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Carol L. Shields - One of the best experts on this subject based on the ideXlab platform.
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Hemorrhagic occlusive retinal vasculitis leading to the diagnosis of Ciliary Body melanoma
Elsevier, 2019Co-Authors: Basil K. Williams, Joel R. Welch, Kristen H. Nwanyanwu, Carol L. ShieldsAbstract:Hemorrhagic occlusive retinal vasculitis (HORV) is a condition associated with intraocular vancomycin during surgical intervention, most frequently following bilateral sequential cataract surgery. Because of the high rate of ophthalmic vascular complications in this condition, gonioscopic evaluation for identification of neovascularization of the angle and iris is essential. Careful examination can reveal previously asymptomatic and unassociated lesions of the iris or Ciliary Body. We present the case of a 71-year-old female who was diagnosed with a Ciliary Body melanoma secondary to complete ophthalmic examination associated with HORV. She reported decreased vision to light perception in the left eye (OS) following sequential, bilateral cataract surgery. Fundus examination OS demonstrated diffuse retinal vasculitis, retinal ischemia, and extensive hemorrhage. Evaluation included inflammatory and coagulopathy laboratory evaluation, carotid ultrasonography and magnetic resonance imaging (MRI) of the brain and orbits, all of which proved unrevealing, except for an enhancing mass OS on MRI. Further evaluation revealed a pigmented mass with features of melanoma in the anterior chamber angle extending into the Ciliary Body. Fine needle aspiration biopsy revealed high risk cytogenetic characteristics, and plaque radiotherapy was successfully employed. Keywords: Ciliary Body, Hemorrhagic occlusive retinal vasculitis, Intracameral vancomycin, Malignant melanoma, Plaque radiotherapy, Uve
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hemorrhagic occlusive retinal vasculitis leading to the diagnosis of Ciliary Body melanoma
Saudi Journal of Ophthalmology, 2019Co-Authors: Basil K. Williams, Joel R. Welch, Kristen H. Nwanyanwu, Carol L. ShieldsAbstract:Abstract Hemorrhagic occlusive retinal vasculitis (HORV) is a condition associated with intraocular vancomycin during surgical intervention, most frequently following bilateral sequential cataract surgery. Because of the high rate of ophthalmic vascular complications in this condition, gonioscopic evaluation for identification of neovascularization of the angle and iris is essential. Careful examination can reveal previously asymptomatic and unassociated lesions of the iris or Ciliary Body. We present the case of a 71-year-old female who was diagnosed with a Ciliary Body melanoma secondary to complete ophthalmic examination associated with HORV. She reported decreased vision to light perception in the left eye (OS) following sequential, bilateral cataract surgery. Fundus examination OS demonstrated diffuse retinal vasculitis, retinal ischemia, and extensive hemorrhage. Evaluation included inflammatory and coagulopathy laboratory evaluation, carotid ultrasonography and magnetic resonance imaging (MRI) of the brain and orbits, all of which proved unrevealing, except for an enhancing mass OS on MRI. Further evaluation revealed a pigmented mass with features of melanoma in the anterior chamber angle extending into the Ciliary Body. Fine needle aspiration biopsy revealed high risk cytogenetic characteristics, and plaque radiotherapy was successfully employed.
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Distinguishing midzonal iris pigment epithelial cyst from adenoma and Ciliary Body melanoma
Wolters Kluwer Medknow Publications, 2018Co-Authors: Eileen L Mayro, Jerry A. Shields, Thamolwan Surakiatchanukul, Carol L. ShieldsAbstract:A 49-year old male with corrected visual acuity of 20/25 OD and 20/20 OS was found to have an asymptomatic dark iris mass OD with suspicion for Ciliary Body melanoma. Predilation slit-lamp biomicroscopy revealed a well-circumscribed brown round mass arising posterior to the iris, presumably from the Ciliary Body; however, postdilation demonstrated an extended fusiform mass of the iris pigment epithelium (IPE). By anterior segment imaging, the well-circumscribed IPE mass was cystic and measured 1440 microns in thickness peripherally predilation and 300 microns in thickness postdilation. There was no solid IPE or Ciliary Body component. A diagnosis of midzonal IPE cyst was rendered and observation advised. Clinical features and anterior segment imaging can assist in differentiation of midzonal IPE cyst from adenoma and Ciliary Body melanoma. The diagnostic methodology described in this paper can be used by ophthalmologists to promptly rule out underlying melanoma
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Ciliary Body medulloepithelioma association with pleuropulmonary blastoma in a familial tumor predisposition syndrome
Journal of Pediatric Ophthalmology & Strabismus, 2014Co-Authors: Gregory D Kramer, Carol L. Shields, Sruthi Arepalli, Jerry A. ShieldsAbstract:Ciliary Body medulloepithelioma can rarely present in association with pleuropulmonary blastoma as part of a familial tumor predisposition syndrome. This is thought to occur secondary to a germline mutation in the DICER1 gene. The authors describe a case of Ciliary Body medulloepithelioma in a 9-year-old girl with a known medical history of pleuropulmonary blastoma.
