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Biomedical subjects

Jerry A Shields

Publications and source records attributed to Jerry A Shields.

At least 55 records · Page 3Linked to original sources
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↗
PubMed2005

Pseudomelanomas of the posterior uveal tract: the 2006 Taylor R. Smith Lecture.

PURPOSE: To determine the types and frequency of lesions that clinically simulate choroidal or ciliary body melanoma (posterior uveal melanoma; PUM). PATIENTS AND METHODS: A review was conducted on cases of patients referred to the ocular oncology service from October 1978 through September 2003 with the diagnosis of possible PUM but who were subsequently diagnosed by the authors to have a simulating lesion rather than PUM. The type and percent of pseudomelanomas were tabulated and compared with findings of a similar study from our service on data collected before 1978. RESULTS: There were approximately 12,000 patients referred because of a lesion believed to be a PUM during the 25 years included in the data collection. Of these patients, 1,739 (14%) were found to have a simulating condition. There were 54 different conditions that simulated melanoma. The most frequent condition was choroidal nevus, accounting for 851 cases (49%) of the pseudomelanomas. This was followed by peripheral exudative hemorrhagic chorioretinopathy (139 cases; 8%), congenital hypertrophy of the retinal pigment epithelium (108 cases; 6%), hemorrhagic detachment of the retina or pigment epithelium (86 cases; 5%), circumscribed choroidal hemangioma (79 cases; 5%) and age-related macular degeneration (76 cases; 4%). Compared with the 1980 report, the rate of pseudomelanomas diagnosed as choroidal nevus increased from 26% to 49%. CONCLUSION: A variety of lesions can simulate PUM. Suspicious choroidal nevus is still the lesion most difficult to differentiate from PUM. Most other pseudomelanomas account for a lower percent compared with findings from the prior study, suggesting that clinicians are now more familiar with the other pseudomelanomas and less likely to refer them to rule out PUM.

Adolescent↗
PubMed2005

Chemoembolization of the hepatic artery with BCNU for metastatic uveal melanoma: results of a phase II study.

Uveal melanoma is the most common primary intraocular malignancy in adults and the liver is the most common site for systemic metastases. We conducted a phase II clinical trial for patients with hepatic metastases from uveal melanoma using chemoembolization of the hepatic artery with 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) dissolved in ethiodized oil. Gelatin sponge particles were used as a transiently occlusive agent. The responses in hepatic metastases, overall survival, time to progression and side-effects related to chemoembolization were evaluated. Thirty patients were enrolled. Twenty-four patients completed at least one treatment to all targeted liver metastases and were evaluable for hepatic response. Eighteen of these 24 patients experienced regression or stabilization of hepatic metastases for at least 6 weeks (one complete response in hepatic metastases; four partial responses; 13 stable disease). One of the 13 patients with stable disease was rendered free of disease by surgical removal of metastases after chemoembolization (surgical complete response). The overall response rates (complete and partial responses) for intention-to-treat patients and for patients who were evaluable for response were 16.7 and 20.4%, respectively. The median overall survival of the entire intention-to-treat group of patients was 5.2 months (range, 0.1-27.6 months), for patients with complete or partial response in hepatic metastases 21.9 months (range, 7.4-27.6 months), for patients with stable disease 8.7 months (range, 2.9-14.4 months) and for patients with progressive disease 3.3 months (range, 1.6-5.6 months). Importantly, 13 of the 18 patients who achieved complete response, partial response or stable disease subsequently developed progression of extrahepatic metastases with control of hepatic metastases. Chemoembolization with BCNU is a useful palliative treatment for the control of hepatic metastases in uveal melanoma patients. However, progression in extrahepatic sites after stabilization of hepatic metastases requires further improvement in the therapeutic approach to this disease.

Adult↗
PubMed2005

Conjunctival mascaroma masquerading as melanoma.

PURPOSE: To describe a conjunctival mass composed of keratin with mascara deposition that clinically simulated a melanoma. METHODS: A 50-year-old woman, who used mascara for 20 years, was referred for a conjunctival mass that was suspected to be melanoma. Excisional biopsy was performed, and the lesion was studied histopathologically. RESULTS: The lesion consisted of conjunctival epithelium with a keratin plaque that contained multiple dark particles that exhibited birefringence with polarized light. The final diagnosis was conjunctival hyperkeratosis containing foreign bodies compatible with mascara deposition. The patient continues to use mascara, and there is no recurrence after 12 months. CONCLUSIONS: Deposition of mascara can assume tumorous proportions and simulate a conjunctival melanoma.

