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Melanoma: HELP
Articles by Jerry A. Shields
Based on 103 articles published since 2010
(Why 103 articles?)
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Between 2010 and 2020, J. A. Shields wrote the following 103 articles about Melanoma.
 
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5
1 Editorial Bilateral uveal melanoma: hunting for the source. 2018

Shields, Carol L / Masoomian, Babak / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. ·Clin Exp Ophthalmol · Pubmed #30003677.

ABSTRACT: -- No abstract --

2 Editorial Surgical removal of intraocular tumors: dismissing old wives' tales. 2013

Shields, Carol L / Shields, Jerry A. · ·Am J Ophthalmol · Pubmed #23791370.

ABSTRACT: -- No abstract --

3 Editorial Uveal melanoma trapped in the temple of doom. 2012

Shields, Carol L / Ganguly, Arupa / O'Brien, Joan / Sato, Takami / Shields, Jerry A. · ·Am J Ophthalmol · Pubmed #22813447.

ABSTRACT: -- No abstract --

4 Review Prognostication of uveal melanoma is simple and highly predictive using The Cancer Genome Atlas (TCGA) classification: A review. 2019

Shields, Carol L / Dalvin, Lauren A / Vichitvejpaisal, Pornpattana / Mazloumi, Mehdi / Ganguly, Arupa / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Suite, Philadelphia, PA, United States. · Department of Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States. ·Indian J Ophthalmol · Pubmed #31755428.

ABSTRACT: Purpose: The cancer genome atlas (TCGA) is a comprehensive project supported by the National Cancer Institute (NCI) in the United States to explore molecular alterations in cancer, including uveal melanoma (UM). This led to TCGA classification for UM. In this report, we review the American Joint Committee on Cancer (AJCC) classification and TCGA classification for UM from the NCI's Center for Cancer Genomics (NCI CCG) (based on enucleation specimens [n = 80 eyes]) and from Wills Eye Hospital (WEH) (based on fine needle aspiration biopsy [FNAB] specimens [n = 658 eyes]). We then compare accuracy and predictability of AJCC versus (vs.) TCGA. Methods: Review of published reports on AJCC and TCGA classification for UM was performed. Outcomes based on AJCC 7 Results: In the NCI CCG study, there were 80 eyes with UM sampled by enucleation (n = 77), resection (n = 2), or orbitotomy (n = 1) and analysis revealed four distinct genetic classes. Metastasis and death outcomes were subsequently evaluated per class in the WEH study. The WEH study reviewed 658 eyes with UM, sampled by FNAB, and found Class A (n = 342, 52%), B (n = 91, 14%), C (n = 118, 18%), and D (n = 107, 16%). Comparison by increasing class (A vs. B vs. C vs. D) revealed older mean patient age (P < 0.001), worse entering visual acuity (P < 0.001), greater distance from the optic disc (P < 0.001), larger tumor diameter (P < 0.001), and greater tumor thickness (P < 0.001). Regarding outcomes, more advanced TCGA class demonstrated increased 5-year risk for metastasis (4% vs. 20% vs. 33% vs. 63%,P < 0.001) with corresponding increasing hazard ratio (HR) (1.0 vs. 4.1, 10.1, 30.0,P= 0.01 for B vs. A andP < 0.001 for C vs. A and D vs. A) as well as increased 5-year estimated risk for death (1% vs. 0% vs. 9% vs. 23%,P < 0.001) with corresponding increasing HR (1 vs. NA vs. 3.1 vs. 13.7,P= 0.11 for C vs. A andP < 0.001 for D vs. A). Comparison of AJCC to TCGA classification revealed TCGA was superior in prediction of metastasis and death from UM. Conclusion: TCGA classification for UM is simple, accurate, and highly predictive of melanoma-related metastasis and death, more so than the AJCC classification.

5 Review Melanocytoma of the optic disk: A review. 2019

Shields, Jerry A / Demirci, Hakan / Mashayekhi, Arman / Eagle, Ralph C / Shields, Carol L. ·Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. · Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. ·Indian J Ophthalmol · Pubmed #31755427.

ABSTRACT: Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas and erroneous enucleation is rarely done. Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. Although traditionally believed to be a relatively stationary lesion, it is now known to exhibit minor enlargement in 10--15% of cases and can cause minor visual loss by a variety of mechanisms. In rare instance, it can induce severe visual loss due to spontaneous necrosis of the lesion or compressive optic neuropathy. More importantly, it can exhibit malignant transformation into melanoma in 1--2% of cases. Ophthalmologists should be familiar with melanocytoma of the optic disk and affected patients should be followed periodically.

6 Review Tumors of the conjunctiva and cornea. 2019

Shields, Carol L / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. ·Indian J Ophthalmol · Pubmed #31755426.

ABSTRACT: Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. The caruncle displays a slightly different array of tumors compared to those elsewhere on the conjunctiva, as nevus and papilloma are most common, but oncocytoma and sebaceous gland hyperplasia, adenoma, and carcinoma can be found. In this report, we provide clinical description and illustration of the many conjunctival and corneal tumors and we discuss tumor management.

7 Review Small choroidal melanoma: detection with multimodal imaging and management with plaque radiotherapy or AU-011 nanoparticle therapy. 2019

Shields, Carol L / Lim, Li-Anne S / Dalvin, Lauren A / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. ·Curr Opin Ophthalmol · Pubmed #30844944.

