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Annual Report 2019

Department of Ophthalmic Oncology

Shigenobu Suzuki, Hidetomo Izawa, Shuichi Sano

Introduction

 The Department of Ophthalmic Oncology is one of the rare groups specializing in ocular tumors, especially intraocular tumors. Recently, more than 70% of patients nationwide with retinoblastoma, which is the most frequent intraocular malignancy in childhood, and more than 50% of patients with choroidal melanoma, which is 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: (Tables 1, 2).

Table 1. Number of patients
Table 1. Number of patients

Table 1. Number of patients
Table 1. Number of patients(Full Size)

Table 2. Type of procedure
Table 2. Type of procedure

Table 2. Type of procedure
Table 2. Type of procedure (Full Size)

1. Retinoblastoma

 Unless the patient’s family has concerns about preserving the affected eye, if the eye has already suffered from complications such as secondary glaucoma or severe hemorrhage, or if extraocular extension of the retinoblastoma is strongly suspected, we can offer the family eye-preserving treatment. Initial systemic chemotherapy and additional local therapies, called “chemoreduction therapy”, comprise the main strategy. If the tumor is localized in the peripheral retina, plaque radiotherapy using ruthenium-106 is also available. Transpupillary thermotherapy or cryotherapy is also used in cases with localized small tumors. Patients with extraocular extension, recurrence or metastasis who need intensive systemic chemotherapy are treated with 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 of eye-preserving treatment with plaque radiotherapy is equivalent to that of enucleation (COMS, medium-sized tumor study). For localized tumors up to 5 mm thick, ruthenium-106 plaque radiotherapy is the first choice. In Japan, plaque radiotherapy is only available 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 and this is invariably fatal. Life-long follow-up with liver imaging is routinely conducted for our patients. Patients with liver and systemic metastases are treated by the Department of Dermatologic Oncology.

3. Ocular adnexal tumors

 The major orbital tumors include rhabdomyosarcoma in childhood, and cavernous hemangioma, lacrimal gland tumor, lymphoma, metastatic tumor, and orbital inflammatory disease 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 margin, and orbital exenteration is selected in cases of orbital invasion. Ocular adnexal lymphomas are treated 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 has been developed in our hospital since 1987, and has been modified and performed after 2009 in more than 20 countries.

 Direct injection of diluted melphalan into the vitreous cavity is performed for retinoblastoma eyes with vitreous seeding. Vitreous seeding can be cured for eyes with vitreous seeds after other treatment modalities, and about 65% of eyes were rescued using this strategy.

 The National Registry of Retinoblastoma in Japan was organized in 1975, and more than 3,000 patients are registered. We contribute to this registry as an administrator of personal data and check overlapping. 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 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 or genetic changes for eye tumors, especially retinoblastoma, choroidal melanoma, and ciliary body tumors.

 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 enlighten general ophthalmologists about these events.

Future prospects

 We plan to establish the multicenter study group for eye tumors to employ clinical studies, confirm the diagnostic criteria and guidelines, and clarify the carcinogenesis for eye tumors.

