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There are a diverse range of cancers that can arise in the eyes. The two most common types are intraocular melanoma and retinoblastoma.

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Ocular Melanoma

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    MeSH term: Eye Neoplasms
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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Al-Haddad C, El Salloukh NA, El Moussawi Z
β-blockers in the treatment of periocular infantile hemangioma.
Curr Opin Ophthalmol. 2019; 30(5):319-325 [PubMed] Related Publications
PURPOSE OF REVIEW: To evaluate the medical literature on the use of β-blockers, through different routes, for the treatment of periorbital infantile hemangiomas and to summarize the recommendations available on dosage and monitoring.
RECENT FINDINGS: β-blockers for the treatment of infantile hemangioma are now considered to be first-line treatment. Growing literature on the role of oral propranolol confirmed its efficacy but also presented its multiple side-effects including hypotension, bradycardia, hypoglycemia, and bronchospasm. No universal guidelines exist concerning pretreatment evaluation, dosage, monitoring, and duration of treatment but different protocols have been created.In the aim of minimizing side-effects, other routes of administration and more selective β-blockers have emerged. Many studies showed promising results for topical timolol especially in the treatment of superficial hemangiomas. Few studies evaluated intralesional propranolol. Limited data exist on the use of more selective β-blockers promising similar results to propranolol with fewer side-effects.
SUMMARY: Oral β-blockers are now the mainstay of treatment for periorbital hemangiomas but still with no consensus on their administration and monitoring. The topical form or more selective β-blockers may be the solution to minimize side-effects.

Kim JY, Lee MY, Lee YC, Shin HY
Neurofibromatosis type 1 with tarsal conjunctiva thickening: A case report.
Medicine (Baltimore). 2019; 98(31):e16699 [PubMed] Related Publications
RATIONALE: We report a rare case of neurofibroma in the form of tarsal conjunctival thickening of the eyelid in patients with neurofibromatosis type 1 (NF1), common ocular complications of which are Lisch nodules, choroidal nodules, and optic nerve glioma.
PATIENT CONCERNS: A 46-year-old female patient was diagnosed with neurofibroma after biopsy and removal of 2 lumbar level intradural masses 15 years ago. She was being monitored without recurrence. When the patient visited our hospital, multiple iris Lisch nodules were found in both her eyes with ill-defined, diffuse thickening in the upper eyelid tarsal conjunctiva of the right eye.
DIAGNOSIS: Neurofibroma was diagnosed by incisional biopsy and immunohistochemistry of the tarsal conjunctiva.
INTERVENTIONS: The patient of the present case did not undergo any additional surgical treatment because tarsal conjunctiva thickening caused little functional problem.
OUTCOMES: The patient has only been regularly examined for changes in size of neurofibroma, and there was no change in size over a 12-month period.
LESSONS: Neurofibroma should be considered as a differential diagnosis if a patient diagnosed with NF1 shows tarsal conjunctiva thickening.

Welch RJ, Malik K, Considine SP, et al.
Uveal Metastasis Based on Patient Sex in 2214 Tumors of 1111 Patients. A Comparison of Female Versus Male Clinical Features and Outcomes.
Asia Pac J Ophthalmol (Phila). 2019 Jul-Aug; 8(4):298-303 [PubMed] Related Publications
BACKGROUND: Lacking in previous studies on uveal metastasis is a robust statistical comparison of patient demographics, tumor features, and overall survival based on patient sex.
OBJECTIVE: The aim of this study was to evaluate demographics, clinical features, and overall survival of patients with uveal metastasis based on sex.
METHOD: This is a retrospective analysis. All patients were evaluated on the Ocular Oncology Service, Wills Eye Hospital, PA between January 1, 1974 and June 1, 2017.
RESULTS: A total of 2214 uveal metastases were diagnosed in 1310 eyes of 1111 consecutive patients. A comparison (female versus male) revealed differences across several demographic and clinical features including, among others, mean age at metastasis diagnosis (58 vs 63 years, P < 0.001), bilateral disease (21% vs 11%, P < 0.001), and mean number of metastases per eye (1.8 vs 1.6 tumors per eye, P = 0.04). There were differences in overall mean survival (20 vs 13 months, P = 0.03) and 5-year survival (Kaplan-Meier estimate) (31% vs 21%, P < 0.001).
CONCLUSIONS: There are demographic, clinical, and survival differences when patients with uveal metastases are compared by sex. Understanding these differences can aid the clinician in better anticipating patient outcomes.

Silverstein M, Salvin J
Ocular manifestations of Sturge-Weber syndrome.
Curr Opin Ophthalmol. 2019; 30(5):301-305 [PubMed] Related Publications
PURPOSE OF REVIEW: Sturge-Weber syndrome (SWS) is a rare, congenital disease which frequently involves the eye. It is important that ophthalmologists recognize this syndrome and are aware of its range of ocular manifestations. The aim of this article is to present our understanding of the pathogenesis and clinical manifestations of this syndrome and provide updated information on the treatment of SWS glaucoma and choroidal hemangioma.
RECENT FINDINGS: SWS glaucoma usually fails medical management. Surgical options include angle procedures, filtering procedures, device placement, and combination procedures. Combination procedures have become popular in this population due to the single procedure failure rate of angle surgery and the complications associated with device implantation. Choroidal hemangioma is best treated by photodynamic therapy.
SUMMARY: Lifelong monitoring for ocular complications related to SWS is essential. There is a need for consensus guidelines on care and surveillance of patients with SWS to provide the best care for these patients.