ABSTRACT: PURPOSE OF REVIEW: To explore risk factors for choroidal nevus transformation into melanoma using multimodal imaging and review current treatment options. RECENT FINDINGS: A recently published longitudinal study of 3806 choroidal nevi, imaged with optical coherence tomography (OCT), ultrasonography, and standard wavelength autofluorescence, revealed transformation into melanoma in 5.8% at 5 years and 13.9% at 10 years, using Kaplan-Meier analysis. Multivariate factors predictive of transformation included thickness more than 2 mm (by ultrasonography), fluid subretinal (by OCT), symptoms vision loss (by Snellen acuity), orange pigment (by autofluorescence), melanoma hollow (by ultrasonography), and DIaMeter more than 5 mm (by photography). These important factors can be recalled by the mnemonic 'To Find Small Ocular Melanoma Doing IMaging' (TFSOM-DIM). The mean 5-year estimate of nevus growth into melanoma was 1.1% for those with 0 risk factor, 11% with one factor, 22% with two factors, 34% with three factors, 51% with four factors, and 55% with five risk factors. Management of small choroidal melanoma typically involves plaque radiotherapy with 5 and 10-year rates of tumor recurrence at 7 and 11%, visual acuity loss (≥3 Snellen lines) at 39 and 49%, and melanoma-related metastasis at 4 and 9%. A novel infrared dye-conjugated virus-like nanoparticle (AU-011) is currently under investigation for treatment of small choroidal melanoma, with a goal to induce tumor regression and minimize vision loss. SUMMARY: The mnemonic, TFSOM-DIM, can assist the clinician in detection of small choroidal melanoma. Treatment of small melanoma with plaque radiotherapy offers tumor control but with potential vision loss. A novel nanoparticle therapy using AU-011 is currently under trial.

8 Review Overview of BAP1 cancer predisposition syndrome and the relationship to uveal melanoma. 2018

Masoomian, Babak / Shields, Jerry A / Shields, Carol L. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA. ·J Curr Ophthalmol · Pubmed #29988936.

ABSTRACT: Purpose: The aim of this study was to review the genetics, epidemiology, clinical findings, and management of BRCA1-associated protein-1 (BAP1) cancer predisposition syndrome, particularly focusing on the development of uveal melanoma (UM). Methods: This is a review article based on eligible studies identified by systematically searching PubMed, Web of Science, and reference lists. Results: UM is the most common primary intraocular malignancy. Most UM cases are sporadic, but a small percentage has been documented with familial tendency. Until recently, there was little information regarding the genetics of this malignant tumor, and we have now begun to understand the pathways of development. BAP1 is a scavenger protein that regulates cell cycle, cellular differentiation, and DNA damage response. Patients and families with germline BAP1 mutation are predisposed to familial cancers including UM, mesothelioma, cutaneous melanoma (CM), renal cell carcinoma (RCC), and others. Clinicians should be aware of the implications of germline BAP1 mutation and advise genetic testing and assessment for BAP1 germline mutation in suspected patients and families. Conclusions: The ability of BAP1 gene mutation to cause multiple tumor types and high penetrance in carriers suggests that this gene has an important role for influencing cancer cell growth. With progress in understanding the molecular landscape of UM and the development of treatments targeted to the pathways involving BAP1 and other gene mutations, it is possible to improve the outcome of this malignant cancer.

9 Review Prognosis of uveal melanoma based on race in 8100 patients: The 2015 Doyne Lecture. 2015

Shields, C L / Kaliki, S / Cohen, M N / Shields, P W / Furuta, M / Shields, J A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA. · 1] Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA [2] Dr Kaliki currently practices at the Ocular Oncology Service, L.V. Prasad Eye Institute, Hyderabad, India. ·Eye (Lond) · Pubmed #26248525.

ABSTRACT: A retrospective, nonrandomized, interventional case series of 8100 patients with uveal melanoma were evaluated for melanoma-related metastasis based on patient race. The patient race was Caucasian (n=7918, 98%), Hispanic (n=105, 1%), Asian (n=44, <1%), or African American (n=33, <1%). On the basis of race (Caucasian, Hispanic, Asian, and African American), significant differences were noted in mean age at presentation (58, 48, 44, and 52 years; P<0.001), distance of posterior tumor margin to foveola (5, 5, 6, and 4 mm; P<0.001), distance of posterior tumor margin to optic disc (5, 5, 6, and 4 mm) (P<0.001), tumor base (11, 12, 12, and 13 mm; P<0.001), tumor thickness (5.4, 7.1, 6.5, and 7.5 mm; P<0.001), intraocular hemorrhage (10, 14, 11, and 24%; P=0.02), and rupture of Bruch's membrane (20, 27, 39, and 36%; P=0.001). On the basis of multivariate analysis, the rate of metastasis increased with increasing age (P<0.001), ciliary body location (P<0.001), increasing tumor base (P<0.001), increasing tumor thickness (P<0.001), pigmented tumor (P=0.001), subretinal fluid (P=0.001), intraocular hemorrhage (P=0.045), and extraocular extension (P=0.036). Kaplan-Meier estimates of metastasis at 3, 5, and 10 were 8, 15, and 25% in Caucasians; 13, 13, and 13% in Hispanics; 4, 4, and 36% in Asians; and 8, 8, and 8% in African Americans. Compared with Caucasians, despite relative risk for metastasis of 0.31 for African Americans, 0.73 for Hispanics, and 1.42 for Asians, there was no statistical difference in metastasis, or death from uveal melanoma based on race. In summary, uveal melanoma showed similar prognosis for all races.