Conjunctival Diseases↗
PubMed2005

Review of optical coherence tomography for intraocular tumors.

PURPOSE OF REVIEW: Optical coherence tomography has assumed an important role in the management of numerous ocular conditions. With regard to ocular oncology, optical coherence tomography can illustrate retinal changes overlying choroidal tumors. Some of these features include photoreceptor loss, intraretinal edema, and retinal thinning overlying choroidal nevus; fresh subretinal fluid with preservation of photoreceptors overlying choroidal melanoma; and intraretinal edema, retinoschisis, and retinal thinning overlying irradiated choroidal melanoma. RECENT FINDINGS: The optical coherence tomography features of tumors of the retinal pigment epithelium include typical findings of peaked vitreoretinal traction and retinal disorganization with combined hamartoma of the retina and retinal pigment epithelium, full-thickness retinal shadowing with congenital simple hamartoma, and photoreceptor loss and retinal thinning overlying congenital hypertrophy of the retinal pigment epithelium. SUMMARY: Optical coherence tomography of retinal tumors, such as retinoblastoma and astrocytic hamartoma, reveals full-thickness replacement of the retinal anatomic layers with the tumor and shadowing corresponding to the intralesional calcification. For all intraocular tumors, optical coherence tomography provides valuable information regarding the status of the retina and the retinal pigment epithelium and can be useful in ascertaining reasons for visual loss.

Choroid Neoplasms↗
PubMed2005

Expanding MIRAgel scleral buckle simulating an orbital tumor in four cases.

PURPOSE: To describe four patients with an enlarging orbital mass from a swollen MIRAgel scleral buckle that simulated an orbital neoplasm. METHODS: In a retrospective, single-center case series at the Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University, 4 eyes of 4 patients were referred for evaluation and treatment of a suspected orbital tumor. RESULTS: The initial presenting features were orbital mass (case 1), strabismus (case 2), and conjunctival mass with orbital extension (cases 3 and 4). Each patient vaguely recalled previous uncomplicated retinal detachment surgery 12 to 20 years earlier. Confirmation of the buckling implant material was made with the retina surgeon in 3 cases. A nontender, forniceal conjunctival mass, deep to the Tenon fascia and appearing as a translucent firm elevation was seen in all 4 cases. Axial CT (case 1) revealed a circumscribed anterior temporal orbital mass, believed to be a large inclusion cyst, 4 times thicker than the nasal scleral buckle. Ocular ultrasonography depicted an echolucent mass in the episcleral region (cases 3 and 4) that was 2 times thicker than the nasal scleral buckle (case 3). Excision was attempted in case 1, but only piecemeal removal was achieved, leading to extensive postoperative inflammation and decreased vision. The other 3 cases were followed conservatively without excision because they were each recognized to be a swollen MIRAgel implant and not an orbital tumor. CONCLUSIONS: MIRAgel scleral buckle material can greatly enlarge over a period of 10 years and simulate an orbital tumor or orbital cyst. Patients often do not recall details of the retinal surgery. Caution is advised regarding excision of this material because it is friable and can lead to extensive postoperative inflammation.

Adult↗
PubMed2005

Conjunctival epithelial involvement by eyelid sebaceous carcinoma. The 2003 J. Howard Stokes lecture.

PURPOSE: To determine incidence and distribution of conjunctival epithelial involvement by eyelid sebaceous carcinoma and to make recommendations regarding its management. METHODS: The medical records were reviewed retrospectively on patients with histopathologically confirmed sebaceous carcinoma of the eyelids managed at the Oncology Service at Wills Eye Hospital. Those tumors with involvement of the conjunctival epithelium were identified and selected for further study. The incidence and distribution of epithelial involvement was determined, based on histopathology of resected tumors and map biopsies. The incidence of metastasis and tumor-related mortality was determined. Based on these findings and personal surgical experience, recommendations are made regarding management of eyelid sebaceous carcinoma with involvement of the conjunctival epithelium. RESULTS: Of 60 patients with sebaceous carcinoma, epithelial involvement of the conjunctiva was identified in 28 (47%). Of the 28 cases, the neoplasm affected the following sites: superior tarsal and fornical conjunctiva in 28 (100%), inferior tarsal conjunctiva in 19 (68%), inferior fornical conjunctiva in 18 (64%), superior bulbar conjunctiva in 19 (68%), and inferior bulbar conjunctiva in 16 (57%). The caruncle was involved in 15 (54%) and the cornea in 11 (39%). Metastasis occurred in 3 of the 28 cases (11%). Map biopsies, combined with cryotherapy, topical chemotherapy, local surgical resection, and orbital exenteration, were used to achieve local control. CONCLUSIONS: Eyelid sebaceous carcinoma was found to exhibit epithelial involvement of the conjunctiva in 47% of cases, predominantly in the superior tarsal and fornical conjunctiva and less often in the inferior tarsal conjunctiva, caruncle, and cornea. Treatment of this condition is challenging, and map biopsy, cryotherapy, topical chemotherapy, and newer surgical methods are being used more often by our group.