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ring melanoma of the Ciliary Body report on twenty three patients
Retina-the Journal of Retinal and Vitreous Diseases, 2002Co-Authors: Hakan Demirci, Jerry A. Shields, Carol L. Shields, Santosh G Honavar, Ralph C EagleAbstract:BackgroundRing 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.MethodsA retrospective review of the clinical features, management, histopathology, and prognosis of 23 consecutive patients with ring m
Jerry A. Shields - One of the best experts on this subject based on the ideXlab platform.
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Distinguishing midzonal iris pigment epithelial cyst from adenoma and Ciliary Body melanoma
Wolters Kluwer Medknow Publications, 2018Co-Authors: Eileen L Mayro, Jerry A. Shields, Thamolwan Surakiatchanukul, Carol L. ShieldsAbstract:A 49-year old male with corrected visual acuity of 20/25 OD and 20/20 OS was found to have an asymptomatic dark iris mass OD with suspicion for Ciliary Body melanoma. Predilation slit-lamp biomicroscopy revealed a well-circumscribed brown round mass arising posterior to the iris, presumably from the Ciliary Body; however, postdilation demonstrated an extended fusiform mass of the iris pigment epithelium (IPE). By anterior segment imaging, the well-circumscribed IPE mass was cystic and measured 1440 microns in thickness peripherally predilation and 300 microns in thickness postdilation. There was no solid IPE or Ciliary Body component. A diagnosis of midzonal IPE cyst was rendered and observation advised. Clinical features and anterior segment imaging can assist in differentiation of midzonal IPE cyst from adenoma and Ciliary Body melanoma. The diagnostic methodology described in this paper can be used by ophthalmologists to promptly rule out underlying melanoma
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Ciliary Body medulloepithelioma association with pleuropulmonary blastoma in a familial tumor predisposition syndrome
Journal of Pediatric Ophthalmology & Strabismus, 2014Co-Authors: Gregory D Kramer, Carol L. Shields, Sruthi Arepalli, Jerry A. ShieldsAbstract:Ciliary Body medulloepithelioma can rarely present in association with pleuropulmonary blastoma as part of a familial tumor predisposition syndrome. This is thought to occur secondary to a germline mutation in the DICER1 gene. The authors describe a case of Ciliary Body medulloepithelioma in a 9-year-old girl with a known medical history of pleuropulmonary blastoma.
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ring melanoma of the Ciliary Body report on twenty three patients
Retina-the Journal of Retinal and Vitreous Diseases, 2002Co-Authors: Hakan Demirci, Jerry A. Shields, Carol L. Shields, Santosh G Honavar, Ralph C EagleAbstract:BackgroundRing 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.MethodsA retrospective review of the clinical features, management, histopathology, and prognosis of 23 consecutive patients with ring m
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plaque radiotherapy for management of Ciliary Body and choroidal melanoma with extraocular extension
American Journal of Ophthalmology, 2000Co-Authors: Kaan Gunduz, Jerry A. Shields, Carol L. Shields, Jacqueline Cater, Luther W BradyAbstract:Abstract PURPOSE: To report the results of plaque radiotherapy of Ciliary Body and choroidal melanoma with extraocular extension. METHODS: This retrospective study of 17 eyes of 17 patients with Ciliary Body and choroidal melanoma demonstrated extraocular extension that was clinically evident but less than 3 mm in thickness. In 14 eyes, the extraocular extension was located anteriorly and in three posteriorly. The outcome of plaque radiotherapy in terms of intraocular and extraocular tumor responses and the development of metastasis were analyzed. RESULTS: At a median follow-up of 63 months (range, 23 to 164 months) after plaque radiotherapy, all patients showed control of both the intraocular and extraocular components of Ciliary Body and choroidal melanoma. In no case did the intraocular or extraocular portion of the tumor relapse. The initial median thickness of the intraocular component was 4.9 mm, and the final median thickness was 2.4 mm. The initial median thickness of the extraocular component was 2.0 mm, and the final median thickness was 1.0 mm. The initial median basal diameter of the extraocular component was 3.5 mm, and the final median basal diameter was 2.0 mm. Of 17 patients, 11 (10 with anterior and one with posterior extraocular extension) showed partial regression in base and thickness of the extraocular extension, and in six there was little or no change. In those with anterior extrascleral extension, the sclera remained intact without clinically observable necrosis, and the scleral fibers were gradually more visible as the tumor regressed. Three patients (18%) died of metastatic melanoma at a median interval of 38 months after plaque radiotherapy. CONCLUSIONS: Plaque radiotherapy appears to be a reasonable treatment option for selected cases of Ciliary Body and choroidal melanoma with clinically visible extraocular extension less than 3 mm in thickness.
