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Middle East African Journal of Ophthalmology Middle East African Journal of Ophthalmology
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   2018| April-June  | Volume 25 | Issue 2  
    Online since July 18, 2018

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Uveal metastasis: Clinical features and survival outcome of 2214 tumors in 1111 patients based on primary tumor origin
Carol L Shields, R Joel Welch, Kunal Malik, Luis A Acaba-Berrocal, Evan B Selzer, Jennifer H Newman, Eileen L Mayro, Alexandru B Constantinescu, Meredith A Spencer, Mark P McGarrey, Austen N Knapp, Alexander E Graf, Alex J Altman, Sean P Considine, Jerry A Shields
April-June 2018, 25(2):81-90
DOI:10.4103/meajo.MEAJO_6_18  PMID:30122853
PURPOSE: The purpose of this study is to evaluate patients with uveal metastasis based on primary tumor site. METHODS: Retrospective analysis from Wills Eye Hospital, Philadelphia, PA, USA, for uveal metastasis clinical features and outcomes based on the primary tumor site. RESULTS: There were 2214 uveal metastases diagnosed in 1111 consecutive patients. The demographics included mean age of 60 years (median 61 years), Caucasian race (88%), and female gender (64%). The tumor was unilateral (82%) and primary site was established before uveal metastasis (67%). The primary tumor originated in the breast (37%), lung (26%), kidney (4%), gastrointestinal (GI) tract (4%), cutaneous melanoma (2%), lung carcinoid (2%), prostate (2%), thyroid (1%), pancreas (1%), other sites (3%), and unknown (16%). Comparative analysis of the 5 most common primary sites (breast, lung, kidney, GI tract, and cutaneous melanoma), revealed metastasis at mean age (57, 62, 66, 61, 59 years), as unilateral tumor (74%, 86%, 85%, 93%, 85%), with mean number of metastasis/eye (1.9, 1.7, 1.0, 1.1, 2.0), and in females (99%, 46%, 26%, 25%, 30%). Choroidal metastases measured mean base (9.3, 10.2, 9.1, 11.0, 7.3 mm), mean thickness (2.4, 3.6, 4.4, 4.0, 2.9 mm), and demonstrated predominant color yellow (94%, 91%, 56%, 97%, 36%). Of the 769 patients with documented follow-up, mean patient survival was poor (22.2, 11.5, 8.6, 12.4, 11.4 months) and Kaplan–Meier analysis revealed 3-year survival (33%, 19%, 0%, 14%, 21%) and 5-year survival (24%, 13%, 0%, 14%, 21%). The worst survival was found in patients with pancreatic metastasis (mean 4.2 months) and best survival with lung carcinoid (92% at 5 years). CONCLUSION: In a tertiary referral service, uveal metastasis originates from cancer in the breast, lung, kidney, GI tract, cutaneous melanoma, or others. Overall prognosis is poor with 5-year survival at 23% and worst survival with pancreatic metastasis whereas best survival with lung carcinoid metastasis.
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Orbital metastases: When to suspect? When to biopsy?
Richard Cutler Allen
April-June 2018, 25(2):60-64
DOI:10.4103/meajo.MEAJO_93_18  PMID:30122850
Incidental orbital masses that are asymptomatic and appear benign are often observed without surgical intervention unless there is a clinical or radiographic change in the mass. There is a burgeoning population of cancer patients with incidental masses that have been detected while under surveillance for metastasis. This population of patients is growing due to a number of reasons, including more extensive imaging, an aging population, and more effective cancer treatments. Closer scrutiny should be applied to these patients, due to the possibility of the mass being an orbital metastasis. In addition, the approach to these patients may have implications regarding the adult patient without a cancer history who presents with a symptomatic orbital mass. The purpose of this paper is to explore the approach to the patient with and without a cancer history who presents with an orbital mass.
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Choroidal melanoma resection
Hany S Hamza, Abdelrahman M Elhusseiny
April-June 2018, 25(2):65-70
DOI:10.4103/meajo.MEAJO_73_18  PMID:30122851
Uveal melanoma is the most common intraocular malignant tumor, with the choroid being the most common site. Management of choroidal melanoma has evolved greatly over the past years. In the past, the conventional method of treatment was enucleation of the affected eye with a debate regarding the effect of enucleation to promote or prevent metastasis. However, nowadays, there are many therapeutic options available including plaque radiotherapy, proton beam radiotherapy, argon laser photocoagulation, transpupillary thermotherapy, stereotactic radiotherapy using gamma knife, enucleation, and surgical resection of the tumor whether through transscleral approach “Exoresection” or less commonly through internal resection approach “Endoresection.” The indications and complications of each technique are reviewed. Although radiotherapy is the primary treatment of choroidal melanoma in most ocular oncology centers, it is used as an adjuvant therapy in combination with surgical resection. Preoperative stereotactic radiotherapy before endoresection and brachytherapy to the surgical bed in both exo and endoresection can effectively decrease the recurrence rate. In this article, we will focus on surgical resection of choroidal melanoma whether endoresection or exoresection. We collected data published in indexed journals and related books.