Grumet P, Kerever S, Gilbert T, et al.
Clinical and etiologic characteristics of de novo uveitis in patients aged 60 years and above: experience of a French tertiary center.
Graefes Arch Clin Exp Ophthalmol. 2019; 257(9):1971-1979 [PubMed] Related Publications
PURPOSE: To describe the characteristics of de novo uveitis in patients ≥ 60 years old.
METHODS: Retrospective review of patients with uveitis followed in our tertiary center over a 14-year period. Patients aged 60-70 years and patients aged > 70 years were compared.
RESULTS: A total of 283/1044 (27.1%) patients with uveitis were ≥ 60 years of age. Idiopathic uveitis (36.1%) and sarcoidosis (31.5%) were the most frequent etiologies. Sarcoidosis was significantly more frequent (31.5% vs. 13.7%, p < 0.0001) after the age of 60 years. Intraocular lymphoma (5.0% vs. 1.1%) and herpes virus infection (5.0% vs. 0.9%) were also more common in this age group, unlike HLA B27-related uveitis and spondyloarthritis (4.6% vs. 14.9%). Pure ophthalmologic entities: birdshot retinochoroidopathy (2.8%) or Fuchs uveitis (0.4%), were rare in patients ≥ 60 years of age and Posner Scholssman, Pars planitis, White dots syndrome, Behçet's disease, and Multiple Sclerosis were never reported. In patients > 70 years old, idiopathic uveitis (41.1% vs. 31.7%) and presumed sarcoidosis (56.5% vs. 25.6%) were more frequent than in the 60-70-year age group.
CONCLUSION: In our center, sarcoidosis is the leading cause of non-idiopathic uveitis in older patients. Idiopathic uveitis and other entities account for less than two-thirds of cases. Ophthalmologic entities are rare after 60 years of age. We also report for the first time the characteristics of uveitis after 70 years of age.

Williams BK, Elimimian EB, Shields CL
Asteroid Hyalosis Simulating Vitreous Seeds in a Patient With Retinoblastoma.
J Pediatr Ophthalmol Strabismus. 2019; 56:e41-e44 [PubMed] Related Publications
A patient treated for retinoblastoma developed vitreous floaters 15 years later and was referred for recurrence with vitreous seeding. Clinical examination demonstrated a regressed scar and numerous calcified vitreous opacities with a "clear zone" on ultrasonography. The final diagnosis was asteroid hyalosis in an eye with regressed retinoblastoma. [J Pediatr Ophthalmol Strabismus. 2019;56:e41-e44.].

Yabaş Kızıloğlu Ö, Paksoy Türköz F, Totuk Gedar ÖM, et al.
Breast Carcinoma Metastasis to the Medial Rectus Muscle: Case Report
Turk J Ophthalmol. 2019; 49(3):168-170 [PubMed] Free Access to Full Article Related Publications
A 63-year-old woman with metastatic breast carcinoma presented to the ophthalmology clinic with diplopia and right abduction deficit. Magnetic resonance imaging showed isolated enlargement of the right medial rectus muscle. Biopsy of the enlarged muscle revealed metastasis of breast carcinoma. Ocular motility deficit in a patient with breast carcinoma should raise suspicion of metastasis to the orbit involving the extraocular muscles. Orbital imaging and biopsy are necessary for diagnosis and appropriate treatment.

Hashemi S, Aghamiri MR, Kahani M, Jaberi R
Investigation of gold nanoparticle effects in brachytherapy by an electron emitter ophthalmic plaque.
Int J Nanomedicine. 2019; 14:4157-4165 [PubMed] Free Access to Full Article Related Publications

Krásný J, Šach J, Kolín V, Zikmund J
Merkel Cell Carcinoma of the Eyelids (Clinical-Histological Study).
Cesk Slov Oftalmol. 2019; 74(5):198-205 [PubMed] Related Publications
AIM: To get acquainted with the issues of Merkel cell carcinoma (MCC) when the eyelid is affected and with its surgical solution.
MATERIALS: MCC of the right upper eyelid was at the Department of Ophthalmology in the Faculty Hospital Královské Vinohrady in Prague (Czech Republic, EU) in 1998 woman 78 year. Another cases of the MCC on the left upper eyelid were observed in two women aged 48 and 67 years and they were pick up in a retrospective study covered 1033 operated tumors eyelids years 2007 - 2015. 47,5% of operated tumors were benign and 52,5% malignant. Most common malignant processes were basal cell carcinoma in 77,3% and squamous cell carcinoma at 15,7%.
RESULTS: The oldest patient died after three years of cardiopulmonary failure. 48 year old patient (age of diagnosis MCC) has been monitoring for five years without proven recurrence or metastasis dissemination. Oncological initial staging was negative regarding. An ultrasound examination of the lymphatic system of the neck was followed every six months. Another 67 year old patient (age of diagnosis MCC) was followed for 2,5 years. There was a suspicion of a metastatic process in the same side salivary gland and lungs, therefore chemotherapy was performed. Definitely, this process has not been proven. Now there is continuing follow up without sings of local recurrence or metastatic dissemination.
CONCLUSION: The authors confirmed a rare occurrence of MCC, and only 0,37% among malignant eyelids tumors. Essential importance for successful treatment is a sufficiently radical excision supported by histological verification and a subsequent plastic surgery of the eyelid is also necessary. Key words: Merkel cell carcinoma, tumor of eyelid, ultrasound examination of nodes.

