorsonasal lens capsular opacities. CONCLUSIONS: The surgical approach described in these cases is utilized in physician-based medicine. This approach and the use of thermocautery provide a viable surgical option for excision of large iridociliary tumors in dogs.

Intravitreal air bubble during exoresection of an iris-ciliary body melanoma.

In this manuscript, as members of a National Reference Unit in Ocular Oncology, we present succinctly our preferred surgical technique for iris-ciliary body melanomas. We attach a video describing the steps we usually follow [Video Clip 1], in which an impressive image of an intravitreal air bubble through the hyaloid membrane can be observed [Fig. 1].

Neuroblastoma of the Iris in Children.

Neuroblastoma of the iris is an extremely rare clinical entity. An otherwise healthy 2-month-old male infant presented to the oncology clinic with a nodular whitish iris lesion in his right eye. The excisional tumor biopsy was consistent with a pathological diagnosis of neuroblastoma with differentiation and negative MYCN gene mutation. Further systemic evaluation revealed a right adrenal mass with no metastatic lesion. The biopsy of the adrenal lesion was also consistent with neuroblastoma. After four courses of chemotherapy, the adrenal mass was completely resected. The patient underwent two additional courses of postoperative chemotherapy and continued retinoic acid treatment. The patient is under regular follow-up with no evidence of recurrence 36 months after the initial diagnosis. This is the first case report to present a histopathological verification of neuroblastoma of the iris. The authors suggest that neonates and infants who are diagnosed as having neuroblastoma undergo an ophthalmologic examination after the initial diagnosis to investigate the true incidence of small iris lesions in neuroblastoma that may have been unrecognized. Neuroblastoma should be included in the differential diagnosis of amelanotic iris lesions in infants and young children. [J Pediatr Ophthalmol Strabismus. 2019;56:e12-e16.].

Amelanotic iris melanoma in a 7 year-old hispanic boy with subclinical globe perforation. / Melanoma amelanótico del iris en un niño hispano de 7 años con perforación del globo subclínica.

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.

Iris Melanoma in a Child Simulating Juvenile Xanthogranuloma.

An 8-year-old girl was referred for an amelanotic iris tumor believed to have enlarged slightly over 3 months, suspicious for juvenile xanthogranuloma (JXG). The affected right eye had a lightly pigmented, vascular iris mass measuring 6 mm in basal dimension and 2 mm in thickness. There were no feeder vessels, seeding, inflammatory cells, or cutaneous abnormalities. Diagnostic fine needle aspiration biopsy (FNAB) was performed, revealing spindle B melanoma cells that were immunoreactive for melanocytic markers HMB45 and Melan-A. Complete tumor resection by basal sector iridectomy was performed. Histopathology confirmed spindle B melanoma. At 14-years follow up, there has been no recurrence or metastasis and visual acuity remains 20/25. Iris melanoma can develop in children and clinically resemble nodular JXG.

Genetic Background of Iris Melanomas and Iris Melanocytic Tumors of Uncertain Malignant Potential.

PURPOSE: Uveal melanoma (UM) is the most common primary intraocular malignancy in adults. Iris melanoma comprises 4% to 10% of all UMs and has a lower mortality rate. The genetic changes in iris melanoma are not as well characterized as ciliary body or choroidal melanoma. The aim of this study was to gain more insight into the genetic background of iris melanoma and iris nevi. DESIGN: Multicenter, retrospective case series. PARTICIPANTS: Patients diagnosed with iris melanoma or iris nevi who underwent surgical intervention as primary or secondary treatment. METHODS: Next-generation sequencing of GNAQ, GNA11, EIF1AX, SF3B1, BAP1, NRAS, BRAF, PTEN, c-Kit, TP53, and TERT was performed on 30 iris melanomas and 7 iris nevi. Copy number status was detected using single nucleotide polymorphisms (SNPs) included in the next-generation sequencing (NGS) panel, SNP array, or fluorescent in situ hybridization. BAP1 immunohistochemistry was performed on all samples. MAIN OUTCOME MEASURES: Mutation and copy number status were analyzed. Results of BAP1 immunohistochemistry were used for survival analysis. RESULTS: In 26 of the 30 iris melanoma and all iris nevi, at least 1 mutation was identified. Multiple mutations were detected in 23 iris melanoma and 5 nevi, as well as mutations in GNAQ and GNA11. Furthermore, 13 of 30 BAP1, 5 of 30 EIF1AX, and 2 of 30 SF3B1 mutations were identified in iris melanoma. No correlation between BAP1 status and disease-free survival was found. The iris nevi showed 1 EIF1AX and 3 BAP1 mutations. Two of the nevi, with a BAP1 mutation, were histologically borderline malignant. Mutations in NRAS, BRAF, PTEN, c-KIT, and TP53 were detected in 6 iris melanomas and 4 iris nevi. CONCLUSIONS: Mutations that are often found in uveal and cutaneous melanoma were identified in this cohort of iris melanomas and iris nevi. Therefore, iris melanomas harbor a molecular profile comparable to both choroidal melanoma and cutaneous melanoma. These findings may offer adjuvant targeted therapies for iris melanoma. There was no prognostic significance of BAP1 expression as seen in choroidal melanoma. Consequently, iris melanoma is a distinct molecular subgroup of UM. Histologic borderline malignant iris nevi can harbor BAP1 mutations and may be designated iris melanocytic tumors of uncertain malignant potential.

Four cases of iridociliary tumors in domestic rabbits (Oryctolagus Cuniculus).