Adenocarcinoma, Sebaceous↗
PubMed2004

Conjunctival nevi: clinical features and natural course in 410 consecutive patients.

OBJECTIVES: To describe the clinical features of a conjunctival nevus and to evaluate the lesion for changes in color and size over time. DESIGN: Retrospective, observational, noncomparative case series. PARTICIPANTS: Four hundred ten consecutive patients with conjunctival nevi. MAIN OUTCOME MEASURES: The 2 main outcome measures were changes in tumor color and size. RESULTS: Of the 410 patients, 365 (about 89%) were white, 23 (about 6%) were African American, 8 (2%) were Asian, 8 (2%) were Indian, and 6 (1%) were Hispanic. The iris color was brown in 55% (229/418), blue in 20% (85/418), green in 20% (83/418), and not indicated in 5% (21/418). The nevus was brown in 65%, tan in 19%, and completely nonpigmented in 16%. The anatomical location of the nevus was the bulbar conjunctiva (302 eyes, 72%), caruncle (61 eyes, 15%), plica semilunaris (44 eyes, 11%), fornix (6 eyes, 1%), tarsus (3 eyes, 1%), and cornea (2 eyes, <1%). The bulbar conjunctival lesions most commonly abutted the corneoscleral limbus. The nevus quadrant was temporal (190 eyes, 46%), nasal (184 eyes, 44%), superior (23 eyes, 6%), and inferior (21 eyes, 5%). Additional features included intralesional cysts (65%), feeder vessels (33%), and visible intrinsic vessels (38%). Cysts were clinically detected in 70% of histopathologically confirmed compound nevi, 58% of the subepithelial nevi, 40% of the junctional nevi, and 0% of the blue nevi. Of the 149 patients who returned for periodic observation for a mean of 11 years, the lesion color gradually became darker in 5% (7 patients), lighter in 8% (12 patients), and was stable in 87% (130 patients). The lesion size was larger in 7% (10 patients), appeared smaller in 1% (1 patient), and was stable in 92% (137 patients). There were 3 patients who developed malignant melanoma from a preexisting compound nevus (2 cases) or blue nevus (1 case) over a mean interval of 7 years. CONCLUSIONS: Conjunctival nevus is a benign tumor most often located at the nasal or temporal limbus and rarely in the fornix, tarsus, or cornea. Over time, a change in tumor color was detected in 13% (20/149) and a change in tumor size was detected in 8% (12/149).

Adolescent↗
PubMed2004

Practical approach to management of retinoblastoma.

OBJECTIVE: To present a simplified approach to management of retinoblastoma using basic clinical features. DESIGN: In a prospective, nonrandomized, single-center clinical trial, 158 eyes of 103 patients with retinoblastoma were managed with 6 cycles of chemoreduction (vincristine sulfate, etoposide, and carboplatin). The eyes were classified according to the Reese-Ellsworth classification and were also grouped on the basis of clinical features as follows: group 1, tumor only; group 2, tumor plus subretinal fluid; group 3, tumor plus focal seeds (3a, focal subretinal seeds; 3b, focal vitreous seeds); group 4, tumor plus diffuse seeds (4a, diffuse subretinal seeds; 4b, diffuse vitreous seeds); and group 5, neovascular glaucoma or invasive retinoblastoma. MAIN OUTCOME MEASURE: Treatment success (avoidance of enucleation and external beam radiotherapy). RESULTS: According to the Reese-Ellsworth classification, chemoreduction was successful in 100% of group Ia, 100% of group Ib, 86% of group IIa, 100% of group IIb, 91% of group IIIa, 100% of group IIIb, 50% of group IVa, 77% of group IVb, 50% of group Va, and 27% of group Vb. There was erratic correlation of the Reese-Ellsworth classification with treatment success. In contrast, the simplified grouping system displayed a smooth, nonerratic correlation for treatment success, with 100% success for group 1, 91% for group 2, 59% for group 3, and 12% for group 4 (group 5 always managed by primary enucleation). When all 6 subcategory groups were analyzed, there was consistent correlation for treatment success of 100% for group 1, 91% for group 2, 68% for group 3a, 54% for group 3b, 17% for group 4a, and 11% for group 4b. CONCLUSION: This practical approach to retinoblastoma using basic clinical features is predictive of treatment success for eyes in which modern conservative therapy for retinoblastoma is used.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Chemoreduction for retinoblastoma. Analysis of tumor control and risks for recurrence in 457 tumors.