  4,392 186 -
Characteristics and factors related to eyelid basal cell carcinoma in Saudi Arabia
Manar Al Wohaib, Reem Al Ahmadi, Dalal Al Essa, Azza Maktabbi, Rajiv Khandekar, Eman Al Sharif, Hind Al Katan, Silvana Artioli Schellini, Osama Al Shaikh
April-June 2018, 25(2):96-102
DOI:10.4103/meajo.MEAJO_305_17  PMID:30122855
PURPOSE: Data on basal cell carcinoma (BCC) from the Middle East are deficient. We present the features and management outcomes for BCC over the last 36 years in Saudi Arabia. SUBJECTS AND METHODS: This retrospective chart review included BCC patients diagnosed and treated at Saudi Arabia between 1980 and 2016. Data were collected on patient demographics, clinical and histopathological characteristics of the lesions, management, and follow-up. RESULTS: One hundred and twenty-six patients with BCC were included in this study. The incidence of BCC in Saudi Arabia is 0.8 cases a year. The median age of the patients was 71 years. BCC affected 58.9% of males. The lower lid was the most common site of occurrence (52.7%). Clinically, BCC was most commonly recognized as a mixed lesion (41.1%) and 50.4% were histologically nodular. Risk factors for poor prognosis included tumor localization in the medial aspect of the lid, tumor size > 5 mm, histological subtype being ulcerative or morphea forms, affected margins, and recurrent lesions. CONCLUSION: BCC is a rare condition in Saudi Arabia. The clinical features and histopathology of BCC in Saudi Arabia are similar to the patterns observed in other regions of the world. Early detection and timely management mitigates the extensive destructive ocular/orbital damage due to BCC and results in better patient outcomes.
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Staging uveal melanoma with whole-body positron-emission tomography/computed tomography and abdominal ultrasound: Low incidence of metastatic disease, high incidence of second primary cancers
Victoria M L Cohen, Efthymia Pavlidou, Joanna Costa, Amit K Arora, Teressa Szyszko, Mandeep S Sagoo, Peter Szlosarek
April-June 2018, 25(2):91-95
DOI:10.4103/meajo.MEAJO_96_18  PMID:30122854
PURPOSE: The purpose of this study was to report the results of staging primary uveal melanoma with whole-body (18) fluorodeoxyglucose (FDG) positron-emission tomography/computed tomography (PET/CT) and abdominal ultrasound. MATERIALS AND METHODS: From January 2012, patients with uveal melanoma over 4 mm in thickness were staged with FDG PET/CT and abdominal ultrasound. RESULTS: Over 2 years, 108 patients with medium-to-large melanoma underwent dual imaging. According to the tumor, node, and metastasis classification, there were 75% T3, 11% T2, and 14% T1 uveal melanomas. Only, three of 108 patients (2.8%) were found to have metastatic uveal melanoma. All three had liver metastases confirmed following biopsy; one of three had additional extrahepatic widespread metastases. In these three patients, liver findings using both imaging techniques were consistent in one patient. In the second case, abdominal ultrasound missed the diagnosis of metastatic disease; however, FDG PET/CT revealed intense metabolic activity of the liver. In the third case, PET/CT missed the liver metastases; however, this was identified on abdominal ultrasound. PET/CT identified incidental second primary malignancies in 10 patients (9%). Second malignancies were found in the lung, breast, colon, thyroid, and adrenal gland. Abdominal ultrasound detected benign hepatic abnormalities in 20 patients (18%). CONCLUSIONS: Whole-body PET/CT and abdominal ultrasound complement each other in the staging of uveal melanoma. Benign hepatic abnormalities found using ultrasound is common. Of importance, a second asymptomatic primary malignancy associated with uveal melanoma was detected almost one in 10 patients.
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Botryoid rhabdomyosarcoma of the conjunctiva in a young boy
Justin D Pennington, R Joel Welch, Sara E Lally, Jerry A Shields, Ralph C Eagle, Carol L Shields
April-June 2018, 25(2):111-114
DOI:10.4103/meajo.MEAJO_8_18  PMID:30122858
Primary conjunctival rhabdomyosarcoma (RMS) is a rare entity that can present with or without papillomatous features. A 5-year-old Asian boy was referred for a rapidly growing conjunctival tumor in the superior fornix of the left eye. Surgical excision yielded a 28 mm multilobulated papillomatous specimen that exhibited histopathologic and immunohistochemical features consistent with embryonal (botryoid) RMS. Molecular analysis revealed the absence of the PAX3/FOXO1 fusion gene, indicating favorable prognosis. After surgery, he was promptly treated with systemic chemotherapy and proton beam radiotherapy.
