Recent Developments in Ocular Oncology

  • Bertil DamatoEmail author


This article reviews recent developments in ocular oncology, focusing on uveal melanoma, retinoblastoma, choroidal metastasis, retinal lymphoma, conjunctival melanoma and conjunctival carcinoma. The management of patients with uveal melanoma has been revolutionized by studies revealing genetic predictors of metastatic disease. Ocular treatment continues to develop, so that vision is conserved more successfully. Treatment for radiation-induced ocular morbidity has become more effective thanks to anti-angiogenic agents and ablation or excision of the ‘toxic’ irradiated tumor. The management of patients with retinoblastoma has improved thanks to intra-arterial and intra-vitreal chemotherapy as well as advances in genetics. Choroidal metastases are increasingly diagnosed by biopsy. Novel anti-cancer therapies have resulted in a wide variety of novel adverse effects. The management of patients with vitreoretinal lymphoma has advanced with developments in imaging and laboratory analyses as well as immunotherapy and therapeutic vitrectomy. Conjunctial tumors are treated more successfully with greater use of adjunctive radiotherapy as well as topical therapy with interferon and chemotherapeutic agents. Patients with ocular malignancy are increasingly being treated in specialist ocular oncology centers because of greater awareness of the need for more holistic care, provided by experienced multidisciplinary teams.


Uveal melanoma Retinoblastoma Uveal metastasis Vitreoretinal lymphoma Drug-induced adverse events Conjunctival melanoma Conjunctival carcinoma 


  1. 1.
    Prescher G, Bornfeld N, Hirche H, Horsthemke B, Jockel KH, Becher R. Prognostic implications of monosomy 3 in uveal melanoma. Lancet. 1996;347:1222–5.PubMedGoogle Scholar
  2. 2.
    Dogrusoz M, Jager MJ. Genetic prognostication in uveal melanoma. Acta Ophthalmol. 2018;96:331–47.PubMedGoogle Scholar
  3. 3.
    Robertson AG, Shih J, Yau C, Gibb EA, Oba J, Mungall KL, Hess JM, Uzunangelov V, Walter V, Danilova L, Lichtenberg TM, Kucherlapati M, Kimes PK, Tang M, Penson A, Babur O, Akbani R, Bristow CA, Hoadley KA, Iype L, Chang MT, Network TR, Cherniack AD, Benz C, Mills GB, Verhaak RGW, Griewank KG, Felau I, Zenklusen JC, Gershenwald JE, Schoenfield L, Lazar AJ, Abdel-Rahman MH, Roman-Roman S, Stern MH, Cebulla CM, Williams MD, Jager MJ, Coupland SE, Esmaeli B, Kandoth C, Woodman SE. Integrative analysis identifies four molecular and clinical subsets in uveal melanoma. Cancer Cell. 2018;33:151.PubMedGoogle Scholar
  4. 4.
    Kivela TT, Piperno-Neumann S, Desjardins L, Schmittel A, Bechrakis N, Midena E, Leyvraz S, Zografos L, Grange JD, Ract-Madoux G, Marshall E, Damato B, Eskelin S. Validation of a prognostic staging for metastatic uveal melanoma: a collaborative study of the European Ophthalmic Oncology Group. Am J Ophthalmol. 2016;168:217–26.PubMedGoogle Scholar
  5. 5.
    Damato B, Duke C, Coupland SE, Hiscott P, Smith PA, Campbell I, Douglas A, Howard P. Cytogenetics of uveal melanoma: a 7-year clinical experience. Ophthalmology. 2007;114:1925–31.PubMedGoogle Scholar
  6. 6.
    Eleuteri A, Taktak AFG, Coupland SE, Heimann H, Kalirai H, Damato B. Prognostication of metastatic death in uveal melanoma patients: a Markov multi-state model. Comput Biol Med. 2018;102:151–6.PubMedGoogle Scholar
  7. 7.
    Kalirai H, Dodson A, Faqir S, Damato BE, Coupland SE. Lack of BAP1 protein expression in uveal melanoma is associated with increased metastatic risk and has utility in routine prognostic testing. Br J Cancer. 2014;111:1373–80.PubMedPubMedCentralGoogle Scholar
  8. 8.
