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Biomedical subjects

Jerry A Shields

Publications and source records attributed to Jerry A Shields.

At least 109 records · Page 6Linked to original sources
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

Combined plaque radiotherapy and transpupillary thermotherapy for choroidal melanoma: tumor control and treatment complications in 270 consecutive patients.

OBJECTIVE: To evaluate tumor control and treatment complications following plaque radiotherapy combined with transpupillary thermotherapy for choroidal melanoma. DESIGN: Prospective noncomparative interventional case series. INTERVENTION: All patients received treatment for choroidal melanoma using plaque radiotherapy followed by 3 sessions of transpupillary thermotherapy provided at plaque removal and at 4-month intervals. PARTICIPANTS: Two hundred seventy patients with newly diagnosed choroidal melanoma. MAIN OUTCOME MEASURES: The 2 main outcome measures included local tumor recurrence and treatment-related complications. The clinical data regarding patient features, tumor features, radiotherapy and thermotherapy parameters were analyzed for their effect on the 2 main outcomes using Cox proportional hazards regression models. RESULTS: Prior to treatment, the median base of the tumor was 11 mm (range, 4-21 mm) and the median thickness was 4 mm (range, 2-9 mm). Most tumors were located in the posterior pole with a median proximity of 2 mm to the foveola and 2 mm to the optic disc. The median radiotherapy dose to the tumor apex was 9000 rad. Transpupillary thermotherapy was applied in 3 sessions at 4-month intervals for a median of 700 mW. The tumor decreased in thickness to a median of 2.3 mm by 1 year and 2.1 mm by 2 years' follow-up with stable findings thereafter. Using Kaplan-Meier estimates, tumor recurrence was 2% at 2 years and 3% at 5 years. Risk factors for tumor recurrence included macular location of the tumor epicenter (P =.03), diffuse tumor configuration (P =.005), and tumor margin extending underneath the foveola (P =.001). Using Kaplan-Meier estimates, treatment-related complications at 5 years included maculopathy in 18% of the participants, papillopathy in 38%, macular retinal vascular obstruction in 18%, vitreous hemorrhage in 18%, rhegmatogenous retinal detachment in 2%, cataract in 6%, and neovascular glaucoma in 7%. Enucleation for radiation complications was necessary in 3 cases (1%). CONCLUSION: Plaque radiotherapy combined with transpupillary thermotherapy provides excellent local tumor control with only 3% recurrence at 5 years' follow-up.

Adolescent↗
PubMed2002

Progressive growth of an iris melanocytoma in a child.

PURPOSE: To document progressive growth of a benign iris melanocytoma. METHODS: Interventional case report. A 9-year-old male underwent removal of a pigmented iris tumor that had been documented photographically to double in size. RESULTS: Histopathologic sections revealed a deeply pigmented mass with cytologic features typical of a melanocytoma. CONCLUSION: Although iris melanocytoma is generally a stationary lesion, it can show progressive growth. Such enlargement does not necessarily imply malignant transformation of the lesion.

Child↗
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

Topical mitomycin C for extensive, recurrent conjunctival-corneal squamous cell carcinoma.