Lam SC, Yuen HKL
Medical and sclerosing agents in the treatment of orbital lymphatic malformations: what's new?
Curr Opin Ophthalmol. 2019; 30(5):380-385 [PubMed] Related Publications
PURPOSE OF REVIEW: Currently, there is no ideal management for orbital lymphatic malformations. Significant advances have been made since the discovery of new agents in the treatment. The purpose of this manuscript is to review the recent evidence on new sclerotherapy agents and systemic medications.
RECENT FINDINGS: Traditional sclerosants are OK-432, sodium tetradecyl sulphate and ethanol. More recent developments are the use of doxycycline, bleomycin, and pingyangmycin. Sirolimus as a systemic medication has revolutionized the medical management of lymphatic malformations. Other oral drugs such as propranolol and sildenafil are controversial. Future treatment involves targeting lymphangiogenic pathways including inhibition of vascular endothelial growth factors and the phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit.
SUMMARY: The development of new agents allows multimodal management either as monotherapy or combined therapy to achieve better outcomes in this difficult to manage disease.

Su FF, Chen JL
Expression and clinical significance of p16 and Ki-67 in malignant melanoma of the conjunctiva.
J Biol Regul Homeost Agents. 2019 May-Jun; 33(3):821-825 [PubMed] Related Publications

Ademola-Popoola DS, Opocher E, Reddy MA
Contemporary management of retinoblastoma in the context of a low-resource country.
Niger Postgrad Med J. 2019 Apr-Jun; 26(2):69-79 [PubMed] Related Publications
Retinoblastoma (RB) is the most common ocular cancer, and it typically presents before the age of 5 years in over 90% of cases. In high resource countries, RB patients tend to survive and retain their sight. This is not the case in low-resource countries because of late presentation and delayed intervention arising mostly from sociocultural and socioeconomic challenges. RB has no gender or racial predilection; the incidence is estimated as 1:16,000-1:18,000 live-births or 11/1 million children under 5 years. Most of the world's RB cases are found in Asia and Africa while most RB treatment centres are in America and Europe. RB is easy to detect by caregivers as a glowing white 'cat eye reflex' at night or when captured on camera. Health workers at primary care level can detect RB in early life if red reflex test and/or squint (Hirschberg) tests are deployed as part of wellness checks done especially during routine immunisation and well-baby clinics in the first 24 months of life. In most cases of RB, biopsies for histological confirmation are not required for diagnosis and treatment decisions to be made. Clinical information, ophthalmic evaluation and imaging modalities are typically used. There have been significant changes in the management of RB using various treatment modalities such as enucleation with orbital implant, use of chemotherapy delivered through intravenous, intravitreal, periocular and intra-arterial routes and targeted treatment with laser, cryotherapy and brachytherapy. Algorithm for management and development of the national RB program within the context of a low-resource country is presented from review of data extracted from Mendeley library, PubMed library, Google Scholar and One Network; full-text articles were mostly retrieved through the American Academy of Ophthalmology.

Habib LA, Wolkow N, Freitag SK, Yoon MK
Advances in Immunotherapy and Periocular Malignancy.
Semin Ophthalmol. 2019; 34(4):327-333 [PubMed] Related Publications
Immunotherapy has significantly advanced the field of oncology in recent decades. Understanding normal immunosurveillance, as well as the ways in which tumor cells have evolved to evade it, has provided the knowledge for development of drugs that allow one's own immune system to target and destroy malignant cells (immunotherapy). Cutaneous malignancies are particularly sensitive to this class of drugs. In a very sensitive anatomic region such as the periocular tissue, where surgical excision may come with significant morbidity, this technology has had a strong impact in the successful treatment of historically challenging tumors.