    Smit KN, van Poppelen NM, Vaarwater J, Verdijk R, van Marion R, Kalirai H, Coupland SE, Thornton S, Farquhar N, Dubbink HJ, Paridaens D, de Klein A, Kilic E. Combined mutation and copy-number variation detection by targeted next-generation sequencing in uveal melanoma. Mod Pathol. 2018;31(5):763–71.PubMedGoogle Scholar
  9. 9.
    Kim RS, Chevez-Barrios P, Divatia M, Bretana ME, Teh B, Schefler AC. Yield, techniques, and complications of transvitreal and transscleral biopsies in small uveal melanoma. JAMA Ophthalmol. 2018;136(5):482–8.PubMedPubMedCentralGoogle Scholar
  10. 10.
    Angi M, Kalirai H, Taktak A, Hussain R, Groenewald C, Damato BE, Heimann H, Coupland SE. Prognostic biopsy of choroidal melanoma: an optimised surgical and laboratory approach. Br J Ophthalmol. 2017;101:1143–6.PubMedGoogle Scholar
  11. 11.
    Finn AP, Materin MA, Mruthyunjaya P. Choroidal tumor biopsy: a review of the current state and a glance into future techniques. Retina. 2018;38(Suppl 1):S79–87.PubMedGoogle Scholar
  12. 12.
    Bagger MM. Intraocular biopsy of uveal melanoma risk assessment and identification of genetic prognostic markers. Acta Ophthalmol. 2018;96(Suppl A112):1–28.PubMedGoogle Scholar
  13. 13.
    Hussain RN, Kalirai H, Groenewald C, Kacperek A, Errington RD, Coupland SE, Heimann H, Damato B. Prognostic biopsy of choroidal melanoma after proton beam radiation therapy. Ophthalmology. 2016;123:2264–5.PubMedGoogle Scholar
  14. 14.
    Wen JC, Sai V, Straatsma BR, McCannel TA. Radiation-related cancer risk associated with surveillance imaging for metastasis from choroidal melanoma. JAMA Ophthalmol. 2013;131:56–61.PubMedGoogle Scholar
  15. 15.
    Francis JH, Catalanotti F, Landa J, Barker CA, Shoushtari AN, Abramson DH. Hepatic abnormalities identified by staging MRI and accuracy of MRI of patients with uveal melanoma. Br J Ophthalmol. 2018. pii: bjophthalmol-2018-312612. [Epub ahead of print]PubMedGoogle Scholar
  16. 16.
    Marshall E, Romaniuk C, Ghaneh P, Wong H, McKay M, Chopra M, Coupland SE, Damato BE. MRI in the detection of hepatic metastases from high-risk uveal melanoma: a prospective study in 188 patients. Br J Ophthalmol. 2013;97:159–63.PubMedGoogle Scholar
  17. 17.
    Yang J, Manson DK, Marr BP, Carvajal RD. Treatment of uveal melanoma: where are we now? Ther Adv Med Oncol. 2018;10:1758834018757175.PubMedPubMedCentralGoogle Scholar
  18. 18.
    Damato B, Dukes J, Goodall H, Carvajal RD. Tebentafusp: T cell Redirection for the Treatment of Metastatic Uveal Melanoma. Cancers. (In Press).Google Scholar
  19. 19.
    Karydis I, Gangi A, Wheater MJ, Choi J, Wilson I, Thomas K, Pearce N, Takhar A, Gupta S, Hardman D, Sileno S, Stedman B, Zager JS, Ottensmeier C. Percutaneous hepatic perfusion with melphalan in uveal melanoma: a safe and effective treatment modality in an orphan disease. J Surg Oncol. 2018;117:1170–8.PubMedGoogle Scholar
  20. 20.
    Gomez D, Wetherill C, Cheong J, Jones L, Marshall E, Damato B, Coupland SE, Ghaneh P, Poston GJ, Malik HZ, Fenwick SW. The Liverpool uveal melanoma liver metastases pathway: outcome following liver resection. J Surg Oncol. 2014;109:542–7.PubMedGoogle Scholar
  21. 21.
    Valsecchi ME, Orloff M, Sato R, Chervoneva I, Shields CL, Shields JA, Mastrangelo MJ, Sato T. Adjuvant sunitinib in high-risk patients with uveal melanoma: comparison with institutional controls. Ophthalmology. 2018;125:210–7.PubMedGoogle Scholar
  22. 22.