PURPOSE: To evaluate the efficacy of topical mitomycin C for extensive recurrent conjunctival and corneal squamous cell carcinoma (SCC). DESIGN: Interventional case series. METHODS: A prospective study was conducted in a single institution. Ten patients (ten eyes) with extensive recurrent conjunctival and corneal SCC were studied. The patients received topical mitomycin C 0.04% one drop four times daily in the eye with SCC. Treatment cycles were defined as 1 week using medication followed by 1 week without medication. Such treatment cycles were repeated until resolution of the conjunctival malignancy was clinically evident. The main outcome measures were tumor response and medication-related complications. RESULTS: Of the ten patients, the median age was 66 years (range 33-77 years). Before referral, the patients had undergone a median of two previous conjunctival tumor resections revealing the diagnosis of in situ SCC in three cases and locally invasive SCC in six cases. At presentation, the tumor involved the limbus and cornea in all ten eyes, forniceal conjunctiva in three eyes, and tarsal conjunctiva in one eye. The extensive tumor affected a median of 10 clock hours of limbal conjunctiva and 10 clock hours of cornea, with corneal epithelial invasion for a median of 50% (range 20%-100%) of its surface. Mitomycin C 0.04% four times daily was applied for a median of three cycles (range 1-4 cycles). Complete tumor regression was documented in all ten cases (100%). There was no recurrence over a mean follow up of 22 months (range 6-50 months). Mitomycin C caused moderate temporary local irritation and conjunctival erythema and chemosis, but no long-term intraocular or extraocular complications. CONCLUSIONS: Based on this small series, topical mitomycin C 0.04% appears to be a safe and effective therapy for conjunctival or corneal SCC, even when there is extensive recurrent tumor.

Administration, Topical↗
PubMed2002

Spontaneous appearance of an optic disk melanocytoma in an adult.

PURPOSE: To report a melanocytoma of the optic disk in an adult who had prior photographic documentation that the affected disk was entirely normal. METHODS: Clinical evaluation and fundus photography. RESULTS: An asymptomatic 45-year-old woman was found on routine ocular examination to have a typical melanocytoma in her right optic disk. Fundus photographs made 13 years earlier showed a normal right optic disk with no sign of melanocytoma or other pigmented lesion. CONCLUSIONS: Although optic disk melanocytoma is generally believed to be a congenital lesion, it can apparently develop spontaneously in adulthood.

Female↗
PubMed2002

Choroidal metastasis from medullary thyroid carcinoma in multiple endocrine neoplasia.

PURPOSE: To report choroidal metastasis from medullary thyroid carcinoma in a patient with multiple endocrine neoplasia (MEN) type 2b. DESIGN: Interventional case report. METHODS: Medullary thyroid carcinoma developed in a 20-year-old woman and led to the diagnosis of MEN 2b. She had mucosal neuromas on the lips and tongue and prominent corneal nerves in both eyes. Right choroidal metastasis developed when she was 36 years of age. RESULTS: The eye was treated with irradiation, but 9 months later the patient died of widespread metastases. CONCLUSIONS: In patients with MEN type 2b, choroidal metastasis can develop from medullary thyroid carcinoma. Although fundus abnormalities typically do not occur with MEN 2b, choroidal metastasis from medullary thyroid carcinoma is a potential fundus finding.

Adult↗
PubMed2002

Variability of three-dimensional ultrasonography for assessment of intraocular tumours.

BACKGROUND: Variability is introduced in three-dimensional (3-D) ultrasound assessments of intraocular tumours when multiple observers examine the same tumour or when multiple scans and measurements of the same tumour are carried out. We evaluated the magnitude and sources of variation associated with 3-D ultrasound measurements of choroidal tumour volume. METHODS: Three examiners performed 3-D ultrasonography of five choroidal tumours. Each examiner performed three scans of each tumour and then measured each scan three times to determine the volume. We determined the variation attributable to differences between examiners, scans and measurements by evaluating the contribution of each to the overall variation in tumour volume. A similar process was performed for tumour height for comparison purposes. RESULTS: The reliability of tumour volume measurements was found to be very high. The amount of overall variation, as defined by the coefficient of variation, was similar for tumour volume and tumour height and ranged from 4% to 17%. A total of 97% of the variation in tumour volume measurements and 84% of the variation in tumour height measurements was due to differences between tumours. Differences between examiners accounted for the most of the remaining variation (2% for volume and 7% for height). INTERPRETATION: Three-dimensional ultrasonography of intraocular tumours was highly reliable. Differences between examiners were the main source of variation.

Choroid Neoplasms↗
PubMed2002

Primary transpupillary thermotherapy for small choroidal melanoma in 256 consecutive cases: outcomes and limitations.