Yang J, Zhou C, Wang Y, et al.
Multimodal therapy in the management of lacrimal gland adenoid cystic carcinoma.
BMC Ophthalmol. 2019; 19(1):125 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: To evaluate the prognosis of Chinese patients with lacrimal gland adenoid cystic carcinoma treated with eye-sparing surgery and adjuvant multimodal therapy.
METHODS: The study included 24 consecutive patients with lacrimal gland adenoid cystic carcinoma treated at the Ninth People's Hospital of Shanghai from May 2008 to September 2017. All patients underwent eye-sparing surgical tumor resection and 20 (83.3%) of the 24 patients in the cohort received postoperative RT. Eight (41.7%) patients in the cohort received chemotherapy. Each patient's medical records were reviewed.
RESULTS: The study included 13 male and 11 female patients. The median follow-up time after surgery was 33.5 months. Fifteen (62.5%) patients experienced local recurrence. The 1-, 3-, and 5-year recurrence rates were 27.9, 60.0, and 80.0%, respectively. Eleven (45.8%) patients developed metastasis. The 1-, 3-, and 5-year metastasis rates were 8.7, 48.5, and 66.9%, respectively. Eight (33.3%) patients died of lacrimal gland adenoid cystic carcinoma, with a median survival duration of 34.0 months. The 1-, 3-, and 5-year tumor-related mortality was 4.5, 28.1, and 58.0%, respectively. More advanced T stage (≥ T3a) was a risk factor for local recurrence (hazard ratio [HR]: 5.374, P = 0.02), distant metastasis (HR: 8.585, P < 0.01), and tumor-related survival (HR: 9.654, P < 0.01).
CONCLUSIONS: Eye-sparing tumor resection protocol followed by adjuvant therapy seems to be associated with high rates of local recurrence, metastases and death. In addition, greater attention should be paid to patients with lacrimal gland adenoid cystic carcinoma with ≥ T3a tumors.

Rossi E, Pagliara MM, Orteschi D, et al.
Pembrolizumab as first-line treatment for metastatic uveal melanoma.
Cancer Immunol Immunother. 2019; 68(7):1179-1185 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: No standard treatment has been defined for metastatic uveal melanoma (mUM). Although clinical trials testing Nivolumab/Pembrolizumab for cutaneous melanoma did not include mUM, anti PD-1 agents are commonly used for this disease.
PATIENTS AND METHODS: In this prospective observational cohort single arm study, we investigated efficacy and safety of Pembrolizumab as first-line therapy for mUM. The efficacy was evaluated in terms of progression-free survival (PFS), response rate and overall survival (OS). Toxicity was also assessed.
RESULTS: Seventeen patients were enrolled. A median of 8 cycles were administered (range 2-28). Two patients achieved partial response (11.7%), 6 a disease stabilization (35.3%), whereas 9 (53%) had a progression. No complete response was observed. PFS of the overall population was 3.8 months. PFS was 9.7 months for patients with an interval higher than 5 years from diagnosis of primary tumor to metastatic disease and 2.6 months for patients with an interval lower than 5 years [p = 0.039, HR 0.2865 (95% CI 0.0869-0.9443)]. Median OS was not reached. The two responding patients were still on treatment with Pembrolizumab at the time of data analysis. Survival was 12.8 months for patients with clinical benefit, while OS for progressive patients was 3.1 months. PD-L1 expression and genomic abnormalities predictive of relapse after diagnosis of primary tumor were not associated with PFS. Toxicity was mild, without grade 3-4 side effects.
CONCLUSIONS: The efficacy of Pembrolizumab does not seem particularly different when compared to other agents for mUM, but responding patients had a remarkable disease control.

Artzner C, Mossakowski O, Hefferman G, et al.
Chemosaturation with percutaneous hepatic perfusion of melphalan for liver-dominant metastatic uveal melanoma: a single center experience.
Cancer Imaging. 2019; 19(1):31 [PubMed] Free Access to Full Article Related Publications
OBJECTIVE: To investigate the outcome and safety data of chemosaturation with percutaneous hepatic perfusion (CS-PHP) of melphalan in patients with liver-dominant metastatic uveal melanoma.
MATERIAL AND METHODS: This is a HIPAA compliant, IRB approved, retrospective study. A total of 28 CS-PHPs were performed in 16 individual patients (six men and ten women, median age 63.1 years [range 49.1 to 78.7 years], one to six CS-PHP procedures per patient) for treatment of liver-dominant metastatic uveal melanoma between June, 2015 and December, 2018. All patients received cross-sectional imaging at baseline and during follow-up. CS-PHP was performed with the Hepatic CHEMOSAT® Delivery System (Delcath Systems, Inc., NY, USA) facilitating extracorporeal filtration of hepatic blood for melphalan removal. Ideal body weight-adjusted melphalan doses were administered into the hepatic arteries. Serious adverse events (SAE), progression-free survival based on response criteria in solid tumors, and overall survival were noted. Survival data were analyzed using Kaplan-Meier estimates.
RESULTS: Partial response after first CS-PHP was observed in nine patients (60%), stable disease in five patients (33%) and progressive disease in one patient (7%). Median overall survival was 27.4 months (95% CI 4.1 to 35.4 month) after first CS-PHP. Median progression-free survival was 11.1 months after first CS-PHP (95% CI 4.9 to 23.6 months). SAEs were observed in the majority of patients with most SAEs limited to grades one and two. Thirteen SAEs of grades three and four were observed in seven individual patients. No grade five SAE was observed.
CONCLUSION: CS-PHP is an efficacious and safe treatment for patients presenting with liver-dominant metastatic uveal melanoma.