    Damato B. Ocular treatment of choroidal melanoma in relation to the prevention of metastatic death—a personal view. Prog Retin Eye Res. 2018;66:187–99.PubMedGoogle Scholar
  23. 23.
    Collaborative Ocular Melanoma Study G. The COMS randomized trial of iodine 125 brachytherapy for choroidal melanoma: V. Twelve-year mortality rates and prognostic factors: COMS report No. 28. Arch Ophthalmol. 2006;124:1684–93.Google Scholar
  24. 24.
    Callejo SA, Dopierala J, Coupland SE, Damato B. Sudden growth of a choroidal melanoma and multiplex ligation-dependent probe amplification findings suggesting late transformation to monosomy 3 type. Arch Ophthalmol. 2011;129:958–60.PubMedGoogle Scholar
  25. 25.
    Damato BE, Heimann H, Kalirai H, Coupland SE. Age, survival predictors, and metastatic death in patients with choroidal melanoma: tentative evidence of a therapeutic effect on survival. JAMA Ophthalmol. 2014;132:605–13.PubMedGoogle Scholar
  26. 26.
    Reichstein D, Karan K. Plaque brachytherapy for posterior uveal melanoma in 2018: improved techniques and expanded indications. Curr Opin Ophthalmol. 2018;29:191–8.PubMedGoogle Scholar
  27. 27.
    Milam RW Jr, Batson SA, Breazzano MP, Ayala-Peacock DN, Daniels AB. Modern and novel radiotherapy approaches for the treatment of uveal melanoma. Int Ophthalmol Clin. 2017;57:11–27.PubMedGoogle Scholar
  28. 28.
    Le BHA, Kim JW, Deng H, Rayess N, Jennelle RL, Zhou SY, Astrahan MA, Berry JL. Outcomes of choroidal melanomas treated with eye physics plaques: a 25-year review. Brachytherapy. 2018;17:981–9.PubMedPubMedCentralGoogle Scholar
  29. 29.
    Russo A, Laguardia M, Damato B. Eccentric ruthenium plaque radiotherapy of posterior choroidal melanoma. Graefes Arch Clin Exp Ophthalmol. 2012;250:1533–40.PubMedGoogle Scholar
  30. 30.
    Damato BE, Stewart JM, Afshar AR, Groenewald C, Foulds WS. Surgical resection of choroidal melanoma. In: Schachat AP, editor. Ryan’s retina, vol. 3. Philadelphia: Elsevier; 2018. p. 2591–600.Google Scholar
  31. 31.
    Rospond-Kubiak I, Damato B. The surgical approach to the management of anterior uveal melanomas. Eye. 2014;28:741–7.PubMedPubMedCentralGoogle Scholar
  32. 32.
    Konstantinidis L, Groenewald C, Coupland SE, Damato B. Long-term outcome of primary endoresection of choroidal melanoma. Br J Ophthalmol. 2014;98:82–5.PubMedGoogle Scholar
  33. 33.
    Bechrakis NE, Foerster MH. Neoadjuvant proton beam radiotherapy combined with subsequent endoresection of choroidal melanomas. Int Ophthalmol Clin. 2006;46:95–107.PubMedGoogle Scholar
  34. 34.
    Rice JC, Liebenberg L, Scholtz RP, Torr G. Fatal air embolism during endoresection of choroidal melanoma. Retin Cases Brief Rep. 2014;8:127–9.PubMedGoogle Scholar
  35. 35.
    Damato B, Kacperek A, Chopra M, Sheen MA, Campbell IR, Errington RD. Proton beam radiotherapy of iris melanoma. Int J Radiat Oncol Biol Phys. 2005;63:109–15.PubMedGoogle Scholar
  36. 36.
    Konstantinidis L, Roberts D, Errington RD, Kacperek A, Damato B. Whole anterior segment proton beam radiotherapy for diffuse iris melanoma. Br J Ophthalmol. 2013;97(4):471–4.PubMedGoogle Scholar
  37. 37.
    Konstantinidis L, Roberts D, Errington RD, Kacperek A, Heimann H, Damato B. Transpalpebral proton beam radiotherapy of choroidal melanoma. Br J Ophthalmol. 2015;99:232–5.PubMedGoogle Scholar
  38. 38.