10 Review Uveal melanoma: estimating prognosis. 2015

Kaliki, Swathi / Shields, Carol L / Shields, Jerry A. ·Institute for Eye Cancer, L V Prasad Eye Institute, Banjara Hills, Support provided by Operation Eyesight Institute for Eye Cancer (SK) and Hyderabad Eye Research Foundation (SK), Hyderabad, India. ·Indian J Ophthalmol · Pubmed #25827538.

ABSTRACT: Uveal melanoma is the most common primary malignant tumor of the eye in adults, predominantly found in Caucasians. Local tumor control of uveal melanoma is excellent, yet this malignancy is associated with relatively high mortality secondary to metastasis. Various clinical, histopathological, cytogenetic features and gene expression features help in estimating the prognosis of uveal melanoma. The clinical features associated with poor prognosis in patients with uveal melanoma include older age at presentation, male gender, larger tumor basal diameter and thickness, ciliary body location, diffuse tumor configuration, association with ocular/oculodermal melanocytosis, extraocular tumor extension, and advanced tumor staging by American Joint Committee on Cancer classification. Histopathological features suggestive of poor prognosis include epithelioid cell type, high mitotic activity, higher values of mean diameter of ten largest nucleoli, higher microvascular density, extravascular matrix patterns, tumor-infiltrating lymphocytes, tumor-infiltrating macrophages, higher expression of insulin-like growth factor-1 receptor, and higher expression of human leukocyte antigen Class I and II. Monosomy 3, 1p loss, 6q loss, and 8q and those classified as Class II by gene expression are predictive of poor prognosis of uveal melanoma. In this review, we discuss the prognostic factors of uveal melanoma. A database search was performed on PubMed, using the terms "uvea," "iris," "ciliary body," "choroid," "melanoma," "uveal melanoma" and "prognosis," "metastasis," "genetic testing," "gene expression profiling." Relevant English language articles were extracted, reviewed, and referenced appropriately.

11 Review Melanoma of the eye: revealing hidden secrets, one at a time. 2015

Shields, Carol L / Kels, Jane Grant / Shields, Jerry A. ·The Ocular Oncology Service, Wills Eye Hospital, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA. Electronic address: carol.shields@shieldsoncology.com. · Department of Dermatology, University of Connecticut Health Center, Farmington, CT. · The Ocular Oncology Service, Wills Eye Hospital, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA. ·Clin Dermatol · Pubmed #25704938.

ABSTRACT: Melanoma of the eye can involve the uveal tract with iris, ciliary body, or choroid involvement or it can involve the conjunctiva, eyelid, or orbit. Uveal involvement with choroidal melanoma is the most common, found in light complexion Caucasians with an age-adjusted incidence of 4.3 per million persons. Early detection of uveal melanoma is critical. The ABCDEF guide helps to differentiate iris nevus from iris melanoma. The letters represent: A, age young (≤40 years); B, blood in anterior chamber; C, clock hour of mass inferiorly; D, diffuse configuration; E, ectropion; and F, feathery margins. The mnemonic of TFSOM-UHHD (To Find Small Ocular Melanoma-Using Helpful Hints Daily) helps to differentiate choroidal nevus from small melanoma and represents: T, thickness over 2 mm; F, fluid; S, symptoms; O, orange pigment; M, margin within 3 mm of the optic disc; UH, ultrasound hollow; H, halo absent; and D, drusen absent. Patients with 3 or more of these factors are likely to have melanoma. These key clinical features help to identify small melanoma at a time when therapy could be life-saving. Conjunctival melanoma usually arises from primary acquired melanosis, a flat pigmentation that can lead to melanoma. Wide excision using no touch strategy is important to tumor control. Ocular examination is advised annually for all persons for detection of refractive error, cataract, glaucoma, and other conditions, but also for the detection of asymptomatic malignancies like melanoma. One at a time, we have uncovered the secrets of ocular melanoma and we forge ahead with the goal to solve the riddle of this challenging disease.

12 Review Choroidal melanoma: clinical features, classification, and top 10 pseudomelanomas. 2014

Shields, Carol L / Manalac, Janet / Das, Chandana / Ferguson, Kyle / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. ·Curr Opin Ophthalmol · Pubmed #24614143.

ABSTRACT: PURPOSE OF REVIEW: To review the current features and classification of choroidal melanoma, and to identify the lesions that clinically simulate choroidal melanoma (pseudomelanoma). RECENT FINDINGS: Uveal melanoma is a serious life-threatening intraocular malignancy, most often found in Caucasians (98%) and primarily involving the choroid (90%), ciliary body (7%), or iris (2%). This review will concentrate on choroidal melanoma. At diagnosis, choroidal melanoma usually appears as a pigmented (85%) tumor underlying the retina with a median basal dimension of 11 mm and a mean thickness of 4.5 mm. The American Joint Committee on Cancer classification allows for categorization and staging of melanoma. Following ocular therapy, adjuvant systemic therapy is provided for patients with high-risk melanoma who demonstrate alterations in chromosomes 3, 6, and 8 or those with class 2 on gene-expression profiling, detected by needle biopsy or solid tumor biopsy. The prognosis of choroidal melanoma depends most importantly on the genetic alterations and tumor size. Every millimeter increase in thickness leads to a 5% increased risk for metastasis. The leading conditions that simulate choroidal melanoma include choroidal nevus, peripheral exudative hemorrhagic chorioretinopathy, congenital hypertrophy of the retinal pigment epithelium (RPE), hemorrhagic RPE detachment, choroidal hemangioma, age-related macular degeneration, RPE hyperplasia, and others. These pseudomelanomas can be differentiated from choroidal melanoma by their unique clinical features. SUMMARY: Choroidal melanoma is a serious malignancy with characteristic features. Early detection and therapy is important. Pseudomelanomas can lead to diagnostic confusion; however, clinical features aid in differentiation.