Cortes Barrantes P, Jakobiec FA, Dryja TP
A Review of the Role of Cytogenetics in the Diagnosis of Orbital Rhabdomyosarcoma.
Semin Ophthalmol. 2019; 34(4):243-251 [PubMed] Related Publications
Rhabdomyosarcoma (RMS) is the most common sarcoma of childhood and adolescence. Approximately 10% arise in the orbit, where the embryonal type is most common variant. The alveolar variant is less frequent and has a worse prognosis. Cytogenetic studies have revealed that most alveolar rhabdomyosarcomas have translocations involving the PAX and the FOX01 genes, giving rise to fusion genes that contribute to lack of differentiation and proliferation of the tumor cells. However, approximately 20% of alveolar rhabdomyosarcomas lack translocations and have been found to behave more similarly to embryonal cases. Histopathology remains the basis of diagnosis, but cytogenetic features and molecular signatures are becoming part of the routine analysis of RMS, since they determine not only prognosis, but also management and treatment regimens. A comprehensive review of the recent published literature in relation to orbital rhabdomyosarcomas and their cytogenetic features as well as clinical and therapeutic implications will be discussed.

Kaliki S, Vasanthapuram VH, Mishra DK
Conjunctival Melanoma in Asian Indians: A Study of 42 Patients.
Semin Ophthalmol. 2019; 34(3):182-187 [PubMed] Related Publications

Hung JY, Wei YH, Huang CH, et al.
Survival outcomes of eye-sparing surgery for adenoid cystic carcinoma of lacrimal gland.
Jpn J Ophthalmol. 2019; 63(4):344-351 [PubMed] Related Publications
PURPOSE: To survey adenoid cystic carcinoma of lacrimal glands in Asian population and investigate the predictability in prognosis following the 8
STUDY DESIGN: Retrospective study.
METHODS: The clinical entities and surgical outcomes of the patients who were histologically confirmed with a diagnosis of lacrimal adenoid cystic carcinoma in National Taiwan University Hospital between January 1995 and December 2015 were retrospectively reviewed.
RESULTS: Enrolled were 11 patients. The median follow-up was 7.2 years. Eight patients (72.7%) were diagnosed as T1 or T2 disease, and three patients (27.3%) were diagnosed as T3 or T4 disease according to the AJCC 8
CONCLUSION: This study highlighted the aggressive nature of adenoid cystic carcinoma of lacrimal glands. Eye-sparing surgery with adjunctive radiotherapy may achieve relatively optimal disease control in diseases staged T1 or T2, but in advanced disease metastasis and mortality are usually inevitable.

Tadepalli SH, Shields CL, Shields JA, Honavar SG
Intraocular medulloepithelioma - A review of clinical features, DICER 1 mutation, and management.
Indian J Ophthalmol. 2019; 67(6):755-762 [PubMed] Free Access to Full Article Related Publications
Intraocular medulloepithelioma is a nonhereditary neoplasm of childhood arising from primitive medullary epithelium. It most often involves the ciliary body. Most patients present between 2 and 10 years of age with loss of vision, pain, leucocoria, or conjunctival congestion. The mass appears as a grey-white ciliary body lesion with intratumoral cysts. Presence of a neoplastic cyclitic membrane with extension to retrolental region is characteristic. Secondary manifestations like cataract and neovascular glaucoma may be present in up to 50% and 60% patients, respectively. These could be the first signs for which, unfortunately, about 50% patients undergo surgery before recognition of the hidden tumor. Systemic correlation with pleuropulmonary blastoma (DICER1 gene) has been documented in 5% cases. Histopathology shows primitive neuroepithelial cells arranged as cords closely resembling the primitive retina. Histopathologically, the tumor is classified as teratoid (containing heteroplastic elements) and nonteratoid (containing medullary epithelial elements), each of which are further subclassified as benign or malignant. Retinoblastoma-like and sarcoma-like areas may be seen within the tissue. The treatment modality depends on tumor size and extent of invasion. For small localized tumors (≤3-4 clock hours), conservative treatments with cryotherapy, plaque radiotherapy, or partial lamellar sclerouvectomy (PLSU) have been used. Plaque brachytherapy is generally preferred for best tumor control. Advanced and extensive tumors require enucleation. Rare use of intra-arterial and intravitreal chemotherapy has been employed. Systemic prognosis is favorable, but those with extraocular extension and orbital involvement show risk for local recurrence and metastatic disease, which can lead to death.

Manjandavida FP, Stathopoulos C, Zhang J, et al.
Intra-arterial chemotherapy in retinoblastoma - A paradigm change.
Indian J Ophthalmol. 2019; 67(6):740-754 [PubMed] Free Access to Full Article Related Publications
Intra-arterial chemotherapy (IAC), also known as superselective ophthalmic artery chemotherapy or chemosurgery, is currently widely accepted as one of the primary treatment modalities for intraocular retinoblastoma worldwide. Following the introduction of the technique in 1998, IAC has evolved over the past decades to be safer and more effective. Accumulated evidence shows that IAC is more effective in providing eye salvage in group D and E retinoblastoma as compared to conventional systemic intravenous chemotherapy (IVC). In contrast to IVC, IAC has the added benefits of reduced overall treatment duration and minimal systemic toxicity. This review provides a comprehensive update on the history, technique, indications, contraindications, and outcome of IAC. We have also identified the strengths, weaknesses, opportunities and threats (SWOT analysis) of the technique in this review.