    Wackernagel W, Holl E, Tarmann L, Mayer C, Avian A, Schneider M, Kapp KS, Langmann G. Local tumour control and eye preservation after gamma-knife radiosurgery of choroidal melanomas. Br J Ophthalmol. 2014;98:218–23.PubMedGoogle Scholar
  39. 39.
    Damato B. Vasculopathy after treatment of choroidal melanoma. In: Joussen A, Gardner TW, Kirchhof B, Ryan SJ, editors. Retinal vascular disease. Berlin: Springer; 2007. p. 582–91.Google Scholar
  40. 40.
    Konstantinidis L, Groenewald C, Coupland SE, Damato B. Trans-scleral local resection of toxic choroidal melanoma after proton beam radiotherapy. Br J Ophthalmol. 2014;98:775–9.PubMedGoogle Scholar
  41. 41.
    Seibel I, Hager A, Riechardt AI, Davids AM, Boker A, Joussen AM. Antiangiogenic or corticosteroid treatment in patients with radiation maculopathy after proton beam therapy for uveal melanoma. Am J Ophthalmol. 2016;168:31–9.PubMedGoogle Scholar
  42. 42.
    Matet A, Daruich A, Zografos L. Radiation maculopathy after proton beam therapy for uveal melanoma: optical coherence tomography angiography alterations influencing visual acuity. Invest Ophthalmol Vis Sci. 2017;58:3851–61.PubMedGoogle Scholar
  43. 43.
    Mantel I, Schalenbourg A, Bergin C, Petrovic A, Weber DC, Zografos L. Prophylactic use of bevacizumab to avoid anterior segment neovascularization following proton therapy for uveal melanoma. Am J Ophthalmol. 2014;158:693–701 e692.PubMedGoogle Scholar
  44. 44.
    Dalvin LA, Shields CL, Ancona-Lezama DA, Yu MD, Di Nicola M, Williams BK, Jr., Lucio-Alvarez JA, Ang SM, Maloney SM, Welch RJ, Shields JA. Combination of multimodal imaging features predictive of choroidal nevus transformation into melanoma. Br J Ophthalmol. 2018. pii: bjophthalmol-2018-312967. [Epub ahead of print]PubMedGoogle Scholar
  45. 45.
    Damato B, Hope-Stone L, Cooper B, Brown S, Salmon P, Heimann H, Dunn L. Patient-reported outcomes and quality of life after treatment of choroidal melanoma: a comparison of enucleation vs radiotherapy in 1596 patients. Am J Ophthalmol. 2018;193:230–51.PubMedGoogle Scholar
  46. 46.
    Kines RC, Varsavsky I, Choudhary S, Bhattacharya D, Spring S, McLaughlin R, Kang SJ, Grossniklaus HE, Vavvas D, Monks S, MacDougall JR, de Los Pinos E, Schiller JT. An infrared dye-conjugated virus-like particle for the treatment of primary uveal melanoma. Mol Cancer Ther. 2018;17:565–74.PubMedGoogle Scholar
  47. 47.
    Bidard FC, Madic J, Mariani P, Piperno-Neumann S, Rampanou A, Servois V, Cassoux N, Desjardins L, Milder M, Vaucher I, Pierga JY, Lebofsky R, Stern MH, Lantz O. Detection rate and prognostic value of circulating tumor cells and circulating tumor DNA in metastatic uveal melanoma. Int J Cancer. 2014;134:1207–13.PubMedGoogle Scholar
  48. 48.
    Tura A, Lueke J, Grisanti S. Liquid biopsy for uveal melanoma. In: Scott JF, Gerstenblith MR, editors. Noncutaneous melanoma. Brisbane: Codon Publications; 2018.Google Scholar
  49. 49.
    Abdel-Rahman MH, Pilarski R, Cebulla CM, Massengill JB, Christopher BN, Boru G, Hovland P, Davidorf FH. Germline BAP1 mutation predisposes to uveal melanoma, lung adenocarcinoma, meningioma, and other cancers. J Med Genet. 2011;48:856–9.PubMedGoogle Scholar
  50. 50.
    Masoomian B, Shields JA, Shields CL. Overview of BAP1 cancer predisposition syndrome and the relationship to uveal melanoma. J Curr Ophthalmol. 2018;30:102–9.PubMedPubMedCentralGoogle Scholar
  51. 51.