13 Review Review of cystic and solid tumors of the iris. 2013

Shields, Carol L / Shields, Patrick W / Manalac, Janet / Jumroendararasame, Chaisiri / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, PA., USA. ·Oman J Ophthalmol · Pubmed #24379549.

ABSTRACT: Iris tumors are broadly classified into cystic or solid lesions. The cystic lesions arise from iris pigment epithelium (IPE) or iris stroma. IPE cysts classically remain stable without need for intervention. Iris stromal cyst, especially those in newborns, usually requires therapy of aspiration, possibly with alcohol-induced sclerosis, or surgical resection. The solid tumors included melanocytic and nonmelanocytic lesions. The melanocytic iris tumors include freckle, nevus (including melanocytoma), Lisch nodule, and melanoma. Information from a tertiary referral center revealed that transformation of suspicious iris nevus to melanoma occurred in 4% by 10 years and 11% by 20 years. Risk factors for transformation of iris nevus to melanoma can be remembered using the ABCDEF guide as follows: A=age young (<40 years), B=blood (hyphema) in anterior chamber, C=clock hour of mass inferiorly, D=diffuse configuration, E=ectropion, F=feathery margins. The most powerful factors are diffuse growth pattern and hyphema. Tumor seeding into the anterior chamber angle and onto the iris stroma are also important. The nonmelanocytic iris tumors are relatively uncommon and included categories of choristomatous, vascular, fibrous, neural, myogenic, epithelial, xanthomatous, metastatic, lymphoid, leukemic, secondary, and non-neoplastic simulators. Overall, the most common diagnoses in a clinical series include nevus, IPE cyst, and melanoma. In summary, iris tumors comprise a wide spectrum including mostly iris nevus, IPE cyst, and iris melanoma. Risk factors estimating transformation of iris nevus to melanoma can be remembered by the ABCDEF guide.

14 Article Impact of uveal melanoma thickness on post-plaque radiotherapy outcomes in the prophylactic anti-vascular endothelial growth factor era in 1131 patients. 2020

Yang, Xiaolu / Dalvin, Lauren A / Mazloumi, Mehdi / Chang, Michael / Shields, Jerry A / Mashayekhi, Arman / Shields, Carol L. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. · Department of Ophthalmology, Shanghai General Hospital, Shanghai, China. · Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA. ·Clin Exp Ophthalmol · Pubmed #32277560.

ABSTRACT: IMPORTANCE: The impact of tumour thickness on radiation complications following plaque radiotherapy for uveal melanoma in the anti-vascular endothelial growth factor (VEGF) era remains unknown. BACKGROUND: To evaluate treatment outcomes following plaque radiotherapy and prophylactic intravitreal bevacizumab for uveal melanoma based on initial tumour thickness. DESIGN: This was a retrospective, interventional case series. PARTICIPANTS: Patients with uveal melanoma were included in this study. METHODS: A review of medical records was conducted of patients with uveal melanoma treated with plaque radiotherapy and prophylactic intravitreal bevacizumab from 7 July 2000 to 2 November 2018. MAIN OUTCOMES MEASURES: Radiation-related outcomes of cystoid macular oedema (CME), radiation maculopathy, papillopathy, retinopathy, iris neovascularization (NVI) and neovascular glaucoma (NVG) were compared based on tumour thickness (small [<3.0 mm] vs medium [3.1-8.0 mm] vs large [>8.0 mm]). RESULTS: Of 1131 eyes, 341 (30%) had small, 633 (56%) medium and 157 (14%) large melanoma. Comparison (small vs medium vs large) at 4 years following radiotherapy revealed large melanoma with greater Kaplan-Meier estimated risk of CME (37% vs 37% vs 63%, P < .001), earlier onset of CME (33 vs 26 vs 19 months, P < .001) and greater development of NVI (<1% vs 2% vs 13%, P < .001) and NVG (1% vs 2% vs 12%, P < .001). Radiation-induced maculopathy, papillopathy and retinopathy were not associated with tumour thickness. CONCLUSIONS AND RELEVANCE: Compared with small and medium uveal melanoma, large uveal melanoma demonstrated greater 48-month risk for CME, shorter time to CME onset and greater development of NVI and NVG following plaque radiotherapy and prophylactic intravitreal bevacizumab.

15 Article An Outcome Assessment of a Single Institution's Longitudinal Experience with Uveal Melanoma Patients with Liver Metastasis. 2020

Seedor, Rino S / Eschelman, David J / Gonsalves, Carin F / Adamo, Robert D / Orloff, Marlana / Amjad, Anjum / Sharpe-Mills, Erin / Chervoneva, Inna / Shields, Carol L / Shields, Jerry A / Mastrangelo, Michael J / Sato, Takami. ·Department of Medical Oncology, Sidney Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, PA 19107, USA. · Department of Radiology, Thomas Jefferson University, Philadelphia, PA 19107, USA. · Department of Pharmacology and Experimental Therapeutics, Thomas Jefferson University, Philadelphia, PA 19107, USA. · Oncology Service, Wills Eye Hospital, Philadelphia, PA 19107, USA. ·Cancers (Basel) · Pubmed #31906411.