List of papers published in 2019

Journal

1.  Fabian ID, Abdallah E, Abdullahi SU, Abdulqader RA, Adamou Boubacar S, Ademola-Popoola DS, Adio A, Afshar AR, Aggarwal P, Aghaji AE, Ahmad A, Akib MNR, Al Harby L, Al Ani MH, Alakbarova A, Portabella SA, Al-Badri SAF, Alcasabas APA, Al-Dahmash SA, Alejos A, Alemany-Rubio E, Alfa Bio AI, Alfonso Carreras Y, Al-Haddad C, Al-Hussaini HHY, Ali AM, Alia DB, Al-Jadiry MF, Al-Jumaly U, Alkatan HM, All-Eriksson C, Al-Mafrachi AARM, Almeida AA, Alsawidi KM, Al-Shaheen AASM, Al-Shammary EH, Amiruddin PO, Antonino R, Astbury NJ, Atalay HT, Atchaneeyasakul LO, Atsiaya R, Attaseth T, Aung TH, Ayala S, Baizakova B, Balaguer J, Balayeva R, Balwierz W, Barranco H, Bascaran C, Beck Popovic M, Benavides R, Benmiloud S, Bennani Guebessi N, Berete RC, Berry JL, Bhaduri A, Bhat S, Biddulph SJ, Biewald EM, Bobrova N, Boehme M, Boldt HC, Bonanomi MTBC, Bornfeld N, Bouda GC, Bouguila H, Boumedane A, Brennan RC, Brichard BG, Buaboonnam J, Calderón-Sotelo P, Calle Jara DA, Camuglia JE, Cano MR, Capra M, Cassoux N, Castela G, Castillo L, Català-Mora J, Chantada GL, Chaudhry S, Chaugule SS, Chauhan A, Chawla B, Chernodrinska VS, Chiwanga FS, Chuluunbat T, Cieslik K, Cockcroft RL, Comsa C, Correa ZM, Correa Llano MG, Corson TW, Cowan-Lyn KE, Csóka M, Cui X, Da Gama IV, Dangboon W, Das A, Das S, Davanzo JM, Davidson A, De Potter P, Delgado KQ, Demirci H, Desjardins L, Diaz Coronado RY, Dimaras H, Dodgshun AJ, Donaldson C, Donato Macedo CR, Dragomir MD, Du Y, Du Bruyn M, Edison KS, Eka Sutyawan IW, El Kettani A, Elbahi AM, Elder JE, Elgalaly D, Elhaddad AM, Elhassan MMA, Elzembely MM, Essuman VA, Evina TGA, Fadoo Z, Fandiño AC, Faranoush M, Fasina O, Fernández DDPG, Fernández-Teijeiro A, Foster A, Frenkel S, Fu LD, Fuentes-Alabi SL, Gallie BL, Gandiwa M, Garcia JL, García Aldana D, Gassant PY, Geel JA, Ghassemi F, Girón AV, Gizachew Z, Goenz MA, Gold AS, Goldberg-Lavid M, Gole GA, Gomel N, Gonzalez E, Gonzalez Perez G, González-Rodrguez L, Garcia Pacheco HN, Graells J, Green L, Gregersen PA, Grigorovski NDAK, Guedenon KM, Gunasekera DS, Gündüz AK, Gupta H, Gupta S, Hadjistilianou T, Hamel P, Hamid SA, Hamzah N, Hansen ED, Harbour JW, Hartnett ME, Hasanreisoglu M, Hassan S, Hassan S, Hederova S, Hernandez J, Hernandez LMC, Hessissen L, Hordofa DF, Huang LC, Hubbard GB, Hummlen M, Husakova K, Hussein Al-Janabi AN, Ida R, Ilic VR, Jairaj V, Jeeva I, Jenkinson H, Ji X, Jo DH, Johnson KP, Johnson WJ, Jones MM, Kabesha TBA, Kabore RL, Kaliki S, Kalinaki A, Kantar M, Kao LY, Kardava T, Kebudi R, Kepak T, Keren-Froim N, Khan ZJ, Khaqan HA, Khauv P, Kheir WJ, Khetan V, Khodabande A, Khotenashvili Z, Kim JW, Kim JH, Kiratli H, Kivelä TT, Klett A, Komba Palet JEK, Krivaitiene D, Kruger M, Kulvichit K, Kuntorini MW, Kyara A, Lachmann ES, Lam CPS, Lam GC, Larson SA, Latinovic S, Laurenti KD, Le BHA, Lecuona K, Leverant AA, Li C, Limbu B, Long QB, López JP, Lukamba RM, Lumbroso L, Luna-Fineman S, Lutfi D, Lysytsia L, Magrath GN, Mahajan A, Majeed AR, Maka E, Makan M, Makimbetov EK, Manda