    Munier FL. Classification and management of seeds in retinoblastoma. Ellsworth Lecture Ghent August 24th 2013. Ophthalmic Genet. 2014;35:193–207.PubMedPubMedCentralGoogle Scholar
  52. 52.
    Francis JH, Levin AM, Zabor EC, Gobin YP, Abramson DH. Ten-year experience with ophthalmic artery chemosurgery: ocular and recurrence-free survival. PLoS One. 2018;13:e0197081.PubMedPubMedCentralGoogle Scholar
  53. 53.
    Dimaras H, Corson TW, Cobrinik D, White A, Zhao J, Munier FL, Abramson DH, Shields CL, Chantada GL, Njuguna F, Gallie BL. Retinoblastoma. Nat Rev Dis Primers. 2015;1:15021.PubMedPubMedCentralGoogle Scholar
  54. 54.
    Damato B, Afshar AR, Everett L, Banerjee A, Hetts SW. The University of California, San Francisco documentation system for retinoblastoma: preparing to improve staging methods for this disease. Ocul Oncol Pathol. 2019;5:36–45.PubMedGoogle Scholar
  55. 55.
    Soliman SE, Racher H, Zhang C, MacDonald H, Gallie BL. Genetics and molecular diagnostics in retinoblastoma—an update. Asia Pac J Ophthalmol (Phila). 2017;6:197–207.Google Scholar
  56. 56.
    Ewens KG, Bhatti TR, Moran KA, Richards-Yutz J, Shields CL, Eagle RC, Ganguly A. Phosphorylation of pRb: mechanism for RB pathway inactivation in MYCN-amplified retinoblastoma. Cancer Med. 2017;6:619–30.PubMedPubMedCentralGoogle Scholar
  57. 57.
    Sethi RV, Shih HA, Yeap BY, Mouw KW, Petersen R, Kim DY, Munzenrider JE, Grabowski E, Rodriguez-Galindo C, Yock TI, Tarbell NJ, Marcus KJ, Mukai S, MacDonald SM. Second nonocular tumors among survivors of retinoblastoma treated with contemporary photon and proton radiotherapy. Cancer. 2014;120:126–33.PubMedGoogle Scholar
  58. 58.
    Shields CL, Welch RJ, Malik K, Acaba-Berrocal LA, Selzer EB, Newman JH, Mayro EL, Constantinescu AB, Spencer MA, McGarrey MP, Knapp AN, Graf AE, Altman AJ, Considine SP, Shields JA. Uveal metastasis: clinical features and survival outcome of 2214 tumors in 1111 patients based on primary tumor origin. Middle East Afr J Ophthalmol. 2018;25:81–90.PubMedPubMedCentralGoogle Scholar
  59. 59.
    Al-Dahmash SA, Shields CL, Kaliki S, Johnson T, Shields JA. Enhanced depth imaging optical coherence tomography of choroidal metastasis in 14 eyes. Retina. 2014;34:1588–93.PubMedGoogle Scholar
  60. 60.
    Konstantinidis L, Rospond-Kubiak I, Zeolite I, Heimann H, Groenewald C, Coupland SE, Damato B. Management of patients with uveal metastases at the Liverpool Ocular Oncology Centre. Br J Ophthalmol. 2014;98:92–8.PubMedGoogle Scholar
  61. 61.
    Konstantinidis L, Damato B. Intraocular metastases—a review. Asia Pac J Ophthalmol (Phila). 2017;6:208–14.Google Scholar
  62. 62.
    Stjepanovic N, Velazquez-Martin JP, Bedard PL. Ocular toxicities of MEK inhibitors and other targeted therapies. Ann Oncol. 2016;27:998–1005.PubMedGoogle Scholar
  63. 63.
    Abdel-Rahman O, Oweira H, Petrausch U, Helbling D, Schmidt J, Mannhart M, Mehrabi A, Schob O, Giryes A. Immune-related ocular toxicities in solid tumor patients treated with immune checkpoint inhibitors: a systematic review. Expert Rev Anticancer Ther. 2017;17:387–94.PubMedGoogle Scholar
  64. 64.
