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Carol L Shields

Publications and source records attributed to Carol L Shields.

At least 37 records · Page 2Linked to original sources
PubMed2006

[Retinoblastoma update--management and classification (Part I)].

The aim of this paper is to present updated data on diagnosis, management and classification of retinoblastoma. The authors describe the growth patterns of retinoblastoma including the diffuse infiltrating type, the features of sponatneously regressed and sponatneously arrested retinoblastoma. In addition, retinoblastoma genetics including 13 q deletion syndrome, are discussed. Finally, they present the International Intraocular Retinoblastoma Classification (IIRC) and make recommendations regarding chemotherapy after enucleation based on histopathology findings.

Antineoplastic Agents↗
PubMed2006

[Retinoblastoma update--therapy (Part II)].

The aim of this paper is to present updated data on the treatment of retinoblastoma. The authors describe current management based on the International Intraocular Retinoblastoma Classification (IIRC). They discuss the different methods of treatment, including chemoreduction, subconjunctival carboplatin, transpupillar thermotherapy, cryotherapy, laser photocoagulation, episcleral plaque radiotherapy, external-beem radiotherapy and enucleation.

Antineoplastic Agents↗
PubMed2005

Long-term visual outcome following chemoreduction for retinoblastoma.

OBJECTIVE: To evaluate long-term visual outcome following chemoreduction. DESIGN: Interventional case series evaluating 54 eyes of 40 children with retinoblastoma successfully treated with chemoreduction, consisting of a combination of intravenous carboplatin, etoposide phosphate, and vincristine sulfate plus focal therapy without external beam radiotherapy or enucleation. All patients were followed up for at least 5 years. Patient and tumor data were analyzed for their effect on the main outcome measures (final visual acuities of 20/40 or better and of 20/200 or better) using univariate and multivariate regression models. Patients who failed chemoreduction were excluded. RESULTS: There were 4 eyes in Reese-Ellsworth group I, 7 in group II, 3 in group III, 15 in group IV, and 25 in group V. The mean distance from the posterior tumor margin to the optic disc was 2 mm, and from the tumor margin to the foveola it was 3 mm. After a mean follow-up of 68 months, 27 eyes (50%) had a final visual acuity of 20/40 or better, and 36 eyes (67%) had final visual acuity of 20/200 or better. Of 33 eyes with macular tumor, only 8 (24%) had a final visual acuity of 20/40 or better, and 15 (45%) had a final visual acuity of 20/200 or better. Of 21 eyes with extramacular tumor, 19 (90%) had a final visual acuity of 20/40 or better, and all (100%) had a final visual acuity of 20/200 or better. CONCLUSION: The clinical factors that predicted visual acuity of 20/40 or better were a tumor margin at least 3 mm from the foveola and optic disc and an absence of subretinal fluid.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Factors predictive of tumor growth, tumor decalcification, choroidal neovascularization, and visual outcome in 74 eyes with choroidal osteoma.

OBJECTIVE: To evaluate choroidal osteoma for tumor growth, tumor decalcification, related choroidal neovascularization, visual acuity loss, and poor visual acuity. DESIGN: Retrospective nonrandomized single-center case series. SETTING: Ocular Oncology Service at Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa. PARTICIPANTS: There were 74 eyes of 61 patients with choroidal osteoma evaluated between January 1, 1977, and January 1, 2003. MAIN OUTCOME MEASURES: The 5 outcome measures included tumor growth, tumor decalcification, related choroidal neovascularization, visual acuity loss of 3 or more Snellen lines, and poor visual acuity of 20/200 or worse. RESULTS: At 5 and 10 years, Kaplan-Meier analysis revealed tumor growth in 22% and 51% of eyes, tumor decalcification in 28% and 46% of eyes, choroidal neovascularization in 31% and 31% of eyes, visual acuity loss in 26% and 45% of eyes, and poor visual acuity in 45% and 56% of eyes, respectively. The clinical factor predictive of tumor growth was absent overlying retinal pigment epithelial alterations. The factor predictive of decalcification was irregular tumor surface. Of the 15 tumors that showed partial decalcification at the first visit, there was no further tumor growth in any case. Of the remaining 12 tumors that later developed decalcification, tumor growth, if it occurred, was along the margin opposite the decalcification. No tumor showed growth in the region of decalcification. Factors predictive of choroidal neovascularization included irregular tumor surface and subretinal hemorrhage. In 6 eyes that had both choroidal neovascularization and tumor decalcification, the neovascularization was detected prior to or at the same time as the decalcification. The factor predictive of visual acuity loss was presence of subretinal fluid whereas the factors predictive of poor visual acuity included symptoms and tumor decalcification. A comparison of eyes with subfoveal vs extrafoveal choroidal osteoma showed poor visual acuity in 15 (34%) of 44 eyes and 3 (10%) of 30 eyes, respectively. Eyes with decalcified choroidal osteomas manifested poor visual acuity in 13 (48%) of 27 eyes whereas those with nondecalcified tumors showed poor visual acuity in 5 (11%) of 47 eyes. CONCLUSIONS: Choroidal osteoma showed evidence of growth in 51% of eyes and decalcification in nearly 50% of eyes by 10 years. Tumors with any degree of decalcification at the initial visit showed no further growth. Overall, poor visual acuity of 20/200 or worse was found in 56% of eyes by 10 years, and decalcified subfoveal choroidal osteomas displayed a particularly poor visual prognosis.