ABSTRACT: There is no FDA-approved treatment for metastatic uveal melanoma (UM) and overall outcomes are generally poor for those who develop liver metastasis. We performed a retrospective single-institution chart review on consecutive series of UM patients with liver metastasis who were treated at Thomas Jefferson University Hospital between 1971-1993 (Cohort 1,

16 Article Choroidal amelanotic tumours: clinical differentiation of benign from malignant lesions in 5586 cases. 2020

Welch, R Joel / Newman, Jennifer H / Honig, Stephanie E / Mayro, Eileen L / McGarrey, Mark / Graf, Alexander E / Selzer, Evan B / Acaba-Berrocal, Luis A / Considine, Sean P / Malik, Kunal / Shields, Jerry A / Shields, Carol L. ·Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, USA. · Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, USA carolshields@gmail.com. ·Br J Ophthalmol · Pubmed #31023712.

ABSTRACT: PURPOSE: To investigate demographics and clinical features of patients with amelanotic choroidal tumours. DESIGN: Retrospective analysis. METHODS: Comparison of demographic and clinical features of various amelanotic choroidal tumours based on stratification by patient age, sex and tumour diameter. Included were all patients with amelanotic choroidal tumours evaluated on the Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, USA, over a 45-year time period. RESULTS: A total of 5586 amelanotic choroidal tumours in 4638 eyes of 4441 patients were included with a mean age at presentation of 58 years (median 60, range 0.1-100 years). Most patients were white (95%), female (56%) and with unilateral lesion (96%). By comparison, amelanotic melanoma presented at a younger mean age (57 years) compared with metastasis (60 years, p<0.001), nevus (61 years, p<0.001), lymphoma (65 years, p<0.001), sclerochoroidal calcification (70 years, p<0.001) and peripheral exudative haemorrhagic chorioretinopathy (80 years, p<0.001). Melanoma presented at an older mean age compared with osteoma (30 years, p<0.001), granuloma (42 years, p<0.001), haemangioma (49 years, p<0.001) and inflammatory choroidal lesions (49 years, p<0.001). Differences in race and sex were also seen between the various amelanotic choroidal lesions. With few exceptions, amelanotic melanoma had significantly larger basal diameter, greater thickness, more frequent association with subretinal fluid and more often ultrasonographically hollow, compared with other amelanotic choroidal lesions. CONCLUSION: Understanding the demographic and clinical features of amelanotic choroidal melanoma and other amelanotic lesions could lead to an earlier and more accurate diagnosis.

17 Article Malignant transformation of choroidal nevus according to race in 3334 consecutive patients. 2019

Marous, Charlotte L / Shields, Carol L / Yu, Michael D / Dalvin, Lauren A / Ancona-Lezama, David / Shields, Jerry A. ·Department of Ophthalmology, Ocular Oncology Service, Wills Eye Hospital, Jefferson University, Philadelphia, PA, USA. · Department of Ophthalmology, Ocular Oncology Service, Wills Eye Hospital, Jefferson University, Philadelphia, PA; Department of Ophthalmology, Ocular Oncology Service, Mayo Clinic, Rochester, MN, USA. ·Indian J Ophthalmol · Pubmed #31755445.

ABSTRACT: Purpose: To evaluate choroidal nevus demographics, clinical features, imaging features, and the rate of transformation into melanoma by race. Methods: In this observational case series,: There were 3334 participants (3806 choroidal nevi) at a single tertiary-referral center evaluated between January 2, 2007, and August 7, 2017. Retrospective chart and multimodal imaging review was performed. Patient demographics, tumor features, and outcomes were compared between different races using Chi-squared test, Fisher's exact test, t-test, and analysis of variance. The main outcome measure was clinical features of choroidal nevus and the rate of transformation into melanoma by race. Results: Of the 3334 patients, there were Caucasian (n = 3167, 95%) and non-Caucasian (n = 167, 5%). The non-Caucasian races included African-American (n = 27, <1%), Hispanic (n = 38, <1%), Asian (n = 15, <1%), Asian Indian (n = 2, <1%), Middle Eastern (n = 4, <1%), and unknown (n = 83, 3%). By comparison (Caucasian versus vs. non-Caucasian), there were differences in the mean age at presentation (61 vs. 56 years,P < 0.0001), female sex (63% vs. 52%,P < 0.01), dysplastic nevus syndrome (<1% vs. 1%,P < 0.01), and previous cutaneous melanoma (5% vs. 1%,P= 0.03). A comparison of tumor features revealed differences in presence of symptoms (12% vs. 20%,P < 0.01) and ≥3 nevi per eye (3% vs. <1%,P= 0.04). A comparison of imaging features showed no differences. A comparison of outcome of nevus transformation into melanoma revealed no difference (2% vs. 3%,P= 0.29). However, of those nevi exhibiting growth to melanoma, ultrasonographic hollowness was less frequent in Caucasians (29% vs. 67%,P= 0.04). Conclusion: In this analysis of 3334 patients with choroidal nevus, we found differences in the mean age of presentation, sex, dysplastic nevus syndrome, previous cutaneous melanoma, presence of symptoms, and multiplicity of nevus per eye by race. However, there was no difference in the rate of transformation into melanoma by race.

18 Article Amelanotic Ciliochoroidal Melanoma in a Patient with Oculocutaneous Albinism. 2019

Sivalingam, Meera D / Dalvin, Lauren A / Shields, Carol L / Mashayekhi, Arman / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. · Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA. ·Ocul Oncol Pathol · Pubmed #31049325.

