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Hakan Demirci

Publications and source records attributed to Hakan Demirci.

At least 37 records · Page 2Linked to original sources
PubMed2004

Clinical factors in the identification of small choroidal melanoma.

The detection and treatment of choroidal melanoma early in its natural course is critical to providing the patient with the best prognosis. Studies of tumour doubling time have indicated that metastasis from choroidal melanoma can occur quite early in the course of the disease, when the tumour is about 3.0 mm in basal dimension and 1.5 mm in thickness. Clinical studies have shown that, at 5 years, metastasis occurs in 16% of patients with small choroidal melanomas (less than 4 mm thick), compared with 32% of those with medium-sized (4-8 mm thick) choroidal melanomas and 53% of those with large (more than 8 mm thick) choroidal melanomas. The difficulty with early detection of choroidal melanoma relates to its clinical similarity to benign choroidal nevus. Factors that assist in differentiating small choroidal melanoma from choroidal nevus can be remembered using the mnemonic "TFSOM" (to find small ocular melanoma), where T = thickness greater than 2 mm, F = subretinal fluid, S = symptoms, O = orange pigment and M = margin touching optic disc. Choroidal melanocytic tumours that display none of these factors have a 3% risk of growth into melanoma at 5 years and most likely represent choroidal nevi. Tumours that display one factor have a 38% risk of growth, and those with two or more factors show growth in over 50% of cases. Most tumours with two or more risk factors probably represent small choroidal melanomas, and early treatment is generally indicated. Therefore, ophthalmologists should be aware of the clinical factors that identify small choroidal melanoma so that early treatment and better prognosis can be achieved for their patients.

Choroid Neoplasms↗
PubMed2003

Histopathologic findings in eyes with retinoblastoma treated only with chemoreduction.

OBJECTIVE: To evaluate the histopathologic findings in the eyes with retinoblastoma that had been treated only with chemoreduction. DESIGN: Clinicopathologic series. Study Material Ten eyes of 8 patients with retinoblastoma that were enucleated after therapy consisting only of systemic chemotherapy (chemoreduction). METHODS: All cases received a chemoreduction regimen including a combination of intravenous carboplatin, etoposide phosphate, and vincristine sulfate. Adjuvant treatment to the tumor was not provided in any case. The enucleated globes were studied by routine light microscopy. Main Outcome Measure Histopathologic features of retinoblastoma following chemoreduction. RESULTS: At presentation, there were 8 eyes in Reese-Ellsworth group V, 1 eye in group IV, and 1 eye in group III. After chemoreduction (mean, 4 cycles; range, 1-6 cycles), the main tumor regressed a mean 34% in thickness and 24% in basal diameter. The indication for enucleation was retinoblastoma recurrence as subretinal and/or vitreous seeds in 7 eyes and extensive vitreous hemorrhage in 3 with uncertainty about viable-appearing tumor. In no case was enucleation performed for recurrence of the main tumor. In all eyes, there was histopathologic evidence of tumor regression. In 8 of 10 eyes, histopathologic examination disclosed tumor regression without viable-appearing retinoblastoma in the main tumor. Of these 8 eyes, 2 showed a completely calcified glial scar and 6 showed an apical calcified glial scar and a basal residual well-differentiated component with retinomalike and/or retinocytomalike features. In the remaining 2 eyes, an area of posttherapeutic regression was present but contained foci of mitotically active, viable-appearing malignant retinoblastoma cells. The 6 eyes found to contain well-differentiated component with retinomalike and/or retinocytomalike features showed a mean decrease of 17% in largest basal dimension and 32% in thickness after a mean of 3 cycles of chemoreduction. In contrast, the 4 eyes that did not contain well-differentiated component with retinomalike and/or retinocytomalike features showed a mean decrease of 35% in largest basal dimension and 55% in thickness after a mean of 5 cycles of chemoreduction. Of those 7 eyes enucleated for recurrent subretinal and/or vitreous seeds, viable tumor seeds were confirmed histopathologically in all cases. There was no histopathologic evidence of chemotherapeutic toxicity to the eye. CONCLUSIONS: Histopathologic examination of 10 enucleated eyes following chemoreduction alone revealed that the main retinoblastoma regressed in all eyes. Additionally 6 eyes showed basal residual well-differentiated component with retinomalike and/or retinocytomalike features, and these eyes also displayed less shrinkage with chemoreduction. Despite the lack of viable-appearing retinoblastoma within the main tumor, enucleation was performed for viable subretinal and/or vitreous seeds in 7 cases and confirmed histopathologically.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Bilateral optic disk melanocytoma in a 10-month-old infant.