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adenoma of the Ciliary Body pigment epithelium the 1998 albert ruedemann sr memorial lecture part 1
Archives of Ophthalmology, 1999Co-Authors: Jerry A. Shields, Carol L. Shields, Kaan Gunduz, Ralph C EagleAbstract:Background Adenoma of the pigment epithelium of the Ciliary Body (CPE) is a rare neoplasm. Most reported cases have been misdiagnosed as Ciliary Body melanoma. Objectives To evaluate clinical features, management, pathological features, and prognosis of adenoma of the CPE and to determine clinical features that may differentiate it from Ciliary Body melanoma. Patients and Methods A retrospective review was performed of medical charts, photographs, and pathological features of patients with adenoma of the CPE who were treated by the authors. Results Of the 8 patients with adenoma of the CPE, 3 were male and 5 were female. Seven were white, and 1 was Asian. The mean age at diagnosis was 51 years (range, 8-73 years). The referring diagnosis was Ciliary Body melanoma in 7 patients and cyst in 1 patient. The lesions were all solitary and unilateral and ranged from 3×3×3 to 13×13×8 mm. Clinically, all tumors were gray to black, had abruptly elevated margins, and were dome shaped. Associated findings included secondary cataract (6 patients) and vitreous hemorrhage (1 patient). Results of ancillary studies such as transillumination, fluorescein angiography, and ultrasonography showed patterns that were helpful in differentiation from Ciliary Body melanoma. Fine needle aspiration biopsy, performed in 3 patients, was an accurate diagnostic adjunct. Microscopic diagnosis was adenoma of the CPE in 7 cases in which tissue was available. A consistent histopathologic feature was the presence of typical clear vacuoles within the tumor. One tumor invaded the sensory retina. Results of immunohistochemical studies were consistent with a tumor of neuroectodermal origin. Conclusions Adenoma of the CPE has characteristic features that may help differentiate it from Ciliary Body melanoma. In contrast to melanoma, it is generally darker and its margins are more abruptly elevated. Although it is benign cytologically, it can exhibit growth. If the diagnosis is suspected, removal of the tumor by local resection is advisable.
Marianne Cilluffo - One of the best experts on this subject based on the ideXlab platform.
