Malignant and Benign Diseases of the Eye and Orbit

  • Tania Kaprealian
  • Kavita K. Mishra
  • Alice Wang-Chesebro
  • Jeanne Marie Quivey


All eye/orbit malignancies are uncommon: per ACS, approximately 2,390 new cases/year. Percentage of malignant tumors increases with age, due to increases in primary orbital lymphoma (OL) and metastatic lesions in the elderly (both in choroid and in the orbit). Most common intraocular malignancy in adults: choroidal metastasis, usually adenocarcinoma, especially from lung, breast, and prostate. Most common primary eye malignancy in adults: uveal melanoma. Most common primary eye malignancy in children: retinoblastoma. Most common primary orbital malignancy in adults: lymphoma. Most common primary orbital malignancy in children: rhabdomyosarcoma. This chapter will discuss uveal melanoma, OL, intraocular lymphoma (IOL), thyroid ophthalmopathy, and orbital lymphoid hyperplasia (for Retinoblastoma and Rhabdomyosarcoma, see Part XI).


Ciliary Body Uveal Melanoma Central Retinal Vein Occlusion Neovascular Glaucoma Intraocular Lymphoma 



  1. Berenbom A, Davila RM, Lin H-S, et al. Treatment outcomes for primary intraocular lymphoma: implications for external beam radiotherapy. Eye 2007;21:1198-1201.PubMedCrossRefGoogle Scholar
  2. Bhatia S, et al. Curative radiotherapy for primary orbital lymphoma. IJROBP 2002;54:818-821.Google Scholar
  3. Bolek T, et al. Radiotherapy in the management of orbital lymphoma. IJROBP 1999;44:31-36.Google Scholar
  4. Bradley EA, Gower EW, Bradley DJ, et al. Orbital radiation for graves ophthalmopathy. Ophthalmology 2008;115:398-409.PubMedCrossRefGoogle Scholar
  5. Char D, Quivey J, et al. Helium ions versus I-125 brachytherapy in management of uveal melanoma. A prospective, dynamically balanced trial. Ophthalmology 1993;100:1547-1554.Google Scholar
  6. COMS. Mortality in patients with small choroidal melanoma. COMS report no. 4. Arch Ophthalmol 1997;115:886.Google Scholar
  7. COMS. 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.Google Scholar
  8. COMS. The COMS randomized trial of pre-enucleation radiation of large choroidal melanoma: IV. Ten-year mortality findings and prognostic factors. COMS report no 24. Am J Ophthalmol 2004;138(6):936.Google Scholar
  9. Diener-West M, et al. Development of metastatic disease after enrollment in the COMS trials for treatment of choroidal melanoma: COMS report no. 26. Arch Ophthalmol 2005;123(12):1639.Google Scholar
  10. Egger E, et al. Maximizing local tumor control and survival after proton beam radiotherapy of uveal melanoma. Int J Radiat Oncol Biol Phys 2001;51:138.PubMedCrossRefGoogle Scholar
  11. Esik O, et al. Retrospective analysis of different modalities for treatment of primary orbital non-Hodgkin’s lymphomas. Radiother Oncol 1996;38:13-18.PubMedCrossRefGoogle Scholar
  12. Gragoudas ES, et al. A randomized controlled trial of varying radiation doses in the treatment of choroidal melanoma. Arch Ophthalmol 2000;118:773.PubMedGoogle Scholar
  13. Gragoudas ES. Proton beam irradiation of uveal melanomas: the first 30 years. Invest Ophthalmol Vis Sci 2006;47:4666-4673.PubMedCrossRefGoogle Scholar
  14. Hoffman PM, et al. Intraocular lymphoma: a series of 14 patients with clinicopathological features and treatment outcomes. Eye 2003;17:513-521.PubMedCrossRefGoogle Scholar
  15. Lanciano R, et al. The results of radiotherapy for orbital pseudotumor. IJROBP 1990;18:407Google Scholar
  16. Le Q, et al. Primary radiotherapy for localized orbital malt lymphoma. IJROBP 2002;52:657-663.Google Scholar
  17. Marucci L, Lane AM, Li W, et al. Conservation treatment of the eye: conformal proton reirradiation for recurrent uveal melanoma. IJROBP 2006;64:1018-1022.Google Scholar
  18. Nag S, et al. The American Brachytherapy Society recommendations for brachytherapy of uveal melanomas. IJROBP 2003;56:544.Google Scholar
  19. Pfeffer MR, Rabin T, Tsvang L, et al. Orbital lymphoma: is it necessary to treat the entire orbit? IJROBP 2004;60:527-530.Google Scholar
  20. Prummel M, et al. Randomized double-blind trial of prednisone versus radiotherapy in Graves’ ophthalmopathy. Lancet 1993;342:949-954.PubMedCrossRefGoogle Scholar
  21. Prummel MF, Terwee CB, Gerding MN, et al. A randomized controlled trial of orbital radiotherapy versus sham irradiation in patients with mild Graves’ ophthalmopathy. J Clin Endocrinol Metab 2004;89:15-20.PubMedCrossRefGoogle Scholar
  22. Quivey J, Char D, et al. High intensity 125-iodine (125I) plaque treatment of uveal melanoma. IJROBP 1993;26(4):613-618.Google Scholar
  23. Shields J, et al. Survey of 1264 patients with orbital tumors and simulating lesions. Ophthalmology 2004;111:997-1008.PubMedCrossRefGoogle Scholar
  24. Weber DC, Bogner J, Verwey J, et al. Proton beam radiotherapy versus fractionated stereotactic radiotherapy for uveal melanomas: a comparative study. IJROBP 2005;63:373-384.Google Scholar
  25. Zytkovicz A, et al: Peripheral dose in ocular treatments with Cyberknife and Gamma Knife radiosurgery compared to proton radiotherapy. Phys Med Biol 2007;52(19):5957.PubMedCrossRefGoogle Scholar

