Uveal melanoma in children and teenagers

To review the features and prognosis of uveal melanoma in children. Methods: Retrospective case series. Of 122 children with uveal melanoma, there were 53 [43%] male and 69 [57%] female patients. In this group, the mean age at presentation was 15 years [median 16 years, range 3-20 years]. Age at presentation was 0 to 5 years in 4 [3%], 5.1 to 10 years in 14 [11%], 10.1 to 15 years in 43 [35%], and 15.1 to

American joint committee on cancer staging of uveal melanoma

In January 2010, the American Joint Committee on Cancer [AJCC] updated its staging criteria for uveal melanoma. Here, we discuss the staging classifications and evaluate an interesting case to demonstrate. The development of a uniform means of staging cancer, such as defined by the AJCC, is critical for information sharing among the research community

Transient increased exudation after photodynamic therapy of intraocular tumors

To report transient increased exudation after photodynamic therapy [PDT] of three different intraocular tumors [retinal hemangioblastoma, retinal astrocytoma, amelanotic choroidal melanoma]. PDT with verteporfin [6 mg/m[2] body surface area] was delivered at a dose of 50 J/cm[2] and intensity of 600 mW/cm[2] over 83 s. All patients experienced decreased vision within a few days following PDT. Optical coherence tomography showed development of subfoveal fluid in all cases and noncystoid intraretinal edema in the eye with juxtapapillary retinal hemangioblastoma. There was complete absorption of retinal/subretinal fluid with improvement of visual acuity to 20/20 in all cases between 3 weeks to 4 months after PDT

Review of cystic and solid tumors of the iris

Iris tumors are broadly classified into cystic or solid lesions. The cystic lesions arise from iris pigment epithelium [1PE] or iris stroma. IPE cysts classically remain stable without need for intervention. Iris stromal cyst, especially those in newborns, usually requires therapy of aspiration, possibly with alcohol-induced sclerosis, or surgical resection. The solid tumors included melanocytic and nonmelanocytic lesions. The melanocytic iris tumors include freckle, nevus [including melanocytoma], Lisch nodule, and melanoma. Information from a tertiary referral center revealed that transformation of suspicious iris nevus to melanoma occurred in 4% by 10 years and 11% by 20 years. Risk factors for transformation of iris nevus to melanoma can be remembered using the ABCDEF guide as follows: A=age young [<40 years], B=blood [hyphema] in anterior chamber, C=clock hour of mass inferiorly D=diffuse configuration, E=ectropion, F= eathery margins. The most powerful factors are diffuse growth pattern and hyphema. Tumor seeding into the anterior chamber angle and onto the iris stroma are also important. The nonmelanocytic iris tumors are relatively uncommon and included categories of choristomatous, vascular, fibrous, neural, myogenic, epithelial, xanthomatous, metastatic, lymphoid, leukemic, secondary, and non-neoplastic simulators. Overall, the most common diagnoses in a clinical series include nevus, IPE cyst, and melanoma. In summary, iris tumors comprise a wide spectrum including mostly iris nevus, IPE cyst, and iris melanoma. Risk factors estimating transformation of iris nevus to melanoma can be remembered by the ABCDEF guide

Multicavitary ciliary body melanoma presenting as a cyst

Cyst-like cavities in uveal melanoma occur rarely and can simulate a benign intraocular cystic lesion resulting in delayed diagnosis and inappropriate management. Herein, we describe a 66-year-old Caucasian female who presented with a "cystic" ciliary body mass in the right eye oculus dexter [OD]. Slit lamp examination OD showed anterior bulging of the iris temporally from an underlying pigmented ciliary body mass and transillumination disclosed slight shadow from the tumor. Ultrasound biomicroscopy [UBM] revealed multiple cyst-like cavities within a tumor, lined by "thick walls" of at least 200 microm and occupying 80% of the tumor volume. A clinical diagnosis of multi-cavitary ciliary body melanoma was suspected and partial lamellar sclero iridocyclectomy was performed. Histopathology confirmed the diagnosis of low-grade spindle melanoma of the ciliary body with multiple empty and fluid filled cyst-like cavities without epithelial lining. UBM is an important diagnostic tool in the differentiation of "thick walled" cavitary melanoma from "thin walled" benign pigment epithelial cyst