PURPOSE: To evaluate retinoblastoma control following chemoreduction. DESIGN: Interventional case series. METHODS: Prospective. SETTING: Single center trial. PATIENT POPULATION: 457 retinoblastomas in 193 eyes of 125 patients. Nonrandomized, noncomparative study. INTERVENTION: All patients received intravenous vincristine, etoposide, and carboplatin,. The tumors were managed with chemoreduction alone (group W) or chemoreduction combined with thermotherapy (group X), cryotherapy (group Y), or both thermotherapy and cryotherapy (group Z). MAIN OUTCOME MEASURE: Tumor recurrence in each treatment group. RESULTS: Of 457 retinoblastomas, 63 (14%) were in group W, 256 (56%) in group X, 127 (28%) in group Y, and 11 (2%) in group Z. The tumor was located in the macula in 33 (52%) of group W, 109 (43%) of group X, 3 (2%) of group Y, and 9 (1%) of group Z. The mean tumor thickness at initial examination was 7 mm for group W, 4 mm for group X, 2 mm for group Y, and 3 mm for group Z. Using Kaplan-Meier estimates, recurrence of the individual retinoblastoma at 7 years was found in 45% of group W and 18% for combined groups X, Y, and Z. Risk factors predictive of tumor recurrence by multivariate analysis included macular tumor location for all groups and additionally female gender for group W and increasing tumor thickness for groups X, Y, and Z. CONCLUSIONS: Chemoreduction alone or combined with cryotherapy or thermotherapy is effective for treatment of retinoblastoma, but tumor recurrence rate is highest for those located in the macula and those with greater thickness.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1.

OBJECTIVE: To determine the incidence of specific orbital tumors based on patients referred to an ocular oncology center. DESIGN: Retrospective, observational case series. PARTICIPANTS: A total of 1264 consecutive patients referred to an ocular oncology service because of space-occupying orbital lesions. METHODS: A retrospective chart review was carried out for 1264 consecutive patients referred for a suspected orbital mass over a 30-year period. The lesions were grouped into general categories, as shown in "Results." The specific diagnosis in each case was based on clinical findings, computed tomography scan results, magnetic resonance imaging results, and histopathologic analysis results, when available. The number and percentage of benign and malignant tumors per age group also was determined. MAIN OUTCOME MEASURES: The incidence of orbital tumors and pseudotumors. RESULTS: Among 1264 consecutive patients, the number and percentage of lesions in each general category were as follows: cystic, 70 cases (6%); vasculogenic, 213 cases (17%); peripheral nerve lesions, 23 (2%); optic nerve and meningeal tumors, 105 (8%); fibrocytic lesions, 13 (1%); osseous and fibro-osseous tumors, 21 (2%); cartilaginous lesions, 1 (<1%); lipocytic and myxoid lesions, 64 (5%); myogenic tumors, 36 (3%); lacrimal gland lesions, 114 (9%); primary melanocytic lesions, 11 (<1%); metastatic tumors, 91 (7%); lymphoma and leukemia lesions, 130 (10%); secondary orbital tumors, 142 (11%); histiocytic lesions, 17 (1%); thyroid-related orbitopathy, 67 cases (5%); other inflammatory lesions, 133 cases (11%); and miscellaneous other lesions, 13 (1%). The most common diagnoses were: lymphoid tumor (139 cases;11%), idiopathic orbital inflammation (135 cases; 11%), cavernous hemangioma (77 cases; 6%), lymphangioma (54 cases; 4%), meningioma (53 cases; 4%), optic nerve glioma (48 cases; 4%), metastatic breast cancer (44 cases;4%), orbital extension of uveal melanoma (41 cases; 3%), capillary hemangioma (36 cases;3%), rhabdomyosarcoma (35 cases; 3%), dermolipoma (31 cases; 3%), herniated orbital fat (30 cases; 2%), dermoid cyst (26 cases; 2%), varix (26 cases; 2%), dacryops (19 cases; 2%), and other less common lesions. Of the 1264 lesions, 810 (64%) were benign and 454 (36%) were malignant. The percentage of malignant lesions was 20% in children (age range, 0-18 years), 27% in young adults and middle-aged patients (age range, 19-59 years), and 58% in older patents (age range, 60-92 years). Rhabdomyosarcoma was the most common malignancy in children, representing 3% of all orbital masses, and lymphoma was the most common malignancy in older patients, representing 10% of cases. CONCLUSIONS: A variety of tumors and pseudotumors can involve the orbit. In this series of 1264 lesions, 64% were benign and 36% were malignant. The percentage of malignant tumors increased with age, with malignancies being common in older patients because of the higher incidence of lymphoma and metastasis in the elderly.