Nagesha CK, Ganne P
Combined Parafoveal Telangiectasia, Multifocal Central Serous Chorioretinopathy, and Choroidal Hemangioma.
Middle East Afr J Ophthalmol. 2019 Jan-Mar; 26(1):49-51 [PubMed] Free Access to Full Article Related Publications
The exact pathogenesis of parafoveal telangiectasia (PFT) is unknown, but various associations have been reported in literature such as diabetes and hypertension. Coexisting pathologies like diabetic retinopathy with PFT are not uncommon. We encountered a unique combination of PFT with multifocal central serous chorioretinopathy and choroidal hemangioma in a patient with diabetes mellitus. Multimodal imaging confirmed the findings and focal treatment to central serous chorioretinopathy leaks led to resolution of the subretinal fluid. This unique combination has been rarely reported so far. The long-term follow-up of such cases can help us to understand if there exists common pathogenesis of these varied diseases.

Choudhury AD, Beltran H
Retinoblastoma Loss in Cancer: Casting a Wider Net.
Clin Cancer Res. 2019; 25(14):4199-4201 [PubMed] Article available free on PMC after 15/01/2020 Related Publications
Capturing both genomic and nongenomic mechanisms of retinoblastoma gene dysfunction has potential to improve risk stratification and patient selection for biomarker-driven therapy. A 186-gene expression signature is capable of identifying Rb loss across cancer types, providing a new framework for assessing Rb dysfunction based on transcriptome data.

Brouwer NJ, Marinkovic M, Luyten GPM, et al.
Pigmentation of conjunctival melanoma recurrences and outcome.
Graefes Arch Clin Exp Ophthalmol. 2019; 257(8):1783-1788 [PubMed] Related Publications
PURPOSE: In primary conjunctival melanoma (CoM), one of the characteristics that is associated with an increased risk of metastases and death is a lack of tumour pigmentation. The aim of this study was to investigate whether the degree of pigmentation of CoM recurrences relates similarly to clinical outcome.
METHODS: A data set of 177 patients with a CoM recurrence from the Wills Eye Hospital (USA) and the Leiden University Medical Center (The Netherlands) was analysed. The relation between clinical tumour pigmentation of the recurrences, the characteristics of the primary lesions and clinical outcome was investigated.
RESULTS: In 117 (66%) of 177 patients with a CoM recurrence, tumour pigmentation was known: 71 patients (61%) had recurrences with low pigmentation. Primary lesions had low pigmentation in 39% of cases, which is significantly different (p = 0.001). However, low tumour pigmentation of recurrences correlated with low tumour pigmentation of the primary lesion (p < 0.001). No association was observed between pigmentation of the recurrences and iris colour (p = 0.66). Low pigmentation of the recurrences was not significantly associated with an increased risk for metastases (HR 1.96, p = 0.12) or death (HR 1.79, p = 0.27), whereas primary tumours with low pigmentation did show a greater risk for metastases (HR 2.82, p = 0.016) and death (HR 2.90, p = 0.037).
CONCLUSIONS: CoM recurrences are more often lightly pigmented compared to primary lesions. A correlation exists between the degree of pigmentation of primary and recurrent lesions, but recurrences can appear with any degree of pigmentation. Unlike primary CoM, the level of pigmentation of CoM recurrences is not related to metastasis or death.

Fallico M, Reibaldi M, Avitabile T, et al.
Intravitreal aflibercept for the treatment of radiation-induced macular edema after ruthenium 106 plaque radiotherapy for choroidal melanoma.
Graefes Arch Clin Exp Ophthalmol. 2019; 257(7):1547-1554 [PubMed] Related Publications
PURPOSE: To assess the efficacy of intravitreal aflibercept in patients suffering from post-radiation macular edema following plaque radiotherapy for choroidal melanoma.
METHODS: This prospective, interventional case series included patients affected by radiation maculopathy (RM) with macular edema secondary to ruthenium-106 plaque brachytherapy for choroidal melanoma. The effect of intravitreal aflibercept on best-corrected visual acuity (BCVA), central foveal thickness (CFT) detected by spectral domain optical coherence tomography (sd-OCT), and Horgan's grading scale of RM was evaluated throughout the 24-month follow-up. Intraocular pressure (IOP) and possible complications were also recorded.
RESULTS: Nine eyes of 9 patients were included. A mean of 4.4 ± 1.2 injections were given over the 24 months. At the end of follow-up, mean BCVA was significantly improved, from 0.9 ± 0.19 logMAR at baseline to 0.56 ± 0.3 logMAR (P = 0.028), and mean CFT was significantly decreased, from 546 ± 123 μm at baseline to 223 ± 34 μm (P < 0.001). Intravitreal aflibercept lowered baseline maculopathy stage as well. No significant change in IOP values and no complications, such as endophthalmitis, was recorded.
CONCLUSION: Intravitreal aflibercept is an effective treatment for patients with radiation-induced macular edema, allowing functional and anatomical improvements to be achieved with a relatively low number of injections.