OBJECTIVE: The objective of this report was to evaluate ocular and systemic outcomes after primary transpupillary thermotherapy for choroidal melanoma and to identify the limitations of this treatment method. DESIGN: Noncomparative interventional case series. PARTICIPANTS: The participants included 256 patients with newly diagnosed choroidal melanoma. MAIN OUTCOME MEASURES: The outcome measures included local tumor recurrence and visual acuity. RESULTS: Before treatment, the mean tumor base was 7.1 mm, and mean tumor thickness was 2.7 mm. Overlying subretinal fluid was present in 215 cases (84%) and orange pigment on the tumor surface in 200 cases (78%). All tumors showed either photographic documentation of growth (114 cases; 45%) or substantial risk factors for growth (142 cases; 55%). After a mean of three treatment sessions, complete tumor control without recurrence was found in 232 cases (91%) and recurrence in 24 cases (9%). The mean time to recurrence was 22 months, and the mean recurrent tumor size was 3.8 mm base and 2.4 mm thick. Of the recurrent tumors, additional thermotherapy was successful in controlling 13 (5%), plaque radiotherapy in 8 (3%), and enucleation in 3 (1%). Using multivariable analysis, the risk factors for tumor recurrence included increasing number of thermotherapy sessions (reflecting less responsive tumor) (P = 0.0001) and optic disc overhung by tumor (P = 0.03). Kaplan Meier estimates revealed that 4% showed recurrence at 1 year, 12% at 2 years, and 22% at 3 years follow-up. When analyzing those 214 patients without multivariable risk factors for recurrence, Kaplan Meier estimates for recurrence were 2% at 1 year, 8% at 2 years, and 10% at 3 years. The visual acuity after treatment was 20/20 to 20/40 in 128 cases (50%), 20/50 to 20/100 in 47 (18%), and 20/200 or worse in 81 (32%). Using multivariable analysis, the most statistically significant factors at initial visit that were predictive of poor visual acuity (20/200 or worse) after treatment included documented tumor growth before treatment (P = 0.0001), mushroom tumor configuration (P = 0.002), initial symptom of blurred vision (P = 0.008), poor initial visual acuity (P = 0.005), superior quadrant tumor location (P = 0.03), underlying diabetes mellitus (P = 0.04), and optic disc overhung by tumor (P = 0.04). Tumor-related mortality occurred in two patients (1%), one of whom showed complete tumor regression to thermotherapy and the other with diffuse choroidal melanoma and local tumor margin recurrence. CONCLUSIONS: Transpupillary thermotherapy is an effective treatment for certain small choroidal melanomas. Appropriate tumor selection is critical to successful treatment. Patients with tumors abutting or overhanging the optic disc or those requiring more than three sessions for tumor control are more likely to develop ultimate tumor recurrence. Transpupillary thermotherapy can cause damaging effects to the retina, leading to visual loss shortly after treatment.

Adolescent↗
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

Treatment of retinal capillary hemangioma.