PURPOSE: To report bilateral optic disk melanocytoma in a healthy infant, METHOD: Case report. RESULTS: A 10-month-old healthy boy presented with intermittent right exotropia. Dilated fundus examination showed a heavily pigmented mass within both optic disks. The optic disks were hypoplastic, and there was diffuse retinal pigment epithelial mottling around the optic disks. Systemic evaluation including thyroid function tests, pituitary gland hormone levels, adrenal gland hormone levels, and magnetic resonance imaging of the brain revealed normal findings. After 1 year, the lesions remained stable, and the patient was healthy with normal growth and development. CONCLUSION: Bilateral optic disk melanocytoma is uncommon. Although this patient had no systemic problem, a literature review revealed an association of bilateral optic disk melanocytoma, optic disk hypoplasia, and central nervous system abnormalities such as meningioma and hypopituitarism. Systemic evaluation for central nervous system abnormalities is warranted in such cases.

Humans↗
PubMed2003

Uveal metastasis from breast cancer in 264 patients.

PURPOSE: Breast cancer is an increasingly important health problem in women and is the most common tumor to metastasize to the uvea. This study was designed to evaluate the clinical features, management, and prognosis of patients with uveal metastasis from breast cancer. DESIGN: Retrospective interventional case series. METHODS: We retrospectively reviewed 264 consecutive patients with uveal metastasis from breast cancer. We assessed the clinical features of the patient and tumor at the time of presentation, management, and prognosis. Kaplan-Meier survival estimates were used to analyze the probability of death as a function of time. RESULTS: Uveal metastasis was the initial manifestation of breast cancer in seven patients (3%) and the first systemic metastatic site of previously diagnosed breast cancer in 43 (16%). Associated with uveal metastasis, optic disk metastasis was found in 13 patients (5%), eyelid metastasis in one patient (1%), and conjunctival and orbital in one patient (<1%). Of 264 patients with uveal metastasis, 225 (85%) had choroidal metastasis, eight (3%) iris metastasis, two (<1%) ciliary body metastasis, and 29 (11%) had metastasis in multiple uveal sites. In the 264 patients with uveal metastasis, the most common symptom was blurred vision in 197 patients (88%), floaters in 15 (5%), photopsia in 12 (5%), and 19 (7%) were asymptomatic. The uveal metastases were bilateral in 99 patients (38%) and unilateral in 165 (62%). In 55 (56%) of the 99 bilateral cases, a uveal metastasis was found in the asymptomatic fellow eye during follow-up examination. External beam radiotherapy was used in 137 patients with uveal metastasis (52%), providing tumor control in 116 patients (85%) at a mean follow-up of 21 months. Using Kaplan-Meier estimates, survival rates of all patients with uveal metastasis from breast cancer was 65% at 1-year, 34% at 3-year, and 24% at 5-year follow-up. CONCLUSIONS: Patients with uveal metastasis from breast cancer presented to ophthalmologists with visual symptoms in 93% of cases. However, asymptomatic metastases were commonly detected in the fellow eye. Local ocular tumor control was excellent with current therapies. However, systemic prognosis for all patients, including those who had been treated with different management options, was poor with survival rates of 65% at 1-year and 24% at 5-year follow-up.

Adult↗
PubMed2002

Chemoreduction for unilateral retinoblastoma.