Spontaneously occurring ocular neoplasia is rarely reported in rabbits. This case series presents four cases of rabbits diagnosed with iridociliary tumors, which have not been previously reported in this species. Major pathological findings include epithelial tumors affecting the anterior uvea with variable pigmentation and basement membrane formation. Follow-up information was only available for two cases, but neither showed evidence of metastasis, suggesting that the prognosis for these tumors in rabbits, as in other species, may be very good.

Orbital recurrence of iris melanoma 21 years after enucleation.

We present the case of a 56-year-old man who developed a neoplasm of epithelioid histology in his anophthalmic left orbit 21 years after he underwent enucleation for a spindle cell iris melanoma. The recurrent tumour was managed by orbital exenteration. Neither further recurrence nor metastasis was diagnosed over a 5-year follow-up period. This case, along with five other similar cases in the literature,1-3 emphasises the importance of long-term follow-up after treatment of iris melanoma.

Symptomatic primary iris cysts treated with frequency-doubled Nd: YAG laser photocoagulation / Cistos irianos primários sintomáticos tratados com fotocoagulação a laser de Nd: YAG de dupla frequência

Abstract We report the case of a 31-year-old woman who presented with a nine-month history of blurred vision in her left eye. Slit-lamp examination and optical coherence tomography showed four cystic masses hanging at the pupillary margin of the left eye. Neodymium:Yttrium-Aluminum-Garnet (Nd:YAG) laser photocoagulation was used to rupture the wall (cystotomy), drain the cyst content and shrink the remnants of the pigment epithelium, using laser parameters at the lowest effective levels. The patient's best corrected visual acuity improved significantly after treatment. Despite the increase of pigment at anterior chamber angle, no complication was observed during 6 months of follow up. This is the first report to describe frequency-doubled Nd:YAG laser (532 nm) photocoagulation as a therapeutic option for patients with symptomatic primary IPE cysts at pupillary margin.

Resumo Relatamos o caso de uma mulher de 31 anos com queixa de visão turva no olho esquerdo. Exame em lâmpada de fenda e tomografia de coerência óptica mostraram quatro lesões císticas nas margens da pupila esquerda. O laser do tipo Neodymium:Yttrium-Aluminum-Garnet (Nd:YAG) de dupla-frequência foi o tratamento de escolha para romper a parede (cistotomia), drenar o conteúdo do cisto e encolher os remanescentes do epitélio de pigmentar utilizando os menores parâmetros efetivos. Após o tratamento do olho comprometido, sua acuidade visual melhorou consideravelmente. Apesar do aumento da pigmentação no ângulo da câmara anterior, nenhuma complicação foi observada durante 6 meses de acompanhamento. Este é o primeiro relato que descreve o laser de Nd:YAG de dupla frequência (532 nm) como uma opção terapêutica para pacientes com cistos irianos primários do epitélio pigmentar sintomáticos na margem pupilar.

[Surgical Methods in the Treatment of Uveal Melanoma]. / Chirurgische Methoden in der Behandlung des Aderhautmelanoms.

Significant advancements have been made in the last decades in the treatment of uveal melanoma. Radiation therapy can achieve, depending on the method, local tumour control in up to 98% of cases. Surgical modalities have been implemented in the ocular therapy for uveal melanoma either to treat/prevent radiation complications, such as so-called toxic tumour syndrome, or to primarily treat the tumour with or without adjunctive radiotherapy. These include endoresection and transscleral resection for melanomas of the choroid/ciliary body or irido-trabeculo-cyclectomy for melanomas of the iris-ciliary body. Furthermore, tumour biopsy is playing a progressively more important role in the diagnosis, treatment and follow-up planning of melanoma cases. This paper focuses on the background, the surgical technique as well as the results and complications of tumour excisional and biopsy surgery.

Late Solitary Extraocular Recurrence From Previously Resected Iris Melanoma.

PURPOSE: To report on cases of late extraocular relapse of previously resected iris melanoma, without concurrent intraocular recurrence. DESIGN: Retrospective case series. METHODS: A retrospective chart review of 4 patients diagnosed with late subconjunctival relapse of previously resected iris melanoma. RESULTS: Three female patients and 1 male patient underwent iris tumor resection and presented to our service with suspicious conjunctival lesions at a median of 22 years later (mean: 21 years). None showed intraocular relapse. Treatment of the conjunctival tumors included excisional biopsy (n = 4), followed by cryotherapy (n = 3) and/or brachytherapy (n = 3). In all cases, histopathology confirmed malignant melanoma, with no intraepithelial component or associated melanosis. Genetic sequencing (n = 3) showed wild-type BRAF and NRAS in all. GNA11 mutation was found in 1 case. On array-based comparative genomic hybridization (n = 3), gain of 6p was found in 2 cases and gain of 8 in 2. Overall, findings were strongly suggestive of a diagnosis of late extraocular relapse from previously resected iris melanoma. In a median of 2.5 years (mean: 7.7 years) from the subconjunctival relapse, no further episodes of intraocular/extraocular recurrence were recorded, and all patients were free from distant metastasis. CONCLUSIONS: Patients undergoing iris melanoma resection are at risk of developing late solitary extraocular relapse even more than 30 years after surgery. In the absence of an intraocular component, diagnosis may be challenging, as tumors mimic a primary conjunctival lesion. Management by excisional biopsy followed by adjuvant therapy was successful, and histopathology and genetic analysis supported a diagnosis of extraocular uveal tumor spread rather than a primary conjunctival tumor.