Adolescent↗
PubMed2005

Macular retinoblastoma managed with chemoreduction: analysis of tumor control with or without adjuvant thermotherapy in 68 tumors.

OBJECTIVE: To evaluate the effectiveness of chemoreduction alone and chemoreduction with thermotherapy for macular retinoblastoma. DESIGN: Prospective, nonrandomized, single-center case series. SETTING: Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at the Children's Hospital of Philadelphia (Pa). PARTICIPANTS: There were 68 macular retinoblastomas in 62 eyes of 49 patients managed with chemoreduction from January 1995 through January 2003. Intervention All patients received 6 cycles of intravenous chemoreduction using vincristine, etoposide, and carboplatin. The patients were then treated according to 1 of 2 approaches: chemoreduction alone with no adjuvant focal therapy (group A) or chemoreduction combined with adjuvant foveal-sparing thermotherapy to each macular retinoblastoma (group B). Main Outcome Measure Tumor recurrence. RESULTS: Of the 68 tumors, 28 were in group A and 40 were in group B. A comparison of both groups revealed that the tumors were similar with regard to clinical features. The mean tumor basal dimension was 12.3 mm for group A and 12.1 mm for group B, and the mean tumor thickness was 6.8 mm for group A and 6.1 mm for group B. Tumors in group A occupied a mean of 71% of the macula, and those in group B occupied 74% of the macula. Following treatment, Kaplan-Meier estimates revealed that group A tumors showed recurrence in 25% by 1 year and 35% by 4 years whereas those in group B showed recurrence in 17% by 1 year and 17% by 4 years. All recurrences were treated with additional focal thermotherapy, cryotherapy, or plaque radiotherapy except for 1 that required external beam radiotherapy and 1 that required enucleation, both in group A. Univariate analysis revealed that predictors of tumor recurrence were intraretinal growth pattern (vs endophytic); small tumor basal dimension (less than 3 mm and occupying a smaller percentage of the macula); absence of subretinal fluid, subretinal seeds, and vitreous seeds; and chemoreduction response with less tumor calcification and tumor regression of type 0 (complete disappearance without a scar). By multivariate analysis, the most important factors predictive of tumor recurrence were smaller macular tumor size (judged by percentage of the macula occupied by the tumor), absence of subretinal or vitreous seeds, and unilateral disease. CONCLUSIONS: Treatment of macular retinoblastoma with chemoreduction plus adjuvant foveal-sparing thermotherapy provides tumor control of 83% by 4 years, and this is slightly more favorable than chemoreduction alone, which provides control of 65% by 4 years. Tumors most destined for recurrence are small tumors.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

New ophthalmic manifestations of branchio-oculo-facial syndrome.

PURPOSE: To report new ocular manifestations of branchio-oculo-facial (BOF) syndrome. DESIGN: Case report. METHODS: A 10-year-old girl with known BOF syndrome was referred because of a fundus lesion in her left eye. RESULTS: She had undergone excision of a left orbital dermoid cyst at age 18 months and a branchial cleft fistula from the right side of neck at age 4 years. Examination disclosed openings of sinus tracts on each side of the nose connecting the lacrimal sac to skin. In the right eye, an iris pigment epithelial cyst was confirmed with ultrasound biomicroscopy. In the left eye, there was a combined hamartoma of the retina and retina pigment epithelium. CONCLUSION: BOF syndrome can display mild to severe craniofacial, auricular, oral, and ophthalmic anomalies. In this case, the ophthalmic manifestations included lacrimal sac fistula, orbital dermoid cyst, iris pigment epithelial cyst, and combined hamartoma of the retina and retinal pigment epithelium.