ABSTRACT: To report a case of amelanotic ciliochoroidal melanoma in a patient with oculocutaneous albinism. A 76-year-old Caucasian male with a past medical history of oculocutaneous albinism and recurrent urothelial carcinoma was found to have a mass in the left eye, suspicious for ciliochoroidal melanoma. On examination, visual acuity was 20/400 in both eyes (OU). External examination showed iris transillumination defects. Funduscopic examination OU revealed blonde fundus, optic nerve hypoplasia, and foveal hypoplasia, confirmed on optical coherence tomography. Funduscopic examination of the left eye revealed an inferonasal amelanotic ciliochoroidal mass, measuring 12.0 mm × 13.0 mm × 8.8 mm. There was visible intrinsic tumor vasculature and overlying subretinal fluid. B-scan ultrasonography demonstrated a hypoechoic, dome-shaped mass. The clinical and imaging features were consistent with amelanotic ciliochoroidal melanoma. The patient was treated with iodine-125 plaque radiotherapy. At the 4-month follow-up, the tumor demonstrated regression from 8.8 mm to 3.2 mm in thickness. Despite the apparent lack of uveal pigmentation, patients with oculocutaneous albinism can develop uveal melanoma.

19 Article Combination of multimodal imaging features predictive of choroidal nevus transformation into melanoma. 2019

Dalvin, Lauren A / Shields, Carol L / Ancona-Lezama, David Arturo / Yu, Michael D / Di Nicola, Maura / Williams, Basil K / Lucio-Alvarez, J Antonio / Ang, Su Mae / Maloney, Sean M / Welch, R Joel / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. · Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA. · Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA carolshields@gmail.com. ·Br J Ophthalmol · Pubmed #30523045.

ABSTRACT: AIM: To characterise combinations of multimodal imaging risk factors and predictive value for choroidal nevus transformation into melanoma. METHODS: This is a retrospective review of multimodal imaging features for 3806 choroidal nevi from 1 January 2007 through 1 January 2017. Kaplan-Meier estimates and Cox regression analyses were used to calculate 5-year percentages of growth to melanoma and HR. RESULTS: Using multimodal imaging, six risk factors predictive of choroidal nevus transformation into melanoma were identified, namely tumour thickness >2 mm, subretinal fluid, symptoms of visual acuity loss to 20/50 or worse, orange pigment, hollow acoustic density and tumour largest basal diameter >5 mm. Kaplan-Meier 5-year estimated tumour growth was found in 1% of nevi with no risk factors, 11% (range 9%-37%) with one factor, 22% (12%-68%) with two factors, 34% (21%-100%) with three factors, 51% (0%-100%) with four factors and 55% (0%-100%) with five factors. HR for growth was 0.1 with no factor, 2.1-7.8 with one factor, 1.8-12.1 with two factors, 4.0-24.4 with three factors, 4.6-170.0 with four factors and 12.0-595.0 with five factors. The highest HR with each combination of two, three, four or five risk factors always included symptoms of visual acuity loss and orange pigment. CONCLUSION: Six risk factors for choroidal nevus transformation into melanoma by multimodal imaging have been identified. Risk for transformation into melanoma is 1% when no factors are present, and approaches 100% with specific combinations of three or more risk factors. Understanding how combinations of factors influence risk of transformation into melanoma can guide counselling and treatment decisions.

20 Article Visual Outcome and Millimeter Incremental Risk of Metastasis in 1780 Patients With Small Choroidal Melanoma Managed by Plaque Radiotherapy. 2018

Shields, Carol L / Sioufi, Kareem / Srinivasan, Archana / Di Nicola, Maura / Masoomian, Babak / Barna, Laura E / Bekerman, Vladislav P / Say, Emil A T / Mashayekhi, Arman / Emrich, Jacqueline / Komarnicky, Lydia / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. · Department of Radiation Oncology, Hahnemann University Hospital, Philadelphia, Pennsylvania. ·JAMA Ophthalmol · Pubmed #30267070.