OBJECTIVE: To evaluate conservative management of unilateral retinoblastoma using chemoreduction and focal treatment. DESIGN: Prospective nonrandomized single-center clinical trial. SETTING: Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University, Philadelphia, Pa, in conjunction with the Division of Oncology at The Children's Hospital of Philadelphia. PARTICIPANTS: Thirty eyes of 30 patients with unilateral retinoblastoma treated with chemoreduction between June 1, 1994, and August 31, 1999, that would otherwise have been managed with enucleation or external beam radiotherapy. INTERVENTION: All patients received treatment for retinoblastoma with a planned 6 cycles of chemoreduction using vincristine sulfate, etoposide, and carboplatin, combined with focal treatment (cryotherapy or thermotherapy) to each retinal tumor. MAIN OUTCOME MEASURES: The main outcome measure was the postchemoreduction need for external beam radiotherapy or enucleation. The cumulative probability of each outcome was estimated using Kaplan-Meier survival analysis. A secondary outcome measure was final visual acuity in the affected eye. The clinical features at the time of patient presentation were analyzed for their impact on the main outcomes using a series of Fisher exact tests and Cox proportional hazards regressions. RESULTS: Eighteen eyes (60%) were classified as having Reese-Ellsworth (RE) groups I through IV retinoblastoma and 12 eyes (40%), group V retinoblastoma. By using Kaplan-Meier estimates, we found a need for either external beam radiotherapy or enucleation in 68% of eyes by 5 years. In fact, 38% of those in groups I through IV required either treatment, whereas all of those in group V required the additional use of either treatment. Specifically, the need for external beam radiotherapy occurred in 27% of eyes by 5 years. Eleven percent of those in groups I through IV and 50% of group V required external beam radiotherapy by 5 years. The factors predictive of the need for external beam radiotherapy included RE group V disease, tumor thickness greater than 5 mm, and presence of vitreous seeds. The need for enucleation occurred in 47% of eyes by 5 years using Kaplan-Meier analysis. Specifically, 29% of those in groups I through IV and 67% of group V required enucleation by 5 years. The factors predictive of the need for enucleation included age at diagnosis older than 12 months, RE group V disease, tumor base diameter greater than 15 mm, and tumor thickness greater than 5 mm. At a mean follow-up of 29 months, the final visual acuity was 20/200 or better in 6 eyes (20%) and worse than 20/200 in 14 (47%); enucleation was needed in 10 (33%). Of the 26 eyes with initial macular involvement of retinoblastoma, final visual acuity was 20/200 or better in 6 (23%). No patient developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms. CONCLUSIONS: Chemoreduction is an option for selected eyes with unilateral retinoblastoma. Those with advanced RE group V retinoblastoma showed poorest results, while those with less advanced groups I through IV disease showed best results, maintaining the globe in 71% of eyes, sometimes with satisfactory functional visual acuity.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Solitary idiopathic choroiditis: the Richard B. Weaver lecture.

OBJECTIVE: To report the clinical characteristics of solitary idiopathic choroiditis and the features that differentiate it from tumors and other inflammatory lesions. DESIGN: Retrospective medical record review. PATIENTS: Sixty consecutive patients with solitary idiopathic choroiditis. MAIN OUTCOME MEASURES: Clinical features, natural course, and follow-up. RESULTS: The mean and median ages of the patients were 35 and 36 years, respectively, 56 (93%) of the 60 patients were white, 38 (63%) were female, and 22 (37%) were male. No patient had a history, clinical findings, or laboratory study results to support a specific cause of uveitis. All were referred to us because of a suspected intraocular tumor. The patient was asymptomatic in 21 cases (35%); the patient had mild visual loss in 36 cases (60%). The lesion was posterior to the equator in 56 cases (93%), was yellow in 58 cases (97%), and had distinct margins in 38 cases (63%). The lesion showed signs of active inflammation in 20 cases (33%) and no inflammation in 40 cases (67%). Fluorescein angiography disclosed that all lesions had early hypofluorescence and late hyperfluorescence. In the late-phase angiograms inactive lesions had distinct margins and active lesions had ill-defined margins. Lesions with active inflammation appeared to show a favorable response to the administration of systemic corticosteroids but generally improved with or without treatment. Most of the inactive lesions remained stable on long-term follow-up. Overall, the condition remained stable in 36 patients (60%), improved in 22 (37%), and recurred in 2 (3%). The clinical and angiographic features and clinical course of solitary idiopathic choroiditis were generally different from known intraocular tumors. CONCLUSIONS: Solitary idiopathic choroiditis is a distinct clinical entity that can simulate an intraocular neoplasm. Recognition of its typical clinical features can assist in differentiating it from tumors and other inflammatory lesions of the ocular fundus.