Branchio-Oto-Renal Syndrome↗
PubMed2005

Sclerochoroidal calcification in a patient with classic Bartter's syndrome.

PURPOSE: To report sclerochoroidal calcification in a patient with classic Bartter's syndrome. DESIGN: Observational case report. METHODS: A 42-year-old woman with a 26-year history of classic Bartter's syndrome was found to have bilateral fundus tumors. The patient presented initially with quivering lips and hand stiffness at age 6 years but was not diagnosed until age 16 years. Treatment included magnesium and potassium supplementation and Amiloride therapy. RESULTS: On ocular examination, there were multifocal, yellow-white, geographic, solid choroidal lesions along the superior and inferior retinal vascular arcades in both eyes. Ultrasonography showed echogenic, placoid calcified lesions at the level of the sclera and choroid, consistent with bilateral sclerochoroidal calcification. CONCLUSIONS: Sclerochoroidal calcification can be associated with classic Bartter's syndrome.

Adult↗
PubMed2005

Iris melanocytoma: clinical features and natural course in 47 cases.

PURPOSE: To describe the clinical features, natural course, management and histopathologic features of iris melanocytoma. DESIGN: Single-center retrospective case series. METHODS PATIENT POPULATION: Forty-seven consecutive patients (47 eyes) with iris melanocytoma. INTERVENTION PROCEDURE: Data regarding patient and tumor features were analyzed for their impact on the main outcome measures using univariate and multivariate regression models. Kaplan-Meier estimates were used to analyze the main outcomes as a function of time. MAIN OUTCOME MEASURES: Increased intraocular pressure (IOP), tumor seeding, and tumor growth. RESULTS: Associated findings at initial presentation included iris stromal seeds in 20 patients (43%), and anterior chamber angle seeds in 12 (26%). Intrinsic vascularization and sector cataract were not seen in any eyes. The management at presentation included observation in 39 patients (83%), tumor removal by sector iridectomy/iridocyclectomy in 7 (15%), and enucleation for blind painful eye with secondary increased IOP in 1 (2%). The diagnosis was confirmed by histopathologic examination in 11 patients (23%). The mean follow-up was 58 months. Using Kaplan-Meier estimates, clinical evidence of growth was observed in 23% at 5 years, 48% at 10 years, and 74% at 15 years. New tumor seeds developed in 34% at 5 years, 63% at 10 years, and 75% at 15 years. Increased IOP was observed in 11% at 5 years, 11% at 10 years, and 55% at 15 years. CONCLUSIONS: Iris melanocytoma represented only 3% of all iris nevi. Related iris stromal and anterior chamber angle seeds were common, and secondary glaucoma occurred in 11% at 5 years. Growth was observed in 23% at 5 years but no malignant transformation was found.

Adolescent↗
PubMed2005

Total blindness from presumed optic nerve melanocytoma.

PURPOSE: To describe total blindness secondary to presumed optic nerve melanocytoma. DESIGN: Interventional case report. METHODS: A 12-year-old African-American girl, who developed loss of light perception secondary to a pigmented lesion of the optic disk, underwent transvitreal fine-needle aspiration biopsy (FNAB) of the lesion. RESULTS: Cytopathology revealed cells with features of melanocytoma and melanophages but no cells compatible with melanoma. The patient is being followed without treatment. CONCLUSIONS: Optic nerve melanocytoma can cause total blindness in the affected eye. Although it has limitations, FNAB can assist in diagnosis and management of melanocytoma with visual loss.

Blindness↗
PubMed2005

Cavitary changes in retinoblastoma: relationship to chemoresistance.