Adolescent↗
PubMed2004

Progressive enlargement of acquired retinal astrocytoma in 2 cases.

PURPOSE: To document 2 cases of progressively enlarging retinal astrocytoma that caused exudative retinal detachment, vitreous hemorrhage, and tumor seeding, simulating retinoblastoma in 1 case and choroidal melanoma in the other. DESIGN: Interventional case reports. PARTICIPANTS: Two patients. METHODS: Two patients with visual loss from an atypical, enlarging amelanotic retinal mass were evaluated. The first patient, a 14 year-old otherwise healthy girl, had a noncalcified gelatinous retinal mass with prepapillary involvement and surrounding retinal traction, as well as overlying vitreous hemorrhage and vitreous seeds. Over 37 months, the tumor grew in basal dimension from 4 mm to 10 mm. Retinal astrocytoma was suspected, but the presence of tumor enlargement and vitreous seeding raised concern for possible retinoblastoma. Fine-needle aspiration biopsy was performed. The second patient, a 33-year-old woman, had an amelanotic mass develop in the macula of her amblyopic left eye. Over 2 years, the mass enlarged to 9.5 mm in basal dimension and 6.3 mm in thickness, and total serous retinal detachment developed. Choroidal melanoma with retinal invasion was clinically suspected. Enucleation was performed. RESULTS: In patient 1, cytologic examination revealed bland spindle cells with fibrillar cytoplasm consistent with an astrocytic tumor of the retina. Enucleation was subsequently performed because of continued tumor growth, progressive retinal detachment, and visual loss. Histopathologic examination confirmed astrocytoma of the retina and optic disc. In patient 2, enucleation revealed astrocytoma of the retina and optic disc with total retinal detachment. There was no evidence of systemic tuberous sclerosis in either patient. CONCLUSIONS: Retinal astrocytoma can show progressive enlargement, retinal detachment, and vitreous seeding, findings that can mislead the clinician toward the diagnosis of retinoblastoma or choroidal melanoma.

Adolescent↗
PubMed2004

Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors.

OBJECTIVE: To report the spectrum and frequency of melanocytic and nonmelanocytic conjunctival tumors in an ocular oncology practice. DESIGN: Retrospective noninterventional case series. PARTICIPANTS: One thousand six hundred forty-three consecutive patients with a conjunctival mass evaluated at an ocular oncology department. METHODS: A chart review was conducted to obtain the clinical features of the patient and tumor and to tabulate and categorize the diagnoses. MAIN OUTCOME MEASURES: Tumor diagnosis overall and relative to patient age, race, and gender and relative to tumor location and laterality. RESULTS: In 1643 consecutive patients, the tumor was classified as melanocytic in 872 cases (53%) and nonmelanocytic in 771 cases (47%). The nonmelanocytic categories included congenital choristomatous (n = 40 [2%]), epithelial (n = 219 [13%]), vascular (n = 63 [4%]), fibrous (n = 7 [<1%]), neural (n = 1 [<1%]), xanthomatous (n = 1 [<1%]), myxomatous (n = 1 [<1%]), lipomatous (n = 23 [1%]), lacrimal gland origin (n = 12 [<1%]), lymphoid (n = 128 [8%]), leukemic (n = 3 [<1%]), metastatic (n = 13 [<1%]), secondary (n = 54 [3%]) tumors, and non-neoplastic lesions simulating a tumor (n = 206 [13%]). Of the 872 melanocytic lesions, the specific tumor diagnosis was nevus in 454 cases (52%), melanoma in 215 (25%), and primary acquired melanosis in 180 (21%). Patients with choristomatous, vascular, fibrous, xanthomatous, and myxomatous tumors presented at a mean age of <40 years, and those with malignant epithelial, lipomatous, leukemic, and secondary tumors presented at a mean age of >60. Of the 219 patients with epithelial tumors, 80% occurred in males, whereas the incidence of melanocytic lesions was equal in males and females. African-American patients represented only 7% of epithelial tumors, <1% of melanomas, and 8% of lymphoid tumors. CONCLUSION: Conjunctival tumors were of melanocytic origin in 53% of cases and nonmelanocytic origin in 47%. Overall, melanocytic tumors, epithelial tumors, and lymphoid tumors accounted for 74% of all cases. These tumors were far more common in Caucasian patients, and epithelial tumors were found more frequently in men.