ABSTRACT: Importance: Early detection of choroidal melanoma at a small tumor size is emphasized in the literature. However, there is little published information on the specific risks of plaque-irradiated small choroidal melanoma on visual acuity and metastasis. Objective: To analyze outcomes of plaque radiotherapy for small choroidal melanoma 3 mm in thickness or less. Design, Setting, and Participants: This retrospective noncomparative series at a tertiary referral center included 1780 consecutive patients who had received plaque radiotherapy treatment for small choroidal melanoma. Main Outcomes and Measures: Visual acuity outcomes and melanoma-associated metastasis, assessed by Kaplan-Meier analyses. Results: The mean (SD) patient age at melanoma diagnosis was 58 (14) years. Of 1780 patients, 908 were female (51.0%), and 1752 were white (98.4%). Visual acuity was 20/40 OU or better in 1276 of the patients (71.7%), and the mean (SD) visual acuity was 20/40 (20/50) OU (median, 20/30; range, 20/20 to counting fingers). The mean (SD) tumor basal dimension was 8.8 (2.9) mm (median, 8.0 mm; range, 2.0-20.0 mm) and mean (SD) tumor thickness was 2.6 (0.5) mm (median, 2.7; range, 0.2-3.4 mm). Mean (SD) distance to the foveola was 3.4 (3.9) mm and to the optic disc was 3.7 (3.7) mm. The Kaplan-Meier rate of visual acuity loss (≥3 Snellen lines) was 9.5% (95% CI, 8.2%-11.0%) at 1 year, 39.2% (95% CI, 36.5%-42.0%) at 5 years, and 48.9% (95% CI, 45.6%-52.3%) at 10 years, whereas poor visual acuity (≤20/200) was 7.1% (95% CI, 5.9%-8.4%) at 1 year, 38.2% (95% CI, 35.5%-41.1%) at 5 years, and 53.5% (95% CI, 50.1%-57.1%) at 10 years. Regarding melanoma-associated metastasis, the rate was 0.2% (95% CI, 0.09%-0.6%) at 1 year, 4.5% (95% CI, 3.4%-5.9%) at 5 years, and 8.8% (95% CI, 6.9%-11.1%) at 10 years. Using 1.0-mm thickness increments, the 10-year risk for metastasis was 25.0% (95% CI, 3.9%-87.2%) at 0-mm to 1.0-mm thickness, 5.9% (95% CI, 2.5%-13.5%) at 1.1-mm to 2.0-mm thickness, 8.1% (95% CI, 5.9%-11.0%) at 2.1-mm to 3.0-mm thickness, and 13.4% (95% CI, 8.7%-20.4%) at thicknesses greater than 3.0 mm. The greater relative risk (RR) for metastasis in thinnest tumors was 1.83 (95% CI, 1.09-3.07), which likely represented more aggressive diffuse (flat) melanoma. By multivariable analysis, clinical features predictive of melanoma-associated metastasis included increasing patient age (RR, 1.32 [95% CI, 1.07-1.63] per decade; P = .01), tumor diameter (RR, 1.15 [95% CI, 1.06-1.24] per mm; P < .001), tumor thickness (RR, 2.22 [95% CI, 1.22-4.05] per mm; P = .01), photopsia symptoms (RR, 2.45 [95% CI, 1.35-4.43]; P = .003), and prior treatment before plaque radiotherapy (RR, 3.31 [95% CI, 1.31-8.33]; P = .01). Conclusions and Relevance: This retrospective study suggests that small choroidal melanoma treated with plaque radiotherapy has a 10-year risk for visual acuity loss of 48.9% (95% CI, 45.6%-52.3%) and a 10-risk of systemic metastasis of 8.8% (95% CI, 6.9%-11.1%). In this analysis, each millimeter of increasing thickness and diameter contributed risk for metastatic disease.

21 Article Iris Melanoma in a Child Simulating Juvenile Xanthogranuloma. 2018

Shields, Jerry A / Shields, Carol L / Lally, Sara E / Milman, Tatyana / Eagle, Ralph C. ·Department of Ocular Oncology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA. · Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA. ·Middle East Afr J Ophthalmol · Pubmed #30122859.

ABSTRACT: An 8-year-old girl was referred for an amelanotic iris tumor believed to have enlarged slightly over 3 months, suspicious for juvenile xanthogranuloma (JXG). The affected right eye had a lightly pigmented, vascular iris mass measuring 6 mm in basal dimension and 2 mm in thickness. There were no feeder vessels, seeding, inflammatory cells, or cutaneous abnormalities. Diagnostic fine needle aspiration biopsy (FNAB) was performed, revealing spindle B melanoma cells that were immunoreactive for melanocytic markers HMB45 and Melan-A. Complete tumor resection by basal sector iridectomy was performed. Histopathology confirmed spindle B melanoma. At 14-years follow up, there has been no recurrence or metastasis and visual acuity remains 20/25. Iris melanoma can develop in children and clinically resemble nodular JXG.

22 Article Cytogenetic results of choroidal nevus growth into melanoma in 55 consecutive cases. 2018

Shields, Carol L / Pefkianaki, Maria / Mashayekhi, Arman / Shields, Jerry A / Ganguly, Arupa. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA. · Department of Genetics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA. ·Saudi J Ophthalmol · Pubmed #29755268.

ABSTRACT: Purpose: To investigate the cytogenetic results of choroidal nevus with photographically-documented transformation into choroidal melanoma. Methods: Retrospective analysis of 55 consecutive patients who underwent fine needle aspiration biopsy (FNAB) for DNA isolation and whole genome array based assay for chromosomes 3, 6, and 8 analysis prior to plaque radiotherapy. Tumors with abnormalities in chromosomes 3 and 8 were considered high-risk for metastasis. Results: At diagnosis of choroidal nevus the mean patient age was 57 years (median 57, range 10-83 years). All patients were Caucasian and 36 (65%) were female. At the time of nevus diagnosis, the mean tumor basal diameter was 7.4 mm (median 6.5, range 1.5-18.0 mm) and tumor thickness was 2.2 mm (median 2.2, range 0.5-3.9 mm). The mean interval between diagnosis of choroidal nevus and transformation into choroidal melanoma was 58 months (median 42, range 3-238 months). At the time of melanoma diagnosis, the mean tumor basal diameter was 9.7 mm (median 9.0, range 5.0-19.0) and tumor thickness was 3.5 mm (median 3.4, range 1.3-8.1). Cytogenetic analysis of FNAB-isolated melanoma revealed 25 patients (45%) with high-risk and 30 (55%) with low-risk cytogenetic findings. The rate of tumor growth into melanoma was inversely related to high-risk cytogenetic profile (p = 0.03) as those with fast transformation ≤ 1 year showed high-risk in 80% compared to those with slow transformation > 1 year whoshowed high-risk profile in only 38%. Fast transformation into melanoma conferred a relative risk (RR) of 2.116 for high-risk cytogenetic profile, compared to slow transformation. Conclusions: Choroidal nevus with rapid transformation into melanoma within 1 year is significantly more likely to demonstrate high-risk cytogenetic profile, at risk for metastatic disease, compared to those with slow transformation.

23 Article By Sleight of Hand, Prognosis Determined-Even for Small Choroidal Melanoma. 2018

Shields, Carol L / Mashayekhi, Arman / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. ·JAMA Ophthalmol · Pubmed #29596557.