Schefler AC, Koca E, Bernicker EH, Correa ZM
Relationship between clinical features, GEP class, and PRAME expression in uveal melanoma.
Graefes Arch Clin Exp Ophthalmol. 2019; 257(7):1541-1545 [PubMed] Related Publications
BACKGROUND: Metastatic risk for uveal melanoma (UM) patients can be characterized by gene expression profiling (GEP) (Castle Biosciences, Friendswood, TX). Class 1A tumors carry low metastatic risk; class 1B tumors have intermediate risk; and class 2 tumors have high risk. Preferentially expressed antigen in melanoma (PRAME) is a tumor-associated antigen which is expressed in various neoplasms including UM. Recently, PRAME expression in uveal melanoma was first recognized to confer an additional metastatic risk beyond GEP status.
METHODS: This was a retrospective, consecutive, multicenter chart review study. All patients diagnosed with UM at two major ocular oncology centers from August 2016 to February 2018 who underwent both GEP and PRAME mRNA expression testing were included. Patient age at diagnosis, gender, and tumor variables such as thickness, largest basal diameter (LBD), tumor volume, TNM stage, and GEP class and PRAME status were extracted from the medical records. Statistical analysis was performed to analyze the association of PRAME +/- status with all clinical and molecular variables.
RESULTS: One hundred forty-eight UM patients were identified. TNM was stage I in 51 (34.5%), stage IIA in 33 (22.3%), stage IIB in 34 (23%), stage IIIA in 20 (13.5%), and stage IIIB in 10 (6.8%) patients. Fifty-five patients (37%) were PRAME-positive, a significant fraction. There was no association between higher TNM stage and positive PRAME status (p = 0.129). PRAME expression was found to be independent of gender, patient age, and tumor thickness. PRAME expression was statistically associated with LBD and tumor volume. Higher GEP class was associated with higher TNM staging (p < 0.001). Worsening GEP class was associated with PRAME+ status with 28% of GEP class 1A tumors having PRAME+ status, 29% of GEP class 1B tumors having PRAME+ status, and 56% of GEP class 2 tumors having PRAME+ status.
CONCLUSIONS: In this study cohort, PRAME+ status was significantly associated with LBD and tumor volume as well as worsening GEP class. Nearly a third of GEP class 1A tumors expressed PRAME. Given the recent published data on increased metastatic risk among patients with tumors expressing PRAME, this study suggests that a significant fraction of 1A patients may harbor an increased metastatic risk. Future large, multicenter studies with long-term follow-up will clarify this finding.

Nava-Castañeda Á, Hernández-Orgaz J, Garnica-Hayashi L, et al.
Management of Ocular Surface Squamous Neoplasia with Topical and Intralesional Interferon Alpha 2B in Mexicans.
Nepal J Ophthalmol. 2018; 10(20):143-150 [PubMed] Related Publications
INTRODUCTION: Ocular surface squamous neoplasia (OSSN) is the most common nonpigmented ocular surface neoplasm and it can originate from the conjunctiva and/ or corneal epithelium. Since the rate of recurrence after surgical excision is high, chemotherapeutic and immunotherapeutic agents such as interferon alpha-2b (IFN α-2b) have been used for its treatment.
OBJECTIVE: Our objective is to describe the demographic variables of patients with OSSN treated with IFN α-2b and to describe the outcomes of its use in Mexican patients, treated between 2011 and 2017 at Instituto de Oftalmología Fundación Conde de Valenciana.
MATERIALS AND METHODS: This is a non-randomized retrospective interventional case series. We reviewed the files of patients older than 18 years, with clinical and/or histopathological diagnosis of OSSN, treated with IFN α-2b alone or in combination with surgery, with a minimum of 6 months follow-up. We studied demographic variables, such as gender, age, eye affected and associated risks factors; we also studied the type of therapy with interferon (topical, intralesional and/or associated with surgery), time of treatment, time of follow up, recurrences and complications.
RESULTS: A total of 39 patients were included. The mean age of diagnosis was 61 ± 16 years and OSSN was more frequent in males (64.1% of cases). Most of the cases (43.6%) had no identifiable risk factors. The mean time of IFN α-2b treatment was 5 ± 2 months, being higher in the intralesional group (6.5 months) than in the topical group (4.2 months). The clinical resolution with the use of IFN α-2b was evidenced between 0.5 and 10 months, being achieved in 87.1% of the patients.
CONCLUSIONS: The use of IFN α-2b is effective for the treatment of OSSN with a high success rate (87.1%) and a low incidence of relapse (5 cases), with no reported complications during follow up.