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Iris melanoma in a child simulating juvenile xanthogranuloma
Jerry A Shields, Carol L Shields, Sara E Lally, Tatyana Milman, Ralph C Eagle
April-June 2018, 25(2):115-117
DOI:10.4103/meajo.MEAJO_7_18  PMID:30122859
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.
  3,218 102 -
Hand-held optical coherence tomography monitoring of submillimeter retinoblastoma treated with indocyanine green-enhanced transpupillary therapy
Meredith A Spencer, R Joel Welch, Carol L Shields
April-June 2018, 25(2):108-110
DOI:10.4103/meajo.MEAJO_280_17  PMID:30122857
Over recent years, hand-held optical coherence tomography (HH-OCT) has become critical for retinoblastoma diagnosis and management. We report precise HH-OCT findings in a case of sub-millimeter retinoblastoma treated with foveal-sparing indocyanine green-enhanced transpupillary thermotherapy (ICG-TTT). A 2-month-old Caucasian female with bilateral Group B retinoblastoma showed two recurrent macular tumors in the right eye, demonstrating 88 μm and 37 μm of growth to 344 μm and 413 μm in thickness, respectively, on HH-OCT. Each was treated with additional intravenous chemotherapy and foveal-sparing ICG-TTT. Tumor regression to 154 μm and 224 μm was documented on HH-OCT and maintained on follow-up. HH-OCT is vital in confirming clinical findings and influencing management decisions in retinoblastoma. In this case, HH-OCT precisely documented submillimeter retinoblastoma recurrence and treatment response.
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Outcome analysis of visual acuity and side effect after ruthenium-106 plaque brachytherapy for medium-sized choroidal melanoma
Tahra AlMahmoud, Sean Quinlan-Davidson, Gregory R Pond, Jean Deschênes
April-June 2018, 25(2):103-107
DOI:10.4103/meajo.MEAJO_198_16  PMID:30122856
PURPOSE: The purpose of this study is to report on treatment outcomes for medium-sized choroidal melanoma treated with Ruthenium-106 (Ru-106) plaque brachytherapy. METHODS: A retrospective case series of 28 patients received Ru-106 brachytherapy treatment for choroidal melanoma. The prescribed tumor dose was 85 Gy to a depth of 5 mm. RESULTS: Median follow-up was 31.2 months. At 12 and 24-month postirradiation, the best corrected visual acuity ≥20/70 (LogMar ≥−0.54) was 53.8% and 64.2%, respectively. Median time to tumor regression was estimated to be 10 months (95% CI = 9–18 months), with 100% of response rate by 32 months. Radiation-induced side effects were limited, and there were no postradiation enucleations. CONCLUSIONS: The majority of patients maintained good visual acuity, with no enucleations and minimal side effects. In this cohort, the Ru-106 plaque brachytherapy proved to be an efficacious and safe treatment option for patients with medium-sized choroidal melanomas with a maximal tumor height of 5 mm.
  2,709 102 -
Ocular oncology: Saving sight, saving lives
Ahmet Kaan Gündüz
April-June 2018, 25(2):59-59
DOI:10.4103/meajo.MEAJO_158_18  PMID:30122849
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Idiopathic orbital inflammation: Review of literature and new advances
Yağmur Seda Yeşiltaş, Ahmet Kaan Gündüz
April-June 2018, 25(2):71-80
DOI:10.4103/meajo.MEAJO_44_18  PMID:30122852
Idiopathic orbital inflammation (IOI) is a benign inflammatory condition usually confined to the orbit but extraorbital extension can also occur. IOI has been classified into categories including anterior, diffuse, posterior or apical, myositis, and dacryoadenitis. Other rare types of IOI include periscleritis, perineuritis, and focal mass. Diagnosis is based on careful history, clinical findings, computed tomography, and magnetic resonance imaging findings. An orbital biopsy is usually done for accessible orbital lesions such as dacryoadenitis. For other types such as myositis and apical IOI where surgery is difficult or dangerous, orbital biopsy is not initially considered. The mainstay of therapy consists of systemic corticosteroids, but other options including external beam radiotherapy, antimetabolites, alkylating agents, T-cell/calcineurin inhibitors, lymphocyte inhibitors, tumor necrosis factor-α inhibitors, and surgical debulking have also been used.
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