Further Reading

  1. Akpek E, et al. Intraocular-central nervous system lymphoma: clinical features, diagnosis, and outcomes. Ophthalmology 1999;106:1805-1810.Google Scholar
  2. Bell D, et al. Choroidal melanoma: natural history and management options. Cancer Control 2004;11(5):296-303.Google Scholar
  3. Chan C, Wallace D. Intraocular lymphoma: update on diagnosis and management. Cancer Control 2004;11(5):285-295.Google Scholar
  4. Char D, et al. Primary intraocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management. Ophthalmology 1988;95:625-630.Google Scholar
  5. COMS. The COMS randomized trial of pre-enucleation radiation of large choroidal melanoma II: initial mortality findings: COMS report no. 10. Am J Ophthamol 1998;124:779-796.Google Scholar
  6. COMS. The COMS randomized trial of iodine 125 brachytherapy for choroidal melanoma III: initial mortality findings: COMS report no. 18. Arch Ophthalmol 2001;119:969-982.Google Scholar
  7. Daftari I, et al. Newer radiation modalities for choroidal tumors. Int Ophthal Clin 2006;46:69.Google Scholar
  8. Diener-West M, et al. Screening for metastasis from choroidal melanoma: the COMS Group report 23. JCO 2004;22:2438-2444.Google Scholar
  9. Kath R, et al. Prognosis and treatment of disseminated uveal melanoma. Cancer 1993;72(7):2219-2223.Google Scholar
  10. Kujala E, et al. Very long term prognosis of patients with malignant uveal melanoma. Invest Ophthalmol Vis Sci 2003;44:4651-4659.Google Scholar
  11. Mishra KK, Quivey J, Char D. Uveal melanoma. In: Leibel S, Phillips T, editors. Textbook of Radiation Oncology, 3rd ed. Chapter 64 In press.Google Scholar
  12. Nguyen LN, Ang K. The orbit. In: Cox J, Ang K, editors. Radiation Oncology. 8th ed. St. Louis: Mosby; 2003. pp 282-292.Google Scholar
  13. Pelloski CE, et al. Clinical stage IEA-IIEA orbital lymphomas: outcomes in the era of modern staging and treatment. Radiother Oncol 2001;59:145-151.Google Scholar
  14. Peterson IA, et al. Prognostic factors in the radiotherapy of Graves’ ophthalmopathy. IJROBP 1990;19:259-264.Google Scholar
  15. Peterson K, et al. The clinical spectrum of ocular lymphoma. Cancer 1993;72:843-849.Google Scholar
  16. Rosenthal S. Benign disease. In: Leibel SA, Phillips TL, editors. Textbook of Radiation Oncology. 2nd ed. Philadelphia: Saunders; 2004. pp 1525-1543.Google Scholar
  17. Shields C, Shields J. Diagnosis and management of retinoblastoma. Cancer Control 2004;11(5):317-327.Google Scholar
  18. Stafford SL, et al. Orbital lymphoma: radiotherapy outcome and complications. Radiother Oncol 2001;59:139-144.Google Scholar

Copyright information

© Springer-Verlag New York 2010

Authors and Affiliations

  • Tania Kaprealian
    • 1
  • Kavita K. Mishra
    • 1
  • Alice Wang-Chesebro
    • 2
  • Jeanne Marie Quivey
    • 1
  1. 1.Radiation OncologyUniversity of California San FranciscoSan FranciscoUSA
  2. 2.Providence Portland Radiation OncologyThe Oregon Clinic, P.C.PortlandUSA

Personalised recommendations