Adolescent↗
PubMed2002

Postenucleation adjuvant therapy in high-risk retinoblastoma.

PURPOSE: The main purpose of this study was to determine the efficacy of postenucleation adjuvant therapy in preventing metastasis in cases of high-risk retinoblastoma. METHODS: This was a retrospective, nonrandomized comparative study. Of 1020 consecutive patients with retinoblastoma had were managed at a referral center between January 1974 and December 1999, 80 (8%) of those analyzed had unilateral sporadic cases that were treated by primary enucleation and that had high-risk characteristics for metastasis on histopathology reports (anterior chamber seeding, iris infiltration, ciliary body infiltration, massive choroidal infiltration, invasion of optic nerve lamina cribrosa, retrolaminar optic nerve invasion, invasion of optic nerve transection, scleral infiltration, and extrascleral extension). The main outcome measure was the development of metastasis at a minimum follow-up period of 12 months. RESULTS: There were 44 male and 36 female patients, with age ranging from 1 day to 16 years (median, 33 months). A single histopathologic high-risk characteristic was present in 50 patients (62.5%). Thirty patients (37.5%) manifested 2 or more high-risk characteristics. Forty-six patients (58%) had received postenucleation adjuvant therapy (chemotherapy with or without orbital external beam radiotherapy). Adjuvant therapy was not administered in 34 patients (42%). Metastasis occurred in 10 patients (13%) at a median of 9 months (range, 6-57 months) following enucleation. Eight (80%) of those who developed metastasis had not received adjuvant therapy. A significant difference (P =.02) was found in the incidence of metastasis between the group that had received adjuvant therapy (4%; 2/46) and the group that had not (24%; 8/34). The beneficial effect of adjuvant therapy was statistically significant in subgroups of patients with massive choroidal infiltration (P =.04) or retrolaminar optic nerve invasion (P =.04). There were no adjuvant therapy-related serious systemic complications. CONCLUSION: Postenucleation adjuvant therapy is safe and effective in significantly reducing the occurrence of metastasis in patients with retinoblastoma manifesting histopathologic high-risk characteristics.

Adolescent↗
PubMed2002

Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation.