Iris melanoma in children: current approach to management

Iris melanoma usually affects middle aged, Caucasian patients with light colored eyes. Pediatric iris melanoma is rare. A 15-year-old Caucasian male presented with 1-month history of a brown nodule in the inferotemporal aspect of his left eye. Iris nevus was diagnosed, and the patient was observed. Nearly 2 years later the lesion had grown in basal diameter and thickness, and the tumor was excised by partial lamellar scleral flap and sector iridectomy. Histopathology confirmed spindle cell iris melanoma. Two years post-operatively, tumor recurrence with anterior chamber angle involvement and secondary glaucoma developed. He was then treated with custom designed Iodine[125] plaque radiotherapy. This case demonstrates the recurrence of iris melanoma despite proper initial surgical management, and outlines current management options for pediatric iris melanoma

Cystoid macular edema as the initial manifestation of choroidal melanoma

Uveal melanomas are a common clinical entity that initially present in a variety of ways. Cystoid macular edema is caused by many conditions, but it is rarely associated with uveal melanoma. We report two cases of patients that presented with visually significant cystoid macular edema that was later found to be secondary to choroidal melanoma. We describe the patients' course and treatment and provide a mechanism for the potential cause of edema in patients with uveal melanoma

Vitreous asteroid hyalosis prolapse into the anterior chamber simulating iris metastasis

Two asymptomatic elderly women who underwent cataract extraction 7 or more years previously and with intraocular lens placement presented with a linear bead-like white multinodular mass in the inferior angle simulating iris metastasis versus large inflammatory precipitates. There was no iris infiltration. In the first case, the posterior lens capsule was intact and there was no evidence of gelatinous vitreous in the anterior chamber, whereas in the second case, the capsule was open and there was gelatinous vitreous prolapse. In both cases, there was asteroid hyalosis in the vitreous. Both patients were diagnosed with prolapsed vitreous asteroid hyalosis into the anterior chamber and managed with observation. Vitreous asteroid hyalosis can prolapse into the anterior chamber of pseudophakic elderly patients with or without capsular opening and can simulate an intraocular tumor

Fundus autofluorescence and optical coherence tomography findings in choroidal melanocytic lesions

To establish the characteristics of secondary retinal and retinal pigment epithelial [RPE] changes associated with the presence of choroidal melanoma and choroidal nevus as documented by optical coherence tomography [OCT] and fundus autofluorescence [FAF]. PubMed review of major English publications examining the correlation between clinical characteristics of choroidal melanoma and nevus with OCT and FAF findings. The intrinsic properties of choroidal melanoma, as well as overlying RPE changes, drusen, and lipofuscin are best characterized by FAF, while OCT is more sensitive for the identification of subretinal and intraretinal fluid as well as a trophy, degeneration, and photoreceptor loss in the neurosensory retina. Secondary retinal changes associated with choroidal melanocytic lesions can be documented by OCT and FAF. OCT evident changes are observed more often with choroidal melanoma than choroidal nevus. OCT is better suited to identify the overlying retinal detachment and edema, even before these findings are clinically apparent. FAF is most useful in documenting the presences of lipofuscin, a finding that represents one of the important criteria in differentiating small choroidal melanoma from benign choroidal nevus

Small choroidal melanoma with monosomy 3

To report a patient with small juxtapapillary choroidal melanoma with chromosome 3 monosomy treated with I[125] plaque and transpupillary thermotherapy [TTT]. A 64-year-old Caucasian male presented with painless blurred vision of the left eye. Ocular examination disclosed a small juxtapapillary choroidal melanocytic tumor with overlying subretinal fluid and orange pigment. Ultrasound showed an elevated choroidal mass of 2 mm thickness with low reflectivity on A-scan and hollowness on B scan, consistent with a small choroidal melanoma. The patient was treated with plaque I[125] radiotherapy combined with one session of TTT. Genetic testing of the tumor cells obtained by fine needle aspiration biopsy showed chromosome 3 monosomy. At 1 year after treatment, the tumor was regressed with resolution of subretinal fluid and 20/40 visual acuity. A small choroidal melanoma can manifest monosomy of chromosome 3, a known predictive factor for the development of systemic metastasis

Pearls in the management of children with retinoblastoma

The management of retinoblastoma has gradually evolved over the past years from enucleation to radiotherapy to current techniques of chemotherapy. Eyes with massive retinoblastoma filling the globe are still managed with enucleation, whereas those with small, medium, or even large tumors can be managed with chemoreduction followed by tumor consolidation with thermotherapy or cryotherapy. Despite multiple or large tumors, visual acuity can reach 20/40 or better in many cases, particularly those eyes with extramacular tumors. Previous complications of dry eye, cataract, retinopathy, and facial deformity that were found following external beam radiotherapy are not anticipated following chemoreduction. Recurrence from subretinal and vitreous seeds can be problematic. Long-term follow up for second cancers is advised