Adolescent↗
PubMed2004

Melanocytoma of optic disc in 115 cases: the 2004 Samuel Johnson Memorial Lecture, part 1.

PURPOSE: To report clinical variations and the natural course of melanocytoma of the optic disc. PATIENTS AND METHODS: A retrospective study was conducted on a consecutive series of patients with melanocytoma of the optic disc who were evaluated at the Ocular Oncology Service at Wills Eye Hospital. Demographic information and variations in appearance and size of the tumor and local ocular complications were noted. Frequency of tumor growth and malignant transformation was recorded. Clinical factors, including patient data and tumor features, were analyzed for their impact on visual acuity loss of 2 or more Snellen lines and the tumor growth, using Cox proportional hazards regression models. Kaplan-Meier survival estimates of probability of visual acuity loss of 2 or more Snellen lines and probability of tumor growth were performed as a function of time from the initial examination. MAIN OUTCOME MEASURES: Visual acuity loss of 2 or more Snellen lines, tumor growth, and malignant transformation of the tumor. RESULTS: There were 115 patients (116 eyes) with melanocytoma of the optic disc. The mean age at diagnosis was 50 years; 38% of patients were male and 62% of patients were female; 65% of patients were white, 29% of patients were African American, and 6% of patients were Asian, Hispanic, Indian, or Arabic. The lesion was unilateral in 99% of patients. Visual symptoms that seemed to be related to the melanocytoma were present in 24% of patients, and an afferent pupillary defect was noted in 9% of patients. Associated ocular abnormalities included ocular melanocytosis (8%), racial melanosis (7%), optic nerve hypoplasia (2%), and retinitis pigmentosa (1%). The melanocytoma was dark brown to black in 100% of patients. The mean tumor diameter was 2 mm and the mean thickness was 1 mm. Associated findings included a choroidal component (54%), retinal component (30%), optic disc edema (25%), retinal edema (16%), localized subretinal fluid (14%), retinal exudation (12%), retinal hemorrhage (5%), vitreous seeds (4%), and retinal vein obstruction (3%). Fluorescein and indocyanine green angiography typically showed persistent hypofluorescence of the lesion. Using Kaplan-Meier survival curves, related visual loss occurred in 18% of patients by 10 years, and minor tumor enlargement occurred in 11% of patients by 5 years and in 32% of patients by 10 years. Malignant transformation was documented in 2 patients (2%). CONCLUSIONS: Although melanocytoma of the optic disc generally is considered to be a benign, stationary lesion, it can produce several local complications, can cause visual loss, can grow slowly, and, rarely, can undergo malignant transformation into melanoma. Patients with optic disc melanocytoma should undergo periodic ocular examination.

Adolescent↗
PubMed2004

Conjunctival metastasis as initial sign of disseminated cutaneous melanoma.

PURPOSE: To describe a patient with conjunctival metastasis as the initial manifestation of metastasis from a cutaneous melanoma. DESIGN: Single interventional case report. METHODS: A 48-year-old woman with a history of cutaneous axillary melanoma developed a rapidly growing conjunctival mass. Subsequent systemic evaluation disclosed asymptomatic liver metastasis. The conjunctival lesion was resected. RESULTS: Histopathologic evaluation of the conjunctival tumor disclosed an epithelioid cell melanoma located in the conjunctival stroma, without appreciable junctional activity, compatible with metastatic melanoma. CONCLUSIONS: Conjunctival metastasis from cutaneous melanoma can rarely be the initial manifestation of disseminated melanoma.

Conjunctival Neoplasms↗