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beta b1 crystallin identification of a candidate Ciliary Body uveitis antigen
Investigative Ophthalmology & Visual Science, 2003Co-Authors: David Stempel, Marianne Cilluffo, Hallie Sandusky, Jonathan Braun, Kirsten J Lampi, Joe Horwitz, Lee Goodglick, Lynn K GordonAbstract:PURPOSE. Perineuclear anti-neutrophil cytoplasmic antiBody (pANCA), a marker antiBody present in 12% of patients with anterior uveitis, recognizes cytoplasmic antigens in the nonpigmented Ciliary Body epithelium, a probable site of immunologic reactivity in this inflammatory disease. In this study, a recombinantly isolated pANCA monoclonal antiBody was used to identify the corresponding antigenic target(s) in the Ciliary Body. METHODS. Proteins from microdissected eye bank ocular Ciliary Body tissue were used to identify the corresponding ANCA antigen. Parallel two-dimensional protein gels were used for simultaneous identification of candidate antigenic protein spots by Western blot analysis and as a source of material for proteomic analysis. Multiple independent methods including Western blot analysis, confocal microscopy, and RT-PCR were used to provide additional characterization of the candidate protein. RESULTS. Proteomic analysis suggested that beta B1 (B1)-crystallin is the primary Ciliary Body antigen. The presence of B1-crystallin in the human Ciliary Body was confirmed by Western blot with a B1 specific anti-peptide antiBody. Confocal microscopy revealed colocalization of the antigenic reactivity of both anti-B1 antiBody and monoclonal pANCA. RTPCR confirmed the presence of B1-crystallin RNA in the Ciliary Body tissues. CONCLUSIONS. This study identified B1-crystallin as a new cytoplasmic Ciliary Body antigenic target of a marker autoantiBody associated with uveitis. This characterization of B1crystallin outside the lens raises questions about its extralenticular expression, intracellular role, and potential target of inflammation in uveitis. (Invest Ophthalmol Vis Sci. 2003;44:203‐209) DOI:10.1167/iovs.01-1261
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p2 purinergic receptor coupled signaling in the rabbit Ciliary Body epithelium
Investigative Ophthalmology & Visual Science, 2002Co-Authors: Nasser A. Farahbakhsh, Marianne CilluffoAbstract:PURPOSE. To identify and characterize P2 purinergic receptors and their signaling pathways in the epithelial cells of the rabbit Ciliary Body. METHODS. Real-time fluorescence ratio imaging of the intact furs-2-loaded nonpigmented Ciliary Body epithelial (NPE) cells of rabbit were used to record changes in the intracellular free calcium concentration ([Ca 2+ ] i ), in response to a number of purinergic agonists and antagonists. The effects of some of these drugs on the inositol phosphate (IP) levels in Ciliary processes were also examined. RESULTS. Adenosine diphosphate (ADP), adenosine triphosphate (ATP), and uridine triphosphate (UTP) dose dependently increased the [Ca 2+ ] i and IP levels. The [Ca 2+ ] i increases induced by ADP and UTP were distinguishable, both kinetically and pharmacologically. The effect of ADP on [Ca 2+ ] i was mimicked by a number of P2Y 1 -selective agonists, and was blocked by three P2Y 1 -receptor-specific antagonists. The [Ca 2+ ] i increases elicited by ADP (or its analogs) and UTP were additive. CONCLUSIONS. Rabbit Ciliary Body epithelium possesses both P2Y 1 and P2Y 2 metabotropic purinergic receptor subtypes, which differentially use the IP 3 /Ca 2+ second-messenger pathway.
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ocular panca antigens are expressed in nonpigmented Ciliary Body epithelium and are conserved in multiple mammalian species
Ocular Immunology and Inflammation, 2001Co-Authors: Hallie Sandusky, Marianne Cilluffo, Jonathan Braun, Lynn K GordonAbstract:PURPOSE: pANCA marker autoantiBody is expressed by a subset of patients with anterior uveitis. A recombinantly isolated pANCA monoclonal antiBody, Fab 5–3, identifies ocular expression of corresponding pANCA antigens in human Ciliary Body and retina. In this study, Fab 5–3 was used to explore pANCA antigen expression in ocular tissues of multiple mammalian species and identify the Ciliary Body cell type expressing the pANCA antigen. METHODS: Ocular tissues were obtained from several mammalian species and evaluated for expression of the pANCA (Fab 5–3) antigen(s) using immunohistochemistry and Western analysis of tissue extracts. Additionally, primary cultures of nonpigmented and pigmented rabbit Ciliary Body epithelium were analyzed for pANCA expression using immunofluorescence and Western analysis. RESULTS: Ocular pANCA (Fab 5–3) antigen expression was observed by immunohistochemistry only in the cytoplasm of retinal ganglion cells and Ciliary Body epithelium. Retinal antigen expression was conserved in ...
