Eyeworld

20 January 2019 EW NEWS & OPINION Presentation spotlight by Stefanie Petrou Binder, MD, EyeWorld Contributing Writer forms of non-Hodgkin lymphoma carry a 55% and 9% 5-year survival rate, respectively. 4 Identifying the lymphoma type is important for effective treat- ment, as not all lymphomas are the same. "Surgical resection with radiotherapy is localized, but we will more often use chemotherapy and rituximab, my drug of choice," Dr. Shields said. "Rituximab treats the eye and all the hidden pockets of lymphoma systemically. We had great results with this new, very tar- geted anti-CD20 medication. Often tumors resolve within 2 months. We achieved 80% tumor control using rituximab and a 2-day regimen of low dose external beam radiation therapy. Rituximab also has good effects on choroidal melanoma." Target the mutation Choroidal melanoma is a serious malignancy with a high risk of metastasis and death. Thanks to advances in cytogenetics, physicians can now pinpoint the mutation in the melanoma and identify high/ low risk patients for metastasis. Mutations in chromosome 3 and 8 are associated with a higher rate for metastatic disease. According to a study Dr. Shields headed, 5 there is a correlation between larger tumor size category/higher number of mu- tations and a higher mutational pro- file by approximately 3- to 6-fold. "We put a needle in the tumor and get adequate DNA in 96% of cases, even in small/flat tumors. Using the DNA, we identify the mutation and give a quoted figure for what the risk for metastasis is. There are more than 50 genetic signatures with increasing risk for metastasis with uveal melanoma," she explained. Treatments for melanoma include systemic targeted drugs, vas- cularization blockade, and immuno- therapy. A new, innovative therapy currently in trial in the U.S. called light-activated nanoparticle AU-011 is showing promise. This new class of therapy selectively targets tumor cells. The technology involves virus-like nanoparticles injected into the eye that binds the heparin sul- fate matrix surrounding tumor cells. It is chromophore attached, laser light stimulated and causes precise tumor cell death. "This innovative therapy was tested in animal studies risk, unreliable parents, or when chemotherapy is not available. Although there is some agree- ment and disagreement on the best methods of treatment, IAC and IVC have completely replaced external beam radiotherapy and dimin- ished enucleations by 90% without evidence of compromising patient survival. 3 "What we did agree on is that we have made huge progress," Dr. Shields said. "The death rate in the U.S. from retinoblastoma is less than 2%. This is the number one most successfully treated cancer in children. IAC should be performed at an experienced center to avoid major risks." Lymphoma and rituximab Lymphoma is the fourth most common malignancy in the U.S., and its incidence is increasing by 4% every year. Lymphoma can be nodal (found in lymph nodes and the spleen) or extranodal elsewhere (found in conjunctiva, lid, orbit, uvea, vitreous, retina). In the eye, it typically presents as a salmon patch on the fornix bilaterally. Unilat- eral lymphoma has a 17% risk for systemic lymphoma, and bilateral lymphoma carries a 50% risk. According to a large, retrospec- tive observational case series, four important non-Hodgkin B cell sub- types were noted: extranodal mar- ginal zone (68%), follicular (16%), diffuse large B cell (5%), and mantle cell (7%). While the first two are low grade with a 5-year survival of 97% and 82%, respectively, the latter two carcinoma. In developed countries we prefer interferon. Although MMC is a good treatment usually given for a 1-month duration, it is toxic and can wipe out stem cells. 5-FU is the drug of choice in less developed nations because it is much more affordable, despite its toxicity to stem cells. In South America, it costs approximately $20 for a bottle of 5-FU compared to $200 for a bottle of interferon. Interferon is given from 3 to 6 months. It is nontoxic and has few complications. 1 Photo- dynamic therapy (PDT) is used for patients who fail all of the above." In a study she performed, Dr. Shields found that interferon was able to resolve squamous neoplasms completely within a few weeks, even in more advanced tumors with extensive disease. Interferon when appropriately combined with surgi- cal excision could provide complete control in 95% of cases. 2 Retinoblastoma When treating retinoblastoma, it is vital to balance systemic safe- ty, globe preservation, and visual potential. Dr. Shields distinguished between unilateral and bilateral treatments. For unilateral retinoblas- toma, intra-arterial chemotherapy (IAC) is her treatment of choice, which has reduced the percentage of eye enucleations to between 3% and 5%. For bilateral tumors, she uses intravenous chemotherapy (IVC). Enucleation is reserved for children in which there is little hope, high Oncology specialist elucidates the newest, most effective treatment methods for ophthalmic neoplasms O cular malignancies today have more sophisticated, nonsurgical treatments op- tions than ever before. Ac- cording to Carol Shields, MD, Ocular Oncology Service, Wills Eye Hospital, Philadelphia, who co-moderated a session on landmark advances in ophthalmology at the 2018 World Ophthalmology Con- gress, there have been tremendous advances in ocular cancer manage- ment. Conjunctival tumors as completely resolvable "According to a recent report on more than 5,000 conjunctival tumors in our practice, the most fre- quent conjunctival malignancies in an ocular oncology practice include ocular surface squamous neoplasias (14%), melanoma (12%), and lym- phoma (7%)," Dr. Shields said. "We deal with these in our center on a day to day basis." Conjunctival squamous cell carcinoma, also known as ocular surface squamous neoplasia, is a gelatinous fleshy vascular mass that can be extensive in size and rarely invades the eye. It tends to occur at the limbus, can present with feeder vessels and leukoplakia, and is usually non-pigmented. These malignancies frequently present in immunosuppressed patients with HIV or after organ transplantation. Other predisposing factors are heavy smoking, xeroderma pigmen- tosum, and autoimmune disease. The tumors can be large, recurrent, bilateral, invasive, and need to be followed lifelong. Dr. Shields advises prophylactic interferon to prevent cancer from developing if the tumor recurs in high risk patients. Treatment options for ocular surface squamous neoplasms are approached surgically and medically. Dr. Shields explained, "The nonsur- gical approach has been used for about 20 years and has been impres- sive. There are several alternatives for the treatment of squamous cell Advances in ocular oncology " Recognizing conjunctival tumors and understanding predisposing factors, biomarkers, and treatment strategies are vital to patient outcomes. These are exciting times in oncologic ophthalmology. " —Carol Shields, MD

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