OBJECTIVE: To report on the methods of treatment and the visual outcome of eyes with retinal capillary hemangioma (RCH). DESIGN: Retrospective consecutive noncomparative case series. PARTICIPANTS: Data on 68 patients with RCH were reviewed for the methods of treatment and visual outcome. METHODS: Statistical analysis using cumulative multinomial logistic regression (generalized estimating equations) was performed to determine significant predictors of visual outcome. RESULTS: Among 68 patients with RCH with von Hippel-Lindau disease (n = 31) and without von Hippel-Lindau disease (n = 37), we identified 174 RCH in 86 eyes. The overall median age at diagnosis of RCH was 30.8 years (range, 2.8-73.8 years). The RCH was bilateral in 18 cases (26%), and a family history of von Hippel-Lindau disease was positive in 19 cases (28%). Twenty-nine of the tumors (17%) touched the optic disc and were classified as juxtapapillary RCH, and the remaining 145 (83%) were extrapapillary in location. Ninety-nine (58%) RCH were 1.5 mm or smaller in size. The RCH were initially managed by observation (46%), laser photocoagulation (25%), or cryotherapy (23%). Small RCH (< or =1.5 mm in size; 63 of 99; 64%) and those touching the optic disc (14 of 29; 48%) were more likely to be initially observed. Sixty-three (82%) of the 77 RCH that were initially observed remained stable for a median follow-up of 84 months. The remaining 14 progressed and were successfully controlled with laser photocoagulation or cryotherapy. Either laser photocoagulation or cryotherapy was effective as the sole method of treatment in controlling 74% (26 of 35) and 72% (28 of 39) of extrapapillary tumors, with a mean number of 1.2 and 1.1 sessions, respectively. In a multivariate model, the only variables that were significantly related to final vision of </= 20/400 were poor initial vision (P = 0.01, odds ratio [OR], 8.5; 95% confidence interval [CI], 1.7,42) and the presence of retinal/vitreous hemorrhage (P = 0.024, OR, 5.7; 95% CI, 1.3, 25.6). CONCLUSIONS: RCH can be safely observed initially in selected cases. Laser photocoagulation and cryotherapy are the mainstays of treatment in most cases. Early detection of RCH and treatment before the onset of severe visual loss is recommended.

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

Topical mitomycin-C for pagetoid invasion of the conjunctiva by eyelid sebaceous gland carcinoma.

PURPOSE: To evaluate the efficacy of topical mitomycin-C for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma. DESIGN: Prospective noncomparative consecutive interventional case series. INTERVENTION: All patients received topical 0.04% mitomycin-C four times daily for 1 week followed by 1 week off medication. The treatment cycles were repeated until resolution of the conjunctival malignancy was clinically evident. PARTICIPANTS: Four patients with histopathologically proven intraepithelial (pagetoid) invasion of the conjunctiva by sebaceous gland carcinoma were managed with this regimen. RESULTS: The mean patient age was 78 years. Before treatment, the main tumor site included the upper eyelid in two cases and the lower eyelid in two cases. Previous resection of the tumor had been performed elsewhere in three cases over the prior 6 years. At the time of our examination, map biopsies confirmed pagetoid invasion involving 25% to 90% of the conjunctival surface, with bulbar, forniceal, and tarsal conjunctival involvement in all four cases and corneal extension in one case. There was no evidence of deep tumor within the conjunctival stroma or tarsus in any case. After treatment, medication intolerance and early discontinuation occurred in one patient, and continued tumor progression was documented. Of the remaining three patients, chemotherapy was used for a mean of four cycles with complete resolution of the pagetoid invasion, confirmed histopathologically in two cases, and without recurrence in all three cases over 12 months (mean) follow-up. The medication caused moderate temporary local irritation but no serious intraocular or extraocular complications. CONCLUSIONS: Preliminary evidence suggests that topical mitomycin-C is effective treatment for pagetoid invasion of the conjunctiva by sebaceous gland carcinoma. Longer follow-up is necessary to assess the duration of tumor control.

Adenocarcinoma, Sebaceous↗
PubMed2002

Solitary retinal capillary hemangioma: lack of genetic evidence for von Hippel-Lindau disease.

PURPOSE: To report the results of genetic testing for von Hippel-Lindau (VHL) disease in patients presenting with solitary retinal capillary hemangioma (RCH). METHODS: Ten patients with solitary RCH, who were excluded clinically as having VHL disease, underwent genetic testing using a combination of Southern blot, conformation sensitive gel electrophoresis, and direct sequence analysis. The results of the genetic tests were used to refine the empiric risk for VHL disease using principles of probability. RESULTS: Genetic testing for VHL disease was negative for mutation in all patients. The negative results of the genetic tests diminished the empiric risk for VHL disease by 100-fold. CONCLUSIONS: Solitary RCH can occur sporadically or be associated with VHL disease. In addition to clinical evaluation, genetic testing should be considered to exclude VHL disease with a high level of certainty.

Adolescent↗
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↗