Lee J, Kwon HJ, Kim M, et al.
Treatment response to intravitreal bevacizumab in small pigmented choroidal lesions with subretinal fluid.
BMC Ophthalmol. 2019; 19(1):103 [PubMed] Article available free on PMC after 15/01/2020 Related Publications
BACKGROUND: To describe the effects of intravitreal bevacizumab injection (IVB) and/or transpupillary thermotherapy (TTT) in the treatment of small pigmented choroidal lesions with subfoveal fluid (SFF), and to investigate prognostic value of the therapeutic response in future tumor growth.
METHODS: Retrospective chart review of 19 patients, who were diagnosed with choroidal neovascularization (CNV)-free small pigmented choroidal lesions and treated with IVB and/or TTT, was performed.
RESULTS: Complete resolution of SFF was achieved in two eyes (2/14; 14.3%) after IVB, and in three eyes (3/4; 75%) after TTT. Best corrected visual acuity was improved in two eyes (2/9; 22%) after IVB, and in three eyes (3/4; 75%) after TTT. Among five patients who underwent TTT after IVB, four patients (4/5; 80%) demonstrated additional advantage. All IVBs could not reduce tumor sizes. Rather, tumor growth was detected in seven out of 14 eyes (7/14; 50%) that underwent IVB. None of the patients who underwent TTT showed tumor growth. The lack of treatment response to IVB was suggestive of malignancy, as most small pigmented lesions that had no response to IVB showed tumor growth (86%, p = 0.010).
CONCLUSION: IVB was not effective in reducing tumor size and subfoveal fluid in small pigmented choroidal lesions. Therapeutic response to IVB can be used as an indicator between melanoma and nevus in small pigmented choroidal lesion.

Xu H, Qiu Y, Wang X, et al.
Improved Tumor Resection on the Palpebral Margin.
J Craniofac Surg. 2019 May/Jun; 30(3):907-910 [PubMed] Related Publications
OBJECTIVE: To resect benign tumors on the palpebral margin using an improved minimally invasive surgery, and to observe the outcome and analyze the possible healing mechanism of this improved surgical technique.
METHODS: Fifty-five patients with a benign tumor on the palpebral margin measuring 2- to 10 mm in diameter were included in this study. The tumors were resected along their edge and basal layer, causing minimal damage to the surrounding structures. Postoperative outcome measures included the following: the wound status, epithelial healing, eyelash growth, recurrence, and complications. Postoperative follow-up time points were 1 day, 7 days, 14 days, 1 month, 3 months, and 12 months.
RESULTS: The wound status ranged from edema to contraction in the early stage postoperatively. The eyelashes that were excised during the operation began to grow from 7 to 14 days postoperatively, and the epithelium began to close simultaneously. From 14 days to 3 months postoperatively, the eyelash and epithelium grew completely, and the scar disappear gradually. Except in 1 patient with trichiasis at 3 months and one patient with tumor recurrence at 12 months postoperatively, there were no other complications, such as ablepharon deformities, alopecia palpebralis, etc. CONCLUSION:: Our improved minimally invasive technique not only resected the tumor effectively but also preserved the important anatomical structure of the palpebral margin. The favorable outcomes resulted from the mechanism of wound healing. This new surgical method is worth implementing in clinical practice.

Jespersen H, Bagge RO, Ullenhag G, et al.
Concomitant use of pembrolizumab and entinostat in adult patients with metastatic uveal melanoma (PEMDAC study): protocol for a multicenter phase II open label study.
BMC Cancer. 2019; 19(1):415 [PubMed] Article available free on PMC after 15/01/2020 Related Publications
BACKGROUND: While recent years have seen a revolution in the treatment of metastatic cutaneous melanoma, no treatment has yet been able to demonstrate any prolonged survival in metastatic uveal melanoma. Thus, metastatic uveal melanoma remains a disease with an urgent unmet medical need. Reports of treatment with immune checkpoint inhibitors have thus far been disappointing. Based on animal experiments, it is reasonable to hypothesize that the effect of immunotherapy may be augmented by epigenetic therapy. Proposed mechanisms include enhanced expression of HLA class I and cancer antigens on cancer cells, as well as suppression of myeloid suppressor cells.
METHODS: The PEMDAC study is a multicenter, open label phase II study assessing the efficacy of concomitant use of the PD1 inhibitor pembrolizumab and the class I HDAC inhibitor entinostat in adult patients with metastatic uveal melanoma. Primary endpoint is objective response rate. Eligible patients have histologically confirmed metastatic uveal melanoma, ECOG performance status 0-1, measurable disease as per RECIST 1.1 and may have received any number of prior therapies, with the exception of anticancer immunotherapy. Twenty nine patients will be enrolled. Patients receive pembrolizumab 200 mg intravenously every third week in combination with entinostat 5 mg orally once weekly. Treatment will continue until progression of disease or intolerable toxicity or for a maximum of 24 months.
DISCUSSION: The PEMDAC study is the first trial to assess whether the addition of an HDAC inhibitor to anti-PD1 therapy can yield objective anti-tumoral responses in metastatic UM.
TRIAL REGISTRATION: ClinicalTrials.gov registration number: NCT02697630 . (Registered 3 March 2016). EudraCT registration number: 2016-002114-50.

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