Iris nevus growth into melanoma: analysis of 1611 consecutive eyes: the ABCDEF guide.

PURPOSE: To determine clinical features predictive of growth of iris nevus into melanoma. DESIGN: Retrospective, comparative case series. PARTICIPANTS: A total of 1611 consecutive patients referred to an ocular oncology center with iris nevus. INTERVENTION: Observation and photographic documentation. MAIN OUTCOME MEASURES: Growth into melanoma. RESULTS: The mean age at referral for iris nevus was 51 years (median, 54; range, <1-94 years). At presentation, the mean tumor basal diameter was 3 mm (median, 3 mm; range, <1-12 mm) and mean tumor thickness was 0.8 mm (median, 0.5 mm; range, 0-5 mm). All patients were initially diagnosed with benign iris nevus. Growth of iris nevus to melanoma was confirmed in 2% of eyes (n = 27) over a mean follow-up of 68 months (median, 46 months; range, 3-465 months). By Kaplan-Meier estimates, iris nevus growth to melanoma occurred in <1%, 3%, 4%, 8%, and 11% at 1, 5, 10, 15, and 20 years, respectively. Factors predictive of iris nevus growth to melanoma by multivariable analysis included age ≤ 40 years at presentation (hazard ratio [HR], 3), episode of hyphema (HR, 9), 4:00 to 9:00 clock hour location of tumor (HR, 9), diffuse tumor (involving entire iris surface) (HR, 14), ectropion uveae (HR, 4), and feathery tumor margins (HR, 3). Additional important factors by univariable analysis included tumor seeding on the iris or in the anterior chamber angle, feeder vessels, and nodule formation. These factors can be remembered using the mnemonic ABCDEF, representing A = age young, B = blood, C = clock hour inferior, D = diffuse, E = ectropion, and F = feathery margin. CONCLUSIONS: In an analysis of 1611 cases of iris nevus referred for evaluation at an ocular oncology center, growth into melanoma occurred in 8% by 15 years. Risk factors for growth, identified by ABCDEF included Age young, Blood (hyphema), Clock hour inferior, Diffuse configuration, Ectropion uveae, and Feathery tumor margin.

Bilateral anterior and posterior scleritis in a patient with acute myelogenous leukemia.

Purpose: To report a novel case of bilateral anterior and posterior scleritis in a patient with acute myelogenous leukemia (AML). Observations: A 69-year-old African American man was admitted to the hospital for relapse of AML. After admission, but prior to induction of chemotherapy, the patient developed ocular redness and proptosis. The diagnosis of bilateral anterior and posterior scleritis was made following an ophthalmic examination, infectious and autoimmune lab work-up, and neuroimaging. The patient was administered immunosuppressive therapy, clinically monitored, and initiated on chemotherapy for AML relapse. About one week later, the patient showed clinical improvement and resolution of the scleritis and proptosis. Conclusion: Scleritis may present during AML relapse, and it may be due to a paraneoplastic syndrome or a reactive anti-leukemic inflammatory response. Clinicians should monitor patients with AML relapse for symptoms such as ocular redness, proptosis, pain, photophobia, and decreased vision, which may indicate development of scleritis.

Iris metastasis from systemic cancer in 104 patients: the 2014 Jerry A. Shields Lecture.

PURPOSE: To describe the clinical features, treatment, and outcome of patients with iris metastasis. METHODS: Retrospective case series of 160 tumors in 107 eyes of 104 patients with iris metastasis from systemic cancer. RESULTS: The median age at presentation with iris metastasis was 60 years. Iris metastasis was more common in whites (n = 93, 89%) and women (n = 65, 62%). The primary tumor was located in the breast (n = 34, 33%), lung (n = 28, 27%), skin (melanoma) (n = 13, 12%), kidney (n = 7, 7%), esophagus (n = 3, 3%), and others (n = 19, 19%). The main symptoms were pain (n = 33, 32%) or blurred vision (n = 31, 30%). The main findings were corectopia (n = 38, 37%) and secondary glaucoma (n = 38, 37%). The metastasis was unifocal (n = 84, 78%) or multifocal (n = 23, 21%) for a total of 160 tumors. The main tumor was centered at the iris root (n = 72, 69%), midzone (n = 22, 21%), or pupillary margin (n = 10, 10%), with median tumor diameter of 5 mm. Coexistent conjunctival or ciliary body/choroidal metastases were found in 39 patients. Management of iris metastasis included systemic chemotherapy (n = 18, 22%), external beam radiotherapy (n = 34, 41%), plaque radiotherapy (n = 20, 24%), surgical excision (n = 4, 5%), enucleation (n = 3, 4%), or observation (n = 4, 5%). Following treatment, tumor control without recurrence was achieved in 95% cases. In 98 cases with adequate follow-up information, death occurred in 85 (87%) at median 10 months (range, <1-239 months) from the date of iris metastasis. CONCLUSIONS: Metastatic tumors to the iris generally originate from primary malignancies in the breast, lung, or skin (melanoma). Despite successful ocular treatment, life prognosis is poor.

Medulloepithelioma: A triad of clinical features.

BACKGROUND: Intraocular medulloepithelioma arises from the primitive medullary epithelium and is diagnosed at a median age of five years. This tumor most commonly appears as a white, gray, or yellow-colored ciliary body tumor. The growth of medulloepithelioma is slow and it is locally invasive. Poor vision and pain are the most common presenting symptoms. The most common clinical signs include cyst or mass in iris, anterior chamber or ciliary body, glaucoma, and cataract. CASE: A 22-month-old Caucasian female twin presented with leukocoria and poor vision in OS. Examination revealed normal findings OD and a mass in OS. Based on the clinical features of leukocoria, lens changes and a white cystic ciliary body mass in a young child, ultrasonographic, and transillumination features, the lesion was diagnosed as a non-pigmented ciliary epithelial medulloepithelioma. After enucleation, the diagnosis of malignant teratoid medulloepithelioma of the non-pigmented ciliary epithelium was confirmed. There was no evidence of tumor recurrence or systemic metastasis at three years follow-up. CONCLUSION: Medulloepithelioma in a child can present as a clinical triad of leukocoria, lens changes, and a white cystic ciliary body mass.