PURPOSE: To report the results of chemoreduction and focal therapy for retinoblastoma with determination of factors predictive of the need for treatment with external beam radiotherapy or enucleation. DESIGN: Interventional case series. METHODS: One-hundred three patients with retinoblastoma (158 eyes with 364 tumors) at the Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at Children's Hospital of Philadelphia from June 1994 to August 1999 were enrolled for this prospective clinical trial. The patients received treatment for retinoblastoma with six planned cycles (one cycle per month) of chemoreduction using vincristine, etoposide, and carboplatin combined with focal treatments (cryotherapy, thermotherapy, or plaque radiotherapy). The two main outcome measures after chemoreduction and focal therapy were the need for external beam radiotherapy and the need for enucleation. The clinical features at the time of patient presentation were analyzed for impact on the main outcome measures using a series of Cox proportional hazards regressions. RESULTS: Using Reese-Ellsworth (RE) staging for retinoblastoma, there were nine (6%) eyes with group I disease, 26 (16%) eyes with group II disease, 16 (10%) eyes with group III disease, 32 (20%) eyes with group IV disease, and 75 (48%) eyes with group V retinoblastoma. All eyes showed initial favorable response with tumor regression. The median follow-up was 28 months (range, 2-63 months). Failure of chemoreduction and need for treatment with external beam radiotherapy occurred in 25% of eyes at 1 year, 27% at 3 years, and no further increase at 5 years. More specifically, external beam radiotherapy was necessary at 5 years in 10% of RE groups I-IV eyes and 47% of RE group V eyes. Multivariate factors predictive of treatment with external beam radiotherapy included non-Caucasian race, male sex, and RE group V disease. Failure of chemoreduction and the need for treatment with enucleation occurred in 13% eyes at 1 year, 29% at 3 years, and 34% at 5 years. More specifically, enucleation was necessary in 15% of RE groups I-IV eyes at 5 years and in 53% of RE group V at 5 years. Multivariate factors predictive of treatment with enucleation included patient age older than 12 months, single tumor in eye, and tumor proximity to foveola within 2 mm. Overall, of the 158 eyes, 50% required external beam radiotherapy or enucleation and 50% were successfully managed without these treatments. No patient developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms over the 5-year follow up. CONCLUSIONS: Chemoreduction offers satisfactory retinoblastoma control for RE groups I-IV eyes, with treatment failure necessitating additional external beam radiotherapy in only 10% of eyes and enucleation in 15% of eyes at 5-year follow-up. Patients with RE group V eyes require external beam radiotherapy in 47% and enucleation in 53% at 5 years.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Orbital tumors in the older adult population.

PURPOSE: To determine the types and frequency of orbital tumors in the older adult population. DESIGN: Retrospective consecutive case series. PARTICIPANTS: Two hundred consecutive patients aged 60 years or older with an orbital tumor managed at a tertiary referral center. MAIN OUTCOME MEASURES: The relative incidence and management of orbital tumors in patients aged 60 years or older. RESULTS: Of approximately 950 consecutive patients with orbital space-occupying lesions managed over a 25-year period on the Oncology Service, 200 patients (21%) were 60 years or older at the time of diagnosis. The orbital tumor was unilateral in 183 patients (92%) and bilateral in 17 patients (8%). The most common clinical features at presentation included mass in 51 patients (26%), proptosis in 36 (18%), and pain in 30 (15%). The mean duration of symptoms was 11 months before referral. The orbital tumor was malignant in 125 patients (63%) and benign in 75 (27%). The most common diagnoses were malignant lymphoma in 47 patients (24%), idiopathic orbital inflammation in 19 (10%), and cavernous hemangioma in 15 (8%). The diagnosis was made with clinical findings and imaging studies in 58 patients (29%) and additional histopathologic examination in 142 patients (71%). Of 217 tumors, the main tumor location was in the superior half of the orbit in 106 lesions (49%), in the anterior orbit in 100 (46%), and in the extraconal space in 135 (62%). Imaging studies showed a mass with well-defined margins in 90 tumors (41%) and ill-defined margins in 127 (59%). The tumors were solid in 203 cases (94%), cystic in 6 (3%), and mixed in 8 (3%). Over a mean of 31 months follow-up, 50 (25%) patients had orbital tumor-related systemic involvement (metastasis or lymphoma), and 150 (75%) remained free of systemic involvement. CONCLUSIONS: Orbital tumors in the senior adult population are malignant in 63% of cases. Malignant lymphoma is the most common tumor in this age group, accounting for 24% of cases. Overall, 25% of patients have systemic problems related to the orbital process develop, so systemic evaluation is warranted.

Aged↗
PubMed2002

Diffuse iris melanoma: a report of 25 cases.