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nonpigmented cells of the rabbit Ciliary Body epithelium tissue culture and voltage gated currents
Investigative Ophthalmology & Visual Science, 1991Co-Authors: Marianne Cilluffo, B N Cohen, Gordon L FainAbstract:The aqueous humor of the eye is thought to be secreted by the epithelium of the Ciliary Body. This epithelium has been difficult to study, in part because of its complicated morphology. The authors attempted to circumvent this difficulty by growing the epithelial cells in tissue culture. A procedure is described for producing pure primary cultures of rabbit nonpigmented Ciliary Body epithelial cells. This procedure was used with whole-cell patch-clamp recording to characterize voltage-activated currents in the nonpigmented cells. These experiments show that most nonpigmented cells contain two kinds of currents: a rapidly activating and inactivating inward current, carried by Na+ and blocked by tetrodotoxin (TTX), and a more slowly activating and inactivating outward current, blocked by tetraethylammonium (TEA+), Ba2+, and 4-aminopyridine (4-AP) and presumably carried by K+. Both of these currents have been observed in freshly dissociated cells and in cultures up to 7 days old. The voltage-gated currents in Ciliary Body epithelial cells are remarkably similar to those of neurons and raise the possibility that these epithelial cells are capable of spike propagation.
Luther W Brady - One of the best experts on this subject based on the ideXlab platform.
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plaque radiotherapy for management of Ciliary Body and choroidal melanoma with extraocular extension
American Journal of Ophthalmology, 2000Co-Authors: Kaan Gunduz, Jerry A. Shields, Carol L. Shields, Jacqueline Cater, Luther W BradyAbstract:Abstract PURPOSE: To report the results of plaque radiotherapy of Ciliary Body and choroidal melanoma with extraocular extension. METHODS: This retrospective study of 17 eyes of 17 patients with Ciliary Body and choroidal melanoma demonstrated extraocular extension that was clinically evident but less than 3 mm in thickness. In 14 eyes, the extraocular extension was located anteriorly and in three posteriorly. The outcome of plaque radiotherapy in terms of intraocular and extraocular tumor responses and the development of metastasis were analyzed. RESULTS: At a median follow-up of 63 months (range, 23 to 164 months) after plaque radiotherapy, all patients showed control of both the intraocular and extraocular components of Ciliary Body and choroidal melanoma. In no case did the intraocular or extraocular portion of the tumor relapse. The initial median thickness of the intraocular component was 4.9 mm, and the final median thickness was 2.4 mm. The initial median thickness of the extraocular component was 2.0 mm, and the final median thickness was 1.0 mm. The initial median basal diameter of the extraocular component was 3.5 mm, and the final median basal diameter was 2.0 mm. Of 17 patients, 11 (10 with anterior and one with posterior extraocular extension) showed partial regression in base and thickness of the extraocular extension, and in six there was little or no change. In those with anterior extrascleral extension, the sclera remained intact without clinically observable necrosis, and the scleral fibers were gradually more visible as the tumor regressed. Three patients (18%) died of metastatic melanoma at a median interval of 38 months after plaque radiotherapy. CONCLUSIONS: Plaque radiotherapy appears to be a reasonable treatment option for selected cases of Ciliary Body and choroidal melanoma with clinically visible extraocular extension less than 3 mm in thickness.
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plaque radiotherapy of uveal melanoma with predominant Ciliary Body involvement
Archives of Ophthalmology, 1999Co-Authors: Kaan Gunduz, Jerry A. Shields, Carol L. Shields, Jacqueline Cater, Jorge Freire, Luther W BradyAbstract:Background There are several options for management of Ciliary Body melanoma, including plaque radiotherapy, charged particle irradiation, local resection, and enucleation. The choice of therapy depends on many factors, and plaque radiotherapy is often used. Objectives To determine the outcome of plaque radiotherapy in the management of Ciliary Body melanoma and to identify the risk factors associated with the development of radiation complications, tumor recurrence, metastasis, and melanoma-related death after plaque radiotherapy of Ciliary Body melanoma. Methods We analyzed the clinical records of 136 patients with Ciliary Body melanoma who were treated with plaque radiotherapy