Annual Report 2022
Department of Ophthalmic Oncology
Shigenobu Suzuki, Yi-Ning Chen, Shuichi Sano
Introduction
The Department of Ophthalmic Oncology is one of the few units specializing in ocular tumors, particularly intraocular tumors. Recently, more than 70% of patients nationwide with retinoblastoma, the most frequent intraocular malignancy in childhood, and approximately 30% of patients with choroidal melanoma, the most frequent primary intraocular malignancy in adults, have been referred to our department.
The Team and What We Do
Our outpatient service is open three days a week. Every week, eight operations under general anesthesia are performed in our department. Our treatment strategies for ocular tumors are as follows (see also Tables 1, 2):
1) Retinoblastoma
Unless the patient’s family has concerns about preserving the affected eye, unless the eye has already had complications such as secondary glaucoma or severe hemorrhage, or unless extraocular extension of the retinoblastoma is strongly suspected, we can offer eye-preserving
treatment. The primary strategy consists of initial systemic chemotherapy and additional local therapies, called “chemoreduction therapy”. For tumors located in the peripheral retina, plaque radiotherapy using ruthenium-106 is also available. Transpupillary thermotherapy and cryotherapy are also used in cases with localized small tumors. Patients with extraocular extension, recurrence or metastasis who need intensive systemic chemotherapy receive dedicated support from the Department of Pediatric Oncology.
2) Choroidal melanoma
Choroidal melanoma is a rare disease in Asians. Recent reports from Western countries have demonstrated that the prognosis for eye-preserving treatment with plaque radiotherapy is equivalent to that for enucleation (Collaborative Ocular Melanoma Study [COMS], a study on medium-sized tumors). For localized tumors up to 5 mm thick, ruthenium-106 plaque radiotherapy
radiotherapy is the first choice. In Japan, plaque radiotherapy is available only in our institute. Patients with much larger tumors are treated by radiotherapy: CyberKnife Robotic Radiosurgery in our institute or carbon ion therapy in the National Institute for Quantum and Radiological Science and Technology (QST). Choroidal melanomas often metastasize to the liver, which is invariably fatal. Our patients are routinely subjected to life-long follow-up with liver imaging. Patients with liver and systemic metastases are treated by the Department of Dermatologic Oncology, mainly with immune checkpoint inhibitors.
3) Ocular adnexal tumors
The major orbital tumors include rhabdomyosarcoma in childhood, and cavernous hemangioma, lacrimal gland tumor, lymphoma, and metastatic tumor in adulthood. The most common malignant eyelid tumors include basal cell carcinoma, sebaceous carcinoma, and squamous cell carcinoma. The most common malignant conjunctival tumors include squamous cell carcinoma and malignant melanoma. The principle of treatment is complete resection and reconstruction with safety margins, and orbital exenteration is selected in cases of orbital invasion. Ocular adnexal lymphomas are managed with radiotherapy, chemotherapy, or no-treatment (watchful waiting strategy), with dedicated support from the Department of Hematology.
Research Activities
One of the unique techniques in our department is local chemotherapy for retinoblastoma via selective ophthalmic artery infusion using a balloon catheter. This procedure was developed in our hospital in 1987, and since 2009, it has been adapted and implemented in more than 20 countries. Direct injection of diluted melphalan into the vitreous cavity is performed for retinoblastoma eyes with vitreous seeding. Approximately 65% of eyes are saved using these strategies.
The National Registry of Retinoblastoma in Japan was organized in 1975, and more than 3,300 patients are currently registered. We contribute to this registry as a committee chair. This registry now covers almost all patients in Japan, and provides epidemiological data.
We have constructed the national eye tumor registration system, called JCOTS (Japan Collaborative Ocular Tumor Study), in cooperation with the Japanese Society of Ocular Oncology. We contribute to this registry as an administrator.
We are now investigating the specific markers and genetic changes for eye tumors, especially retinoblastoma, choroidal melanoma, and eyelid sebaceous carcinoma.
Ocular adverse events caused by anti-cancer drugs used for systemic disease have been recently recognized, and ocular examinations are included in clinical trials, especially for molecular-targeted drugs. Serous retinal detachment (SRD), retinal vein occlusion (RVO), uveitis, and ocular surface complications are major adverse events caused by kinase inhibitor drugs, and stenosis or occlusion of lacrimal drainage systems are common events caused by S-1. We examine and follow these adverse events, with or without additional treatment, to support clinical trials, to contribute to establishing protocols, and to alert general ophthalmologists to these events.
Future Prospects
We are planning to establish selective ophthalmic artery infusion therapy for retinoblastoma in other hospitals to expand the treatment system in Japan. We also plan to establish the multicenter study group for eye tumors to employ clinical studies, confirm the diagnostic criteria and guidelines, and clarify the carcinogenesis of eye tumors.
List of papers published in 2022
Journal
1. Kodate A, Murakami N, Matsumoto Y, Honma Y, Miyakita Y, Suzuki S, Uno T, Igaki H. Iris Metastasis From Hypopharyngeal Carcinoma. Prac Radiat Oncol, 13:1-2, 2023
2. Nakagawa N, Morimoto T, Miyamura T, Suzuki S, Shimojo H, Nishida K. A case of retinoblastoma resulting in phthisis bulbi after proton beam radiation therapy. American journal of ophthalmology case reports, 28:101715, 2022
3. Takeda Y, Kaburaki T, Kakehashi A, Suzuki S. Granular cell tumour in the ciliary body. BMJ case reports, 15:e252691, 2022