C, Martín Begue N, Mason L, Mason JO 3rd, Matende IO, Materin M, Mattosinho CCDS, Matua M, Mayet I, Mbumba FB, McKenzie JD, Medina-Sanson A, Mehrvar A, Mengesha AA, Menon V, Mercado GJVD, Metsá MB, Midena E, Mishra DKC, Mndeme FG, Mohamedani AA, Mohammad MT, Moll AC, Montero MM, Morales RA, Moreira C, Mruthyunjaya P, Msina MS, Msukwa G, Mudaliar SS, Muma KI, Munier FL, Murgoi G, Murray TG, Musa KO, Mushtaq A, Mustak H, Muyen OM, Naidu G, Nair AG, Naumenko L, Ndoye Roth PA, Nency YM, Neroev V, Ngo H, Nieves RM, Nikitovic M, Nkanga ED, Nkumbe H, Nuruddin M, Nyaywa M, Obono-Obiang G, Oguego NC, Olechowski A, Oliver SCN, Osei-Bonsu P, Ossandon D, Paez-Escamilla MA, Pagarra H, Painter SL, Paintsil V, Paiva L, Pal BP, Palanivelu MS, Papyan R, Parrozzani R, Parulekar M, Pascual Morales CR, Paton KE, Pawinska-Wasikowska K, Pe'er J, Peña A, Peric S, Pham CTM, Philbert R, Plager DA, Pochop P, Polania RA, Polyakov VG, Pompe MT, Pons JJ, Prat D, Prom V, Purwanto I, Qadir AO, Qayyum S, Qian J, Rahman A, Rahman S, Rahmat J, Rajkarnikar P, Ramanjulu R, Ramasubramanian A, Ramirez-Ortiz MA, Raobela L, Rashid R, Reddy MA, Reich E, Renner LA, Reynders D, Ribadu D, Riheia MM, Ritter-Sovinz P, Rojanaporn D, Romero L, Roy SR, Saab RH, Saakyan S, Sabhan AH, Sagoo MS, Said AMA, Saiju R, Salas B, San Román Pacheco S, Sánchez GL, Sayalith P, Scanlan TA, Schefler AC, Schoeman J, Sedaghat A, Seregard S, Seth R, Shah AS, Shakoor SA, Sharma MK, Sherief ST, Shetye NG, Shields CL, Siddiqui SN, Sidi Cheikh S, Silva S, Singh AD, Singh N, Singh U, Singha P, Sitorus RS, Skalet AH, Soebagjo HD, Sorochynska T, Ssali G, Stacey AW, Staffieri SE, Stahl ED, Stathopoulos C, Stirn Kranjc B, Stones DK, Strahlendorf C, Suarez MEC, Sultana S, Sun X, Sundy M, Superstein R, Supriyadi E, Surukrattanaskul S, Suzuki S, Svojgr K, Sylla F, Tamamyan G, Tan D, Tandili A, Tarrillo Leiva FF, Tashvighi M, Tateshi B, Tehuteru ES, Teixeira LF, Teh KH, Theophile T, Toledano H, Trang DL, Traoré F, Trichaiyaporn S, Tuncer S, Tyau-Tyau H, Umar AB, Unal E, Uner OE, Urbak SF, Ushakova TL, Usmanov RH, Valeina S, van Hoefen Wijsard M, Varadisai A, Vasquez L, Vaughan LO, Veleva-Krasteva NV, Verma N, Victor AA, Viksnins M, Villacís Chafla EG, Vishnevskia-Dai V, Vora T, Wachtel AE, Wackernagel W, Waddell K, Wade PD, Wali AH, Wang YZ, Weiss A, Wilson MW, Wime ADC, Wiwatwongwana A, Wiwatwongwana D, Wolley Dod C, Wongwai P, Xiang D, Xiao Y, Yam JC, Yang H, Yanga JM, Yaqub MA, Yarovaya VA, Yarovoy AA, Ye H, Yousef YA, Yuliawati P, Zapata López AM, Zein E, Zhang C, Zhang Y, Zhao J, Zheng X, Zhilyaeva K, Zia N, Ziko OAO, Zondervan M, Bowman R. Global Retinoblastoma Presentation and Analysis by National Income Level. JAMA Oncol, 6:1-12, 2020

2.  Ueda T, Koga Y, Yoshikawa H, Tanabe M, Yamana K, Oba U, Nakashima K, Ono H, Ichimura T, Hasegawa S, Kato W, Kobayashi T, Nakayama H, Sakai Y, Yoshitake T, Ohga S, Oda Y, Suzuki S, Sonoda KH, Ohga S. Survival and ocular preservation in a long-term cohort of Japanese patients with retinoblastoma. BMC Pediatr, 20:37, 2020