    Dalvin LA, Shields CL, Orloff M, Sato T, Shields JA. Checkpoint inhibitor immune therapy: systemic indications and ophthalmic side effects. Retina. 2018;38:1063–78.PubMedGoogle Scholar
  65. 65.
    Coupland SE, Damato B. Understanding intraocular lymphomas. Clin Exp Ophthalmol. 2008;36:564–78.PubMedGoogle Scholar
  66. 66.
    Barry RJ, Tasiopoulou A, Murray PI, Patel PJ, Sagoo MS, Denniston AK, Keane PA. Characteristic optical coherence tomography findings in patients with primary vitreoretinal lymphoma: a novel aid to early diagnosis. Br J Ophthalmol. 2018;102:1362–6.PubMedGoogle Scholar
  67. 67.
    Mapelli C, Invernizzi A, Barteselli G, Pellegrini M, Tabacchi E, Staurenghi G, Viola F. Multimodal imaging of vitreoretinal lymphoma. Ophthalmologica. 2016;236:166–74.PubMedGoogle Scholar
  68. 68.
    Dawson AC, Williams KA, Appukuttan B, Smith JR. Emerging diagnostic tests for vitreoretinal lymphoma: a review. Clin Exp Ophthalmol. 2018;46:945–54.PubMedGoogle Scholar
  69. 69.
    Araujo I, Coupland SE. Primary vitreoretinal lymphoma—a review. Asia Pac J Ophthalmol (Phila). 2017;6:283–9.Google Scholar
  70. 70.
    Gonzales J, Doan T, Shantha JG, Bloomer M, Wilson MR, DeRisi JL, Acharya N. Metagenomic deep sequencing of aqueous fluid detects intraocular lymphomas. Br J Ophthalmol. 2018;102:6–8.PubMedGoogle Scholar
  71. 71.
    Pulido JS, Johnston PB, Nowakowski GS, Castellino A, Raja H. The diagnosis and treatment of primary vitreoretinal lymphoma: a review. Int J Retina Vitreous. 2018;4:18.PubMedPubMedCentralGoogle Scholar
  72. 72.
    Shields CL, Sioufi K, Mashayekhi A, Shields JA. Intravitreal melphalan for treatment of primary vitreoretinal lymphoma: a new indication for an old drug. JAMA Ophthalmol. 2017;135:815–8.PubMedGoogle Scholar
  73. 73.
    Riemens A, Bromberg J, Touitou V, Sobolewska B, Missotten T, Baarsma S, Hoyng C, Cordero-Coma M, Tomkins-Netzer O, Rozalski A, Tugal-Tutkun I, Guex-Crosier Y, Los LI, Bollemeijer JG, Nolan A, Pawade J, Willermain F, Bodaghi B, ten Dam-van Loon N, Dick A, Zierhut M, Lightman S, Mackensen F, Moulin A, Erckens R, Wensing B, le Hoang P, Lokhorst H, Rothova A. Treatment strategies in primary vitreoretinal lymphoma: a 17-center European collaborative study. JAMA Ophthalmol. 2015;133:191–7.PubMedGoogle Scholar
  74. 74.
    Rubenstein JL, Treseler PA, Stewart PJ. Regression of refractory intraocular large B-cell lymphoma with lenalidomide monotherapy. J Clin Oncol Off J Am Soc Clin Oncol. 2011;29:e595–7.Google Scholar
  75. 75.
    Hagner PR, Man HW, Fontanillo C, Wang M, Couto S, Breider M, Bjorklund C, Havens CG, Lu G, Rychak E, Raymon H, Narla RK, Barnes L, Khambatta G, Chiu H, Kosek J, Kang J, Amantangelo MD, Waldman M, Lopez-Girona A, Cai T, Pourdehnad M, Trotter M, Daniel TO, Schafer PH, Klippel A, Thakurta A, Chopra R, Gandhi AK. CC-122, a pleiotropic pathway modifier, mimics an interferon response and has antitumor activity in DLBCL. Blood. 2015;126:779–89.PubMedPubMedCentralGoogle Scholar
  76. 76.
    Bever GJ, Kim DJ, Afshar AR, Rubenstein JL, Damato BE. Therapeutic vitrectomy as an adjunct treatment to systemic chemotherapy for intraocular lymphoma. Retin Cases Brief Rep. 2017. [Epub ahead of print]
  77. 77.
