Trisha Ortiz
Wills Eye Hospital
Thomas Jefferson University Hospitals
Drs. Jordan D. Deaner, James P. Dunn, Sara E. Lally, and Carol L. Shields moderated the 6th session of MACULA 2026 on Oncology and Uveitis.
Dr. Jay S. Duker of Tufts University opened the session with a memorable talk entitled “How to Save a Life – Retina Edition – A tribute to Jerry Shields, MD,” where he outlined lessons he has collected from his mentors over the years. Lesson #1 reminded us to always look thoroughly at the fellow eye before jumping straight to the pathology seen in the presenting eye. This was demonstrated by a case diagnosed with branch retinal vein occlusion (BRVO) in the left eye, in which mantle cell lymphoma was ultimately caught due to subtle findings only seen in the right eye. Lesson #2 stated that while common things are common, when something seems atypical, “look for stripes.” Dr. Duker presented a case referred for retinal detachment with findings unusual for a typical retinal detachment. Ultimately, the patient was diagnosed with a choroidal melanoma and treated accordingly. Lesson #3 reminded us to never hesitate to order ancillary tests, displayed by a case of branch retinal artery occlusion (BRAO) in which ancillary testing diagnosed giant cell arteritis. Lastly, Lesson #4, applicable beyond medicine, was “Don’t hesitate to ask a colleague or friend.” From trainees to attendings, these lessons resonated across all levels of practice and underscored important principles of retinal care.
Dr. David H. Abramson of Memorial Sloan Kettering spoke about the efficacy of high dose intravitreal topotecan (90-80 g) in treating recurrent retinal and subretinal retinoblastomas. Topotecan is a semisynthetic topoisomerase-1 (TOP1) inhibitor that targets dividing cells and has not been associated with an increased risk of secondary malignancies. Notably, he highlighted a recent practice shift from 90 g to 180 g over the last eight months, which has proven successful even after failure with topotecan 90 g. To date, Dr. Abramson reports no posterior segment toxicity or electroretinography (ERG) changes with high dose topotecan, suggesting it may serve as a good alternative to melphalan, an alkylating agent, which has been associated with retinal toxicity and ERG decline. In discussion with the audience, Dr. Abramson reviewed safety techniques utilized to prevent drug reflux and extraocular tumor seeding, including the application of cryotherapy to the conjunctiva prior to needle withdrawal.
Dr. Jasmine H. Francis of Memorial Sloan Kettering gave a talk entitled, “Use of Intraarterial Chemotherapy Beyond Retinoblastoma.” Intraarterial chemotherapy (IAC) offers the ability to deliver a high concentration of drug locally, but a low dose to the patient systemically. IAC, with melphalan, topotecan, or carboplatin, is a mainstay of retinoblastoma treatment. Through several cases, Dr. Francis demonstrated the utility of IAC beyond retinoblastoma treatment, especially when involving vital structures not amendable to surgery. IAC served as effective primary treatment in cases of orbital and uveal histiocytosis and orbital and intraocular medulloepithelioma. IAC with melphalan may also be useful as adjuvant therapy in high grade uveal lymphoma and lacrimal adenoid cystic carcinoma. Dr. Francis also showed a case of chiasmal nasopharyngeal squamous cell carcinoma in which IAC provided good palliative treatment. Some tumors, however, such as retinal hemangioblastoma and orbital uveal melanoma, showed some response but with recurrence. Despite cases of recurrence, this series demonstrates IAC is a promising treatment option beyond retinoblastomas where vision preservation can still be meaningful to patients.
Dr. Ivana Kim of Massachusetts Eye and Ear discussed the ongoing debate about the optimal therapy for primary vitreoretinal lymphoma. Due to the rarity of this condition, there is limited strong evidence to guide management. Dr. Kim outlines three main presentations of the disease: unilateral ocular, bilateral ocular, and ocular and central nervous system involvement (CNS). Across these presentations, there is no clear consensus on whether they require different regimens or should be managed equally aggressively. Treatment options involve intravitreal chemotherapy, ocular radiation, and systemic therapy. For unilateral disease, Dr. Kim suggests intravitreal therapy for prompt regression. For bilateral disease, consider combined systemic and ocular therapy with ocular induction first. However, it’s still unclear how long to continue ocular therapy. Additionally, while systemic therapy is indicated and prioritized in CNS disease, questions remain about additional ocular therapy and duration of its course. The role of autologous stem cell transplantation as a treatment option is still unsettled in this disease. Discussion with the moderators highlighted the existing need for collaborative, multicenter treatment protocols.
Dr. Thomas A. Albini from Bascom Palmer Eye Institute gave a talk titled “Management of Acute Retinal Necrosis in 2026.” Acute retinal necrosis (ARN) is a rare, severe inflammatory condition, often presenting as panuveitis and most commonly caused by Varicella-Zoster (VZV) or Herpes Simplex (HSV) viruses. In addition to induction of systemic antiviral therapy, Dr. Albini highlights that patients at his institution also receive four injections of intravitreal foscarnet or ganciclovir and topical or oral steroids. However, more data is still needed on the benefits of this added treatment. Retinal detachments are the main complication of ARN and occur in about 30-60% of cases with about a 64% single-surgery success rate at Bascom Palmer. In terms of early vitrectomy, current data has been unable to demonstrate any benefit. In a cohort of patients with more than five years follow up, Dr. Albini’s group noted that all ARN recurrences were in patients who never started or who stopped prophylactic antiviral therapy, suggesting the benefit of ongoing treatment, especially given the long-term safety of oral valacyclovir.
Dr. Douglas A. Jabs presented on the “Adalimumab versus conventional Immunosuppression for Uveitis (ADVISE) Trial.” This is an international, randomized two-arm comparative effectiveness trial comparing adalimumab (ADA) to conventional immunosuppression in patients with non-infectious intermediate, posterior, or panuveitis. The primary endpoint was successful corticosteroid sparing at six months. ADA was superior at six months (69% vs. 54%), but by one year the difference was no longer significant. Steroid discontinuation favored ADA at one year (55% vs. 40%). Visual acuity was similar between groups. A limitation of this study was that it only followed patients for one year. Additionally, there is no data on anti-adalimumab antibodies in this trial. In discussion with the audience, Dr. Jabs noted that due to this lack of data, he still typically starts treatment with conventional immunosuppression and then adds ADA secondarily.
Finally, Dr. Andre J. Witkin from Tufts University concluded the session with an informative update on the American Society of Retina Specialists (ASRS) Research and Safety in Therapeutics (ReST) Committee. Dr. Witkin outlines three main mechanisms to which physicians should report drug related adverse events: the Food and Drug Administration (FDA) MedWatch, pharmaceutical manufacturers, and to the ReST committee. Benefits of reporting to the ReST committee include less biased surveillance that aggregates cases, easier access to data and images, and community alerts. A key issue in 2024 was vial stopper “coring” in which rubber fragments are introduced when puncturing vials. Although there were no ophthalmic complications related to coring, clinicians should always inspect vials, avoid compromised medications, and use kit-supplied needles to reduce risk. He also noted ongoing reports of drug-associated intraocular inflammation (IOI) and retinal vasculitis, however, there have been no recent clusters or events requiring alerts. Dr. Witkin emphasized checking for inflammation before injections, obtaining ancillary imaging as needed, and remembering endophthalmitis can mimic vasculitis. Although ReST serves an important function to the retina community, it is not a substitute for FDA or manufacturer reporting.