between July 1976 and June 1992. Results The median follow-up period was 70 months. Using Kaplan-Meier survival estimates, the most frequent radiation complication at 5 years' follow-up was cataract, developing in 48% of the patients, followed by neovascular glaucoma (21%), retinopathy (20%), scleral necrosis (12%), and vitreous hemorrhage (11%). Visual acuity decrease (by ≥3 Snellen lines) was noted in 40% of the patients at 5 years. Kaplan-Meier estimates showed that 8% of the patients developed recurrence, 28% had metastasis, and 22% died of melanoma-related causes by 5 years. Univariate analysis demonstrated that the factors predictive of radiation cataract were superonasal ( P =.003) and inferior tumor meridian ( P =.02) compared with inferonasal meridian and apex dose rate greater than 57 cGy/h ( P =.05). The development of neovascular glaucoma was significantly related to iris involvement with the Ciliary Body tumor ( P P =.03) and the presence of diabetes mellitus ( P =.05). The only predictor of metastasis was tumor thickness greater than 7 mm ( P =.02). The risk factors for melanoma-related death were the presence of metastasis ( P P =.02), and recurrence ( P =.02). Multivariate analyses showed that the most significant variables predictive of the development of scleral necrosis were intraocular pressure greater than 15 mm Hg ( P P =.007). The most significant predictive factors for vitreous hemorrhage were visual acuity of 20/40 to 20/200 ( P =.02) and intraocular pressure greater than 15 mm Hg ( P =.02). The best subset of independent predictors of vision decrease were mushroom tumor shape ( P =.002), age older than 61 years ( P =.006), and superonasal meridian ( P =.04). The risks for melanoma-related death were presence of metastasis ( P P =.01). There was no group of significant variables predictive for radiation cataract, neovascular glaucoma, retinopathy, tumor recurrence, and metastasis in multivariate analysis. Conclusions Plaque radiotherapy offers 92% 5-year local control rate for Ciliary Body melanoma. Metastasis occurs in 28% of the patients treated with this method by 5 years. Patients with tumors greater than 7 mm in thickness are at greater risk than patients with thinner tumors for metastatic disease and melanoma-related death. Major radiation complications include radiation cataract, neovascular glaucoma, retinopathy, and scleral necrosis.
Kaan Gunduz - One of the best experts on this subject based on the ideXlab platform.
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plaque radiotherapy for management of Ciliary Body and choroidal melanoma with extraocular extension
American Journal of Ophthalmology, 2000Co-Authors: Kaan Gunduz, Jerry A. Shields, Carol L. Shields, Jacqueline Cater, Luther W BradyAbstract:Abstract PURPOSE: To report the results of plaque radiotherapy of Ciliary Body and choroidal melanoma with extraocular extension. METHODS: This retrospective study of 17 eyes of 17 patients with Ciliary Body and choroidal melanoma demonstrated extraocular extension that was clinically evident but less than 3 mm in thickness. In 14 eyes, the extraocular extension was located anteriorly and in three posteriorly. The outcome of plaque radiotherapy in terms of intraocular and extraocular tumor responses and the development of metastasis were analyzed. RESULTS: At a median follow-up of 63 months (range, 23 to 164 months) after plaque radiotherapy, all patients showed control of both the intraocular and extraocular components of Ciliary Body and choroidal melanoma. In no case did the intraocular or extraocular portion of the tumor relapse. The initial median thickness of the intraocular component was 4.9 mm, and the final median thickness was 2.4 mm. The initial median thickness of the extraocular component was 2.0 mm, and the final median thickness was 1.0 mm. The initial median basal diameter of the extraocular component was 3.5 mm, and the final median basal diameter was 2.0 mm. Of 17 patients, 11 (10 with anterior and one with posterior extraocular extension) showed partial regression in base and thickness of the extraocular extension, and in six there was little or no change. In those with anterior extrascleral extension, the sclera remained intact without clinically observable necrosis, and the scleral fibers were gradually more visible as the tumor regressed. Three patients (18%) died of metastatic melanoma at a median interval of 38 months after plaque radiotherapy. CONCLUSIONS: Plaque radiotherapy appears to be a reasonable treatment option for selected cases of Ciliary Body and choroidal melanoma with clinically visible extraocular extension less than 3 mm in thickness.