BACKGROUND: Diffuse iris melanoma is a rare variant of iris melanoma that has a flat growth pattern and often presents as unilateral hyperchromic heterochromia and glaucoma. There have been no large clinical reports of diffuse iris melanoma. DESIGN: Single-center retrospective case series. PARTICIPANTS: Twenty-five consecutive patients with diffuse iris melanoma. METHODS: A review of the clinical features, management, histopathologic analysis, and prognosis was performed. MAIN OUTCOME MEASURES: Clinical features, histopathologic features, management, and prognosis. RESULTS: At the time of diagnosis, the mean patient age was 49 years. The initial complaint was unilateral darker eye color in 13 cases (52%) and blurred vision in 6 (24%). Six patients (24%) were symptom free. On presentation elsewhere, the initial diagnosis was iris melanoma or nevus in 11 cases (44%) and glaucoma in 14 (56%), 8 (32%) of whom had prior laser or surgical treatment for glaucoma. The 25 patients were observed for a mean of 30 months before the diagnosis of melanoma was suspected and referral to our center for evaluation. The mean intraocular pressure at referral was 36 mmHg, and the mean number of clock hours involved by tumor was 11. Heterochromia iridis was present in all 25 cases (100%), corectopia in 23 (92%), and ectropion iridis in 21 (84%). Associated findings included unilateral cataract in 8 (32%), a prominent episcleral (sentinel) vessel in 7 (28%), and anterior chamber inflammation in 5 (20%), and synechiae (anterior or posterior) in none. The tumor was managed by enucleation in 22 cases (88%) and by plaque brachytherapy in 3 (12%). Five cases (20%) were classified as spindle melanoma, 17 (68%) as mixed cell type, and 3 (12%) as epithelioid cell type. Therefore, histopathologic examination showed that 80% of cases contained epithelioid cells. All 22 enucleated cases were found to have tumor invasion in the trabecular meshwork. Tumor invasion of Schlemm's canal was found in 18 (82%), minor pars plicata in 12 (55%), and episclera in 7 cases (28%). Of seven cases with episcleral invasion, four underwent surgical treatment for glaucoma. Excluding one recent case, the patients were observed for a mean of 78 months. Liver metastasis developed in 3 cases (13%). CONCLUSIONS: Diffuse iris melanoma is a serious ocular condition that causes unilateral hyperchromic heterochromia and secondary glaucoma, often leading to a delay in diagnosis. Local invasion of adjacent ocular structures is common, and distant metastasis occurs in 13% of cases at mean follow-up of 78 months.

Adolescent↗
PubMed2002

Plaque radiotherapy for large posterior uveal melanomas (> or =8-mm thick) in 354 consecutive patients.

OBJECTIVE: To assess treatment complications and tumor control after plaque radiotherapy for large posterior uveal melanomas measuring 8 mm or greater in thickness. DESIGN: Prospective noncomparative interventional case series. PARTICIPANTS: Three hundred fifty-four patients each of whom had a posterior uveal melanoma measuring 8 mm or greater in thickness treated with plaque radiotherapy. MAIN OUTCOME MEASURES: The four endpoints included (1) poor final visual acuity (20/200 or worse), (2) enucleation, (3) local tumor recurrence, and (4) metastasis. The clinical data regarding patient features, tumor features, and radiation parameters were analyzed for their impact on the four main outcomes using Cox proportional hazards regression models. RESULTS: Using Kaplan-Meier estimates, final visual acuity was poor in 57% at 5 years and 89% at 10 years follow-up. Using multivariate analysis, the most important risk factors for poor visual acuity included retinal invasion by melanoma, increasing patient age, iodine 125 (I(125)) isotope, and <2 mm distance to the optic disc. Treatment-related complications at 5 years included proliferative retinopathy (25%), maculopathy (24%), papillopathy (22%), cataract (66%), neovascular glaucoma (21%), vitreous hemorrhage (23%), and scleral necrosis (7%). Enucleation was necessary in 24% at 5 years and 34% at 10 years follow-up. Using multivariate analysis, the risk factors for enucleation included left eye, peripheral tumor margin anterior rather than posterior to the equator, increasing tumor thickness, and ruthenium 106 (Ru(106)) isotope. Using Kaplan-Meier estimates, local tumor recurrence was found in 9% at 5 years and 13% at 10 years follow-up. Using multivariate analysis, risk factors for tumor recurrence included Ru(106) radioisotope and ciliary body involvement with tumor. Tumor-related metastases were found in 30% at 5 years and 55% at 10 years follow-up. Using multivariate analysis, risk factors for metastases included inferotemporal meridian, anterior extension of the tumor to the iris root, increasing tumor base, and posterior margin < 2 mm from the optic nerve. CONCLUSIONS: Plaque radiotherapy provided tumor control at 10 years in 87% of patients with selected large posterior uveal melanomas (>8 mm thick) that otherwise would have been managed with enucleation. The large intraocular mass and associated features and radiation complications led to poor visual acuity in most patients. At 10 years follow-up, enucleation was necessary in 34% of patients, and metastasis developed in 55% of patients.