ABSTRACT: -- No abstract --

24 Article Uveal Melanoma Associated With Myotonic Dystrophy: A Report of 6 Cases. 2018

Dalvin, Lauren A / Shields, Carol L / Pulido, Jose S / Sioufi, Kareem / Cohen, Victoria / Shields, Jerry A. ·The Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. · Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota. · Department of Molecular Medicine, Mayo Clinic, Rochester, Minnesota. · The Ocular Oncology Service, Moorfields Eye Hospital, London, England. · St Bartholomew's Hospital, London, England. ·JAMA Ophthalmol · Pubmed #29596556.

ABSTRACT: Importance: Patients with myotonic dystrophy (MD) have an increased risk of malignancy including uveal melanoma. This case series further explores the association between these 2 diseases. Objective: To describe a cohort of patients with uveal melanoma associated with MD, including a case of iris melanoma, and MD-associated uveal melanoma in relatives. Design, Setting, and Participants: Retrospective case series at 3 tertiary referral centers (Wills Eye Hospital, Philadelphia, Pennsylvania; Mayo Clinic, Rochester, Minnesota; and Moorfields Eye Hospital, London, England), between January 1, 2000, and August 31, 2017. The study included 6 patients with MD and uveal melanoma. Main Outcomes and Measures: Melanoma response to treatment and development of metastatic disease. Results: There were 6 patients, 4 men and 2 women, with MD and uveal melanoma. The mean patient age at melanoma diagnosis was 47 years (median, 43 years; range, 30-67 years), and the tumor involved the choroid in 5 patients (83%) and iris in 1 patient (17%). The diagnosis of MD was known since young adulthood in 2 patients (33%) and was discovered in adulthood in 4 patients (67%). The main clinical features of MD included muscle weakness (n = 5; 83%), myotonia (n = 4; 67%), polychromatic cataract (n = 4; 67%), complications with general anesthesia (n = 4; 67%), myalgia (n = 3; 50%), cardiac arrhythmia (n = 2; 33%), and frontal baldness (n = 2; 33%). Genetic testing revealed MD type 1 (4 of 4 tested patients), and 2 patients demonstrated positive family history of MD with classic clinical features and preferred no testing. Melanoma treatment included plaque radiotherapy (n = 4; 67%), photodynamic therapy (n = 1; 17%), and declined treatment (n = 1; 17%). At follow-up of 6, 6, 41, 42, and 87 months (5 patients), findings included melanoma regression (4 of 5 tumors), melanoma recurrence (1 of 5 tumors), and no metastatic disease (5 of 5 patients). Conclusions and Relevance: Six adult patients with MD demonstrated uveal melanoma involving the choroid or iris, emphasizing the association between these 2 diseases. Further research seems warranted to explore the pathogenesis of uveal melanoma in MD. These findings support the consideration of ophthalmic examination for uveal melanoma in patients with MD.

25 Article LARGE UVEAL MELANOMA (≥10 MM THICKNESS): Clinical Features and Millimeter-by-Millimeter Risk of Metastasis in 1311 Cases. The 2018 Albert E. Finley Lecture. 2018

Shields, Carol L / Sioufi, Kareem / Robbins, Justin S / Barna, Laura E / Harley, Maxwell R / Lally, Sara E / Say, Emil Anthony T / Mashayekhi, Arman / Shields, Jerry A. ·Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. ·Retina · Pubmed #29528980.

ABSTRACT: PURPOSE: To analyze the clinical features and rate of metastatic disease in eyes with large (≥10 mm thickness) uveal melanoma. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: There were 1,311 consecutive patients. METHODS: Retrospective medical chart review. MAIN OUTCOME MEASURES: Clinical features and rate of metastatic melanoma. RESULTS: Of 1,311 patients with large melanoma, the mean age was 59 years (median 60, range 6-98 years) and 95% were white. Mean tumor basal dimension was 17 mm (median 17, range 7-25 mm), and mean tumor thickness was 12 mm (median 12, range 10-24 mm). Mean distance to the foveola was 6 mm (median 6, range 0-19 mm) and to optic nerve was 6 mm (median 5, range 0-19 mm). Of all eyes, using Kaplan-Meier analysis, metastasis occurred in 11, 30, 45, and 52% at 1, 3, 5, and 7 years, respectively. According to tumor thickness (10.0-11.0, 11.1-12.0, 12.1-13.0, 13.1-14.0, 14.1-15.0, 15.1-16.0, and >16.0 mm), metastasis at 1 year was found in 7, 12, 13, 15, 18, 22, and 20%; metastasis at 3 years was 24, 27, 37, 35, 51, 69, and 57%; metastasis at 5 years was 38%, 42%, 56%, 48%, 61%, not available, and 66%; and metastasis at 7 years was 47%, 47%, 61%, 57%, 61%, not available, and 66%. Clinical features associated with fewer metastatic events included Bruch membrane rupture (7-year metastasis at 48%, P = 0.018) and macular location (7-year metastasis at 32%, P = 0.014), whereas those with worse outcome included extraocular extension (7-year metastasis at 79%, P < 0.001). There was no significant difference in rate of melanoma-related metastasis for patients treated with plaque radiotherapy versus enucleation. CONCLUSION: Large uveal melanoma demonstrates 7-year rate of metastasis at 52%, with generalized increasing risk per 1-mm or 2-mm thickness increments. Extraocular extension was associated with greater metastatic rate, whereas Bruch membrane rupture and macular location demonstrated lower rate.

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