OBJECTIVE: To describe the clinical features and outcome of treatment of retinoblastomas presenting with ophthalmoscopically visible cavities. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Sixteen eyes of 15 patients harboring retinoblastomas with ophthalmoscopically visible cavities treated between February 1995 and July 2003. METHODS: Patient demographics, eye and tumor characteristics, and response to treatment were recorded. MAIN OUTCOME MEASURES: Decrease in tumor size and tumor recurrence. RESULTS: The mean patient age at diagnosis was 20 months. The mean number of retinoblastomas per eye was 2 (range, 1-6). Associated subretinal fluid was graded as absent in 5 eyes, minimal in 6 eyes, mild in 4 eyes, and severe in 1 eye. None of the tumors had vitreous seeds, and 5 (31%) had subretinal seeds. Ophthalmoscopically visible calcification occupied a mean of only 3% of the tumor (median, 0%; range, 0%-30%). Intratumoral cavities were characteristically visible in the superficial portion of the tumor and had a mean diameter of 2.0 mm (median, 1.5; range, 0.5-5.0). The mean number of cavities per tumor was 2 (median, 1; range, 1-5). Three tumors were treated with enucleation, 1 with observation, and 12 with chemoreduction. Two months after chemoreduction, the mean reduction in tumor base was 19% (median, 16%; range, 0%-42%), and the mean reduction in tumor thickness was 18% (median, 12%; range, 0%-59%). During a mean follow-up of 32 months (median, 23; range, 12-58), none of the cavitary retinoblastomas recurred or gave rise to vitreous or new subretinal seeds. CONCLUSION: Retinoblastomas containing ophthalmoscopically visible cavitary spaces do not show a substantial decrease in size after chemotherapy. Cavitary changes in retinoblastoma may be a sign that the tumor is well differentiated and may impart a better prognosis.

Antineoplastic Agents↗
PubMed2005

Intermittent and chronic ultraviolet light exposure and uveal melanoma: a meta-analysis.

OBJECTIVE: To examine the association between ultraviolet light exposure and uveal melanoma. DESIGN: Meta-analysis. METHODS: A review of 133 published reports on risk factors for uveal melanoma revealed 12 studies that provided sufficient information to calculate odds ratios (ORs) and standard errors for ultraviolet light exposure factors. Data from these studies were extracted and categorized into intermittent ultraviolet exposure factors (welding, outdoor leisure, photokeratitis) and chronic ultraviolet exposure factors (occupational sunlight exposure, birth latitude, lifetime ultraviolet exposure index). Summary statistics were calculated for all risk factors reported by > or =4 independent studies. MAIN EXPOSURE MEASURES: Welding, outdoor leisure, photokeratitis, occupational sunlight exposure, birth latitude, and lifetime ultraviolet exposure index. RESULTS: For intermittent ultraviolet exposure, welding was found to be a significant risk factor (5 studies, 1137 cases; OR, 2.05 [95% confidence interval [CI], 1.20-3.51]). Outdoor leisure was found to be nonsignificant (4 studies, 1332 cases; OR, 0.86 [95% CI, 0.71-1.04]). Photokeratitis conferred susceptibility in 3 reports studying this variable, but there were too few studies to validate meta-analyses. For chronic ultraviolet exposure, meta-analysis found occupational sunlight exposure to be a borderline nonsignificant risk factor for development of uveal melanoma (4 studies, 572 cases; OR, 1.37 [95% CI, 0.96-1.96]). Latitude of birth was found to be nonsignificant (5 studies, 1765 cases; OR, 1.08 [95% CI, 0.67-1.74]). CONCLUSION: This meta-analysis yielded inconsistent results associating ultraviolet light with development of uveal melanoma. There was evidence implicating welding as a possible risk factor for uveal melanoma.

Environmental Exposure↗
PubMed2005

Primary ductal adenocarcinoma of the lacrimal gland.

OBJECTIVE: To report the case of a 59-year-old man with a right lacrimal gland mass, subsequently diagnosed as primary ductal adenocarcinoma of the lacrimal gland. DESIGN: Interventional case report. METHODS: We report the clinical presentation, histopathologic and immunohistochemical features, and treatment of a patient with primary ductal adenocarcinoma of the lacrimal gland. The current literature regarding this uncommon neoplasm also is reviewed. RESULTS: Excisional biopsy revealed primary ductal adenocarcinoma of the lacrimal gland. The patient subsequently underwent extensive local resection of the tumor and is awaiting radiotherapy to the orbit and neck. A literature search revealed only 3 prior reports of primary ductal adenocarcinoma of the lacrimal gland. CONCLUSIONS: Although uncommon, primary ductal adenocarcinoma of the lacrimal gland displays characteristic histologic and immunohistochemical findings that allow its distinction from the other primary lacrimal gland adenocarcinomas. Awareness and recognition of this rare malignancy may aid in further delineation of its biologic behavior, management, and prognosis.

Carcinoma, Ductal↗