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adenoma of the Ciliary Body pigment epithelium the 1998 albert ruedemann sr memorial lecture part 1
Archives of Ophthalmology, 1999Co-Authors: Jerry A. Shields, Carol L. Shields, Kaan Gunduz, Ralph C EagleAbstract:Background Adenoma of the pigment epithelium of the Ciliary Body (CPE) is a rare neoplasm. Most reported cases have been misdiagnosed as Ciliary Body melanoma. Objectives To evaluate clinical features, management, pathological features, and prognosis of adenoma of the CPE and to determine clinical features that may differentiate it from Ciliary Body melanoma. Patients and Methods A retrospective review was performed of medical charts, photographs, and pathological features of patients with adenoma of the CPE who were treated by the authors. Results Of the 8 patients with adenoma of the CPE, 3 were male and 5 were female. Seven were white, and 1 was Asian. The mean age at diagnosis was 51 years (range, 8-73 years). The referring diagnosis was Ciliary Body melanoma in 7 patients and cyst in 1 patient. The lesions were all solitary and unilateral and ranged from 3×3×3 to 13×13×8 mm. Clinically, all tumors were gray to black, had abruptly elevated margins, and were dome shaped. Associated findings included secondary cataract (6 patients) and vitreous hemorrhage (1 patient). Results of ancillary studies such as transillumination, fluorescein angiography, and ultrasonography showed patterns that were helpful in differentiation from Ciliary Body melanoma. Fine needle aspiration biopsy, performed in 3 patients, was an accurate diagnostic adjunct. Microscopic diagnosis was adenoma of the CPE in 7 cases in which tissue was available. A consistent histopathologic feature was the presence of typical clear vacuoles within the tumor. One tumor invaded the sensory retina. Results of immunohistochemical studies were consistent with a tumor of neuroectodermal origin. Conclusions Adenoma of the CPE has characteristic features that may help differentiate it from Ciliary Body melanoma. In contrast to melanoma, it is generally darker and its margins are more abruptly elevated. Although it is benign cytologically, it can exhibit growth. If the diagnosis is suspected, removal of the tumor by local resection is advisable.
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plaque radiotherapy of uveal melanoma with predominant Ciliary Body involvement
Archives of Ophthalmology, 1999Co-Authors: Kaan Gunduz, Jerry A. Shields, Carol L. Shields, Jacqueline Cater, Jorge Freire, Luther W BradyAbstract:Background There are several options for management of Ciliary Body melanoma, including plaque radiotherapy, charged particle irradiation, local resection, and enucleation. The choice of therapy depends on many factors, and plaque radiotherapy is often used. Objectives To determine the outcome of plaque radiotherapy in the management of Ciliary Body melanoma and to identify the risk factors associated with the development of radiation complications, tumor recurrence, metastasis, and melanoma-related death after plaque radiotherapy of Ciliary Body melanoma. Methods We analyzed the clinical records of 136 patients with Ciliary Body melanoma who were treated with plaque radiotherapy between July 1976 and June 1992. Results The median follow-up period was 70 months. Using Kaplan-Meier survival estimates, the most frequent radiation complication at 5 years' follow-up was cataract, developing in 48% of the patients, followed by neovascular glaucoma (21%), retinopathy (20%), scleral necrosis (12%), and vitreous hemorrhage (11%). Visual acuity decrease (by ≥3 Snellen lines) was noted in 40% of the patients at 5 years. Kaplan-Meier estimates showed that 8% of the patients developed recurrence, 28% had metastasis, and 22% died of melanoma-related causes by 5 years. Univariate analysis demonstrated that the factors predictive of radiation cataract were superonasal ( P =.003) and inferior tumor meridian ( P =.02) compared with inferonasal meridian and apex dose rate greater than 57 cGy/h ( P =.05). The development of neovascular glaucoma was significantly related to iris involvement with the Ciliary Body tumor ( P P =.03) and the presence of diabetes mellitus ( P =.05). The only predictor of metastasis was tumor thickness greater than 7 mm ( P =.02). The risk factors for melanoma-related death were the presence of metastasis ( P P =.02), and recurrence ( P =.02). Multivariate analyses showed that the most significant variables predictive of the development of scleral necrosis were intraocular pressure greater than 15 mm Hg ( P P =.007). The most significant predictive factors for vitreous hemorrhage were visual acuity of 20/40 to 20/200 ( P =.02) and intraocular pressure greater than 15 mm Hg ( P =.02). The best subset of independent predictors of vision decrease were mushroom tumor shape ( P =.002), age older than 61 years ( P =.006), and superonasal meridian ( P =.04). The risks for melanoma-related death were presence of metastasis ( P P =.01). There was no group of significant variables predictive for radiation cataract, neovascular glaucoma, retinopathy, tumor recurrence, and metastasis in multivariate analysis. Conclusions Plaque radiotherapy offers 92% 5-year local control rate for Ciliary Body melanoma. Metastasis occurs in 28% of the patients treated with this method by 5 years. Patients with tumors greater than 7 mm in thickness are at greater risk than patients with thinner tumors for metastatic disease and melanoma-related death. Major radiation complications include radiation cataract, neovascular glaucoma, retinopathy, and scleral necrosis.