    Larsen AC. Conjunctival malignant melanoma in Denmark: epidemiology, treatment and prognosis with special emphasis on tumorigenesis and genetic profile. Acta Ophthalmol. 2016;94 Thesis 1:1–27.PubMedGoogle Scholar
  78. 78.
    Damato B, Coupland SE. Conjunctival melanoma and melanosis: a reappraisal of terminology, classification and staging. Clin Exp Ophthalmol. 2008;36:786–95.PubMedGoogle Scholar
  79. 79.
    Pfeiffer ML, Ozgur OK, Myers JN, Peng A, Ning J, Zafereo ME, Thakar S, Thuro B, Prieto VG, Ross MI, Esmaeli B. Sentinel lymph node biopsy for ocular adnexal melanoma. Acta Ophthalmol. 2017;95:e323–8.PubMedGoogle Scholar
  80. 80.
    Shields CL, Chien JL, Surakiatchanukul T, Sioufi K, Lally SE, Shields JA. Conjunctival tumors: review of clinical features, risks, biomarkers, and outcomes—the 2017 J. Donald M. Gass lecture. Asia Pac J Ophthalmol (Phila). 2017;6:109–20.Google Scholar
  81. 81.
    Damato B, Coupland SE. An audit of conjunctival melanoma treatment in Liverpool. Eye. 2009;23:801–9.PubMedGoogle Scholar
  82. 82.
    Wong JR, Nanji AA, Galor A, Karp CL. Management of conjunctival malignant melanoma: a review and update. Expert Rev Ophthalmol. 2014;9:185–204.PubMedPubMedCentralGoogle Scholar
  83. 83.
    Kim SE, Salvi SM. Immunoreduction of ocular surface tumours with intralesional interferon alpha-2a. Eye. 2018;32:460–2.PubMedGoogle Scholar
  84. 84.
    Lake SL, Jmor F, Dopierala J, Taktak AF, Coupland SE, Damato BE. Multiplex ligation-dependent probe amplification of conjunctival melanoma reveals common BRAF V600E gene mutation and gene copy number changes. Invest Ophthalmol Vis Sci. 2011;52:5598–604.PubMedGoogle Scholar
  85. 85.
    Larsen AC. Conjunctival malignant melanoma in Denmark. Epidemiology, treatment and prognosis with special emphasis on tumorigenesis and genetic profile. Acta Ophthalmol. 2016;94:842.PubMedGoogle Scholar
  86. 86.
    Sagiv O, Thakar SD, Kandl TJ, Ford J, Sniegowski MC, Hwu WJ, Esmaeli B. Immunotherapy with programmed cell death 1 inhibitors for 5 patients with conjunctival melanoma. JAMA Ophthalmol. 2018;136:1236–41.PubMedPubMedCentralGoogle Scholar
  87. 87.
    Kini A, Fu R, Compton C, Miller DM, Ramasubramanian A. Pembrolizumab for recurrent conjunctival melanoma. JAMA Ophthalmol. 2017;135:891–2.PubMedGoogle Scholar
  88. 88.
    Cicinelli MV, Marchese A, Bandello F, Modorati G. Clinical management of ocular surface squamous neoplasia: a review of the current evidence. Ophthalmol Ther. 2018;7:247–62.PubMedPubMedCentralGoogle Scholar
  89. 89.
    Nanji AA, Mercado C, Galor A, Dubovy S, Karp CL. Updates in ocular surface tumor diagnostics. Int Ophthalmol Clin. 2017;57:47–62.PubMedPubMedCentralGoogle Scholar
  90. 90.
    Siedlecki AN, Tapp S, Tosteson AN, Larson RJ, Karp CL, Lietman T, Zegans ME. Surgery versus interferon alpha-2b treatment strategies for ocular surface squamous neoplasia: a literature-based decision analysis. Cornea. 2016;35:613–8.PubMedPubMedCentralGoogle Scholar
  91. 91.
    Kenawy N, Garrick A, Heimann H, Coupland SE, Damato BE. Conjunctival squamous cell neoplasia: the Liverpool Ocular Oncology Centre experience. Graefes Arch Clin Exp Ophthalmol. 2015;253:143–50.PubMedGoogle Scholar

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Nuffield Department of Clinical NeurosciencesUniversity of Oxford, John Radcliffe HospitalOxfordUK

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