Hakan Demirci

Cme Ring Melanoma Of The Ciliary Body: Report on Twenty-three Patients

Background Ring melanoma of the ciliary body is a rare variant of uveal melanoma that has a poor prognosis. Only isolated cases have been reported in the literature. Methods A retrospective review of the clinical features, management, histopathology, and prognosis of 23 consecutive patients with ring melanoma of the ciliary body was performed. Results Of 8,800 patients with uveal melanoma treated on the Oncology Service at Wills Eye Hospital over a 25-year period, only 23 patients (0.3%) were found to have ring melanoma of the ciliary body. The mean patient age was 59 years (median, 63 years; range, 8–81 years). The only visual symptom was blurred vision in 17 patients (74%), and the other six patients (26%) were asymptomatic. At the initial presentation elsewhere, melanoma was recognized in 13 cases (57%). In the remaining 10 cases (43%), the initial clinical diagnosis was glaucoma in three (13%), iris nevus in three (13%), cataract in two (9%), ciliary body detachment in one (4%), and iridocyclitis in one (4%). The patients were observed elsewhere for a mean of 3 months before the diagnosis of melanoma was suspected and referral to Oncology Service was made. On examination, the tumor involved a mean of eight clock hours of the ciliary body. Seven patients (30%) had complete circumferential (360°) involvement of the ciliary body, and 16 (70%) had incomplete (180–360°) ring involvement. The mean tumor thickness was 8 mm. Associated findings included neovascularization of the iris in one case (4%), shallow anterior chamber in 11 (48%), anterior chamber inflammation in five (22%), cataract in nine (39%), lens indentation in eight (35%), and lens subluxation in three (13%). A prominent episcleral (sentinel) vessel (17 cases, 74%), multilobulation of the mass (19 cases, 83%), blockage of light on transillumination (23 cases, 100%), and ultrasonographic hollowness with intrinsic pulsations (23 cases, 100%) were important features differentiating this tumor from simulating conditions. The tumor was managed with enucleation in all 23 patients. Histopathologic studies revealed epithelioid cell–type melanoma in two patients (9%), mixed cell–type in 17 (74%), and spindle cell–type in four (17%). Extraocular extension was present in eight patients (35%). Metastasis developed in 12 of 23 patients (52%) after a mean follow-up of 55 months. Conclusions Ring melanoma of the ciliary body is a rare tumor that can remain hidden from ophthalmoscopic examination. Certain features, such as prominent episcleral sentinel vessels, multilobulation of the mass, light blockage on transillumination, and ultrasonographic hollowness with intrinsic vascular pulsations, suggest the diagnosis. The life prognosis is poor.

Iodine I 125 Plaque Radiotherapy for Vasoproliferative Tumors of the Retina in 30 Eyes

OBJECTIVEnTo report the efficacy of iodine I 125 plaque radiotherapy for the treatment of vasoproliferative tumors (VPTs) of the ocular fundus.nnnMETHODSnThe clinical features and outcomes of patients with VPTs who underwent iodine I 125 plaque radiotherapy were evaluated. Univariate and multivariate logistic regression analyses were performed to assess the effect of preoperative findings on visual acuity and complications. Kaplan-Meier survival estimates for the probability of adverse outcomes were performed.nnnRESULTSnOf the 30 eyes treated, 17 (57%) had primary and 13 (43%) had secondary VPTs. The median tumor base was 8.6 mm (range, 3.5-18.0 mm) and median tumor thickness was 3.7 mm (range, 2.5-6.3 mm). Exudative retinal detachment was present in 23 eyes (77%). Tumor regression was observed in 29 of 30 eyes (97%) and retinal detachment completely resolved in 15 of 23 eyes (65%). Visual acuity improved or remained stable in 22 eyes (73%). The only factor predictive of visual improvement was the classification of primary VPT (relative risk, 19; 95% confidence interval, 2-185; P = .01). Kaplan-Meier estimates of radiation complications at 5 years predicted cataract in (48%), transient vitreous hemorrhage in (16%), and neovascular glaucoma in (8%) of eyes. No patient developed radiation maculopathy or papillopathy.nnnCONCLUSIONSnIodine I 125 plaque radiotherapy is an effective method of treating larger (>2.5-mm thickness) VPTs with extensive exudative retinal detachment.

Dramatic regression of conjunctival and corneal acquired melanosis with topical mitomycin C

Conjunctival primary acquired melanosis is the most important precursor of conjunctival malignant melanoma.1,2 Primary acquired melanosis appears as flat, patchy, non-cystic pigmentation in the conjunctival epithelium and can remain dormant for years or show slow progression.2 Studies have shown that it leads to conjunctival melanoma in approximately 1% to 30% patients.3,4 Treatment of primary acquired melanosis includes observation, excisional biopsy, alcohol epitheliectomy, cryotherapy, and topical chemotherapy.1,2,5,6 We illustrate a dramatic case where topical chemotherapy provided complete regression of advanced, aggressive primary acquired melanosis.nnA 73 year old white man had noted slowly progressive pigmentation on the surface of his right eye for 5 years. A biopsy revealed intraepithelial conjunctival melanosis with atypia. The patient was referred to the Oncology Service at Wills Eye Hospital for evaluation and management. On examination, his visual acuity was 20/20 right …

Leucocoria as the presenting sign of a ciliary body melanoma in a child

Editor,—Uveal melanoma is generally a disease of adulthood. It has been reported that 0.6% to 1.6% of all uveal melanomas occur in patients under 20 years of age. In a review of 3706 consecutive patients with uveal melanoma, Shields and associates found that 1.1% were children and teenagers younger than 20 years of age, of whom only 0.3% had ciliary body melanoma. nnPatients with ciliary body melanoma usually are asymptomatic until the tumour impinges on the lens and causes visual distortion. Children with intraocular tumours generally have few visual symptoms and adapt to visual distortion without complaints. Leucocoria in childhood is the most frequent presenting sign of retinoblastoma, but it is generally not associated with uveal melanoma. We report an unusual case of a 9 year old child with a ciliary body melanoma who presented with leucocoria.nn### CASE REPORTnnA 9 year old white girl was referred to Oncology Service at Wills Eye Hospital with a 1 month history of leucocoria and strabismus in her right eye (Fig 1A). She was otherwise healthy and her medical history was unremarkable.nnnnFigure 1 nSlit lamp photograph showing the inferonasal dark tumour, subluxing the cataractous lens, causing leucocoria (A). Gross pathology reveals …

Benign lymphoid infiltrate of the iris simulating a malignant melanoma

Purpose. To report a clinicopathologic correlation of an unusual benign lymphocytic iris mass in a patient who had no systemic lymphoproliferative disease. Methods. Case report. Results. A 49-year-old man developed a circumscribed, tan lesion in his left iris. The lesion was suspected clinically to be an atypical iris melanoma. Histopathologic studies of the resected mass revealed a solid tumor that was comprised of lymphocytes and histiocytes. Immunohistochemical studies identified that most of the cells were T lymphocytes. The histopathologic diagnosis was atypical lymphoid infiltrate. Workup for systemic lymphoma and Epstein-Barr virus infection was negative. Conclusion. Lymphoid infiltrate can manifest as a solitary mass that can simulate an iris melanoma.