Adult↗
PubMed2002

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.

Diagnosis, Differential↗
PubMed2002

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 degrees) involvement of the ciliary body, and 16 (70%) had incomplete (180-360 degrees) 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.

Adult↗
PubMed2002

Factors predictive of recurrence of retinal tumors, vitreous seeds, and subretinal seeds following chemoreduction for retinoblastoma.

OBJECTIVE: To identify the clinical features of eyes with retinoblastomas that predict the recurrence of retinal tumors, vitreous seeds, and subretinal seeds following treatment with chemoreduction. DESIGN: Prospective nonrandomized single-center clinical trial. SETTING: Ocular oncology service at Wills Eye Hospital of Thomas Jefferson University (Philadelphia, Pa) in conjunction with the division of oncology at Children's Hospital of Philadelphia. PARTICIPANTS: There were 158 eyes with 364 tumors in 103 consecutive patients with retinoblastoma managed with chemoreduction between June 1994 and August 1999. INTERVENTION: All patients received treatment for retinoblastoma with 6 cycles of chemoreduction using vincristine, etoposide, and carboplatin combined with focal treatment (cryotherapy, thermotherapy, or plaque radiotherapy) for each retinal tumor. MAIN OUTCOME MEASURES: The 3 main outcome measures included recurrence of retinal tumors, recurrence of vitreous seeds, and recurrence of subretinal seeds. The clinical features at the initial examination were analyzed for their association with the main outcome measures using a series of Cox proportional hazards regressions. RESULTS: All retinal tumors, vitreous seeds, and subretinal seeds showed an initial favorable response of regression during this treatment regimen. Using Kaplan-Meier estimates, at least 1 retinal tumor recurrence per eye was found in 37% of eyes at 1 year, 51% at 3 years, and no further increase at 5 years. By multivariate analysis, the only factor predictive of retinal tumor recurrence was the presence of tumor-associated subretinal seeds at the initial examination. Of the 54 eyes that had vitreous seeds at the initial examination, vitreous seed recurrence was found in 26% of eyes at 1 year, 46% at 3 years, and 50% at 5 years. By univariate analysis, the only factor predictive of vitreous seed recurrence was the presence of tumor-associated subretinal seeds at the initial examination. Of the 71 eyes that had subretinal seeds at the initial examination, subretinal seed recurrence was detected in 53% of eyes at 1 year, 62% at 3 years, and no further increase at 5 years. By multivariate analysis, factors predictive of subretinal seed recurrence included a tumor base greater than 15 mm and a patient age of 12 months or younger at diagnosis. There were no patients who developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms. CONCLUSIONS: Chemoreduction combined with focal therapy is effective for selected eyes with retinoblastomas. Eyes with subretinal seeds at initial examination are at particular risk for recurrence of retinal tumor and vitreous seeds. Younger patients with large tumors are at risk for recurrence of subretinal seeds. Retinal tumor and subretinal seed recurrence seems to manifest within 3 years of follow-up. Close follow-up of all patients treated with chemoreduction is warranted.

Antineoplastic Combined Chemotherapy Protocols↗