What is the prognosis of ocular lymphoma?

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Death can occur as a result of the systemic spread of lymphoma.^[4]Prior or concurrent systemic disease has been noted as the most significant predictive factor for lymphoma-related death, but tumor-related death was also found to be more common and earlier with bilateral disease. Tumor-related death is slightly less where symptoms have been present for more than a year and slightly greater in the elderly.

Patients with primary central nervous system lymphoma with ocular involvement (PCNSLO) have a poor prognosis even with chemoradiation, and many succumb to central nervous system (CNS) disease within 2 years. Yet, median survival of primary central nervous system lymphoma (PCNSL) has increased from 1-1.5 years to over 3 years with newer therapies. Features affecting the prognosis of PCNSLO are not well understood.^[57]Death ensues by CNS dissemination. Ocular lymphoma may be the initial manifestation of PCNSL.

In a study of patients with ocular adnexal lymphomas, age, sex, and anatomic location of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years.^[43]The major prognostic criteria for ocular adnexal lymphomas include anatomic location of the tumor, stage of disease at first presentation, subtype of lymphoma, immunohistochemical markers determining factors such as tumor growth rate, and the serum lactate dehydrogenase (LDH) level.^[41]

The extent of disease at the time of presentation was the most important clinical prognostic factor. Advanced disease correlated with increased risk ratios of having persistent disease at the final follow-up and lymphoma-related death.^[43]

The longest survival has been seen in patients with low-grade lymphomas (ie, marginal zone lymphoma, follicular lymphoma).^[58]However, T-cell lymphomas are associated with high mortality with conventional treatment, as there is a high incidence of systemic involvement.^[59]

The overall prognosis for ocular adnexal lymphoid tumors is excellent; when lumped together, 67% are not found to be associated with systemic disease with a mean follow-up of over 4 years. Over the course of follow-up, it could be anticipated that 20-25% of patients not known to have systemic lymphoma develop evidence of disseminated disease within 5 years.^[60]

Conjunctival lymphoma is known to have the lowest rate of extraorbital spread and lymphoma-related death, the rate of these 2 events being sequentially greater for patients with predominantly deep orbital lymphoma, lacrimal gland lymphoma, or eyelid lymphoma.^{[61, 60]}

With radiotherapy for orbital disease, the 5-year disease-free survival and overall survival rate has been between 65-73.6% and 65.5-78%.^{[47, 53]}Most relapses occur in the first 2 years after therapy. The risk of late relapse is higher in patients with a divergent histology of both indolent disease and aggressive disease.

Intraocular lymphoma can lead to blindness due to damage of the intraocular structures involved. Orbital lymphoma can cause blindness in untreated or aggressive cases with severe proptosis leading to corneal complications or optic neuropathy. Local irradiation can also cause reversible blindness due to cataract and irreversible blindness in cases of radiation-induced optic neuropathy and retinopathy.

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Valvassori GE, Sabnis SS, Mafee RF, Brown MS, Putterman A. Imaging of orbital lymphoproliferative disorders. Radiol Clin North Am. 1999 Jan. 37(1):135-50, x-xi. [Medline].
Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology. 2004 May. 111(5):997-1008. [Medline].
Bonavolontà G, Strianese D, Grassi P, Comune C, Tranfa F, Uccello G, et al. An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthal Plast Reconstr Surg. 2013 Mar-Apr. 29(2):79-86. [Medline].
Ahmed S, Shahid RK, Sison CP, Fuchs A, Mehrotra B. Orbital lymphomas: a clinicopathologic study of a rare disease. Am J Med Sci. 2006 Feb. 331(2):79-83. [Medline].
Özkan MC, Palamar M, Tombuloğlu M, Hekimgil M, Özsan N, Saydam G, et al. Ocular Adnexal Lymphomas: Single-Center Experience. Clin Lymphoma Myeloma Leuk. 2015 Jun. 15 Suppl:S158-60. [Medline].
Russell-Jones R. World Health Organization classification of hematopoietic and lymphoid tissues: implications for dermatology. J Am Acad Dermatol. 2003 Jan. 48(1):93-102. [Medline].
Rosado MF, Byrne GE Jr, Ding F, Fields KA, Ruiz P, Dubovy SR, et al. Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci. Blood. 2006 Jan 15. 107(2):467-72. [Medline].
Chanudet E, Zhou Y, Bacon CM, Wotherspoon AC, Muller-Hermelink HK, Adam P, et al. Chlamydia psittaci is variably associated with ocular adnexal MALT lymphoma in different geographical regions. J Pathol. 2006 Jul. 209(3):344-51. [Medline].
Moslehi R, Devesa SS, Schairer C, Fraumeni JF Jr. Rapidly increasing incidence of ocular non-hodgkin lymphoma. J Natl Cancer Inst. 2006 Jul 5. 98(13):936-9. [Medline].
National Cancer Institute. Surveillance, Epidemiology, and End Results Cancer Fact Stat Sheet, Cancer of the Eye and Orbit. Available at http://seer.cancer.gov/statfacts/html/eye.html. Accessed: 2008.
Jardel P, Caujolle JP, Gastaud L, Maschi C, Sauerwein W, Thariat J. [Malignant tumours of the eye: Epidemiology, diagnostic methods and radiotherapy]. Cancer Radiother. 2015 Oct 21. [Medline].
Ohtsuka K, Hashimoto M, Suzuki Y. High incidence of orbital malignant lymphoma in Japanese patients. Am J Ophthalmol. 2004 Nov. 138(5):881-2. [Medline].
Shikishima K, Kawai K, Kitahara K. Pathological evaluation of orbital tumours in Japan: analysis of a large case series and 1379 cases reported in the Japanese literature. Clin Experiment Ophthalmol. 2006 Apr. 34(3):239-44. [Medline].
Ko YH, Kim CW, Park CS, Jang HK, Lee SS, Kim SH, et al. REAL classification of malignant lymphomas in the Republic of Korea: incidence of recently recognized entities and changes in clinicopathologic features. Hematolymphoreticular Study Group of the Korean Society of Pathologists. Revised European-American lymphoma. Cancer. 1998 Aug 15. 83(4):806-12. [Medline].
Seregard S, Sahlin S. Panorama of orbital space-occupying lesions. The 24-year experience of a referral centre. Acta Ophthalmol Scand. 1999 Feb. 77(1):91-8. [Medline].
Burnier MN Jr, Stockl FA, Dolmetsch AM. Large B-cell lymphoma of the retina and CNS. Presented at the 1994 Annual Meeting of the Eastern Ophthalmic Pathology Society, Boston, Mass. October 1994.
Levy-Clarke GA, Chan CC, Nussenblatt RB. Diagnosis and management of primary intraocular lymphoma. Hematol Oncol Clin North Am. 2005 Aug. 19(4):739-49, viii. [Medline].
Schabet M. Epidemiology of primary CNS lymphoma. J Neurooncol. 1999 Jul. 43(3):199-201. [Medline].
Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older adult population. Ophthalmology. 2002 Feb. 109(2):243-8. [Medline].
Char DH, Ljung BM, Miller T, Phillips T. Primary intraocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management. Ophthalmology. 1988 May. 95(5):625-30. [Medline].
Char DH, Margolis L, Newman AB. Ocular reticulum cell sarcoma. Am J Ophthalmol. 1981 Apr. 91(4):480-3. [Medline].
Whitcup SM, de Smet MD, Rubin BI, Palestine AG, Martin DF, Burnier M Jr, et al. Intraocular lymphoma. Clinical and histopathologic diagnosis. Ophthalmology. 1993 Sep. 100(9):1399-406. [Medline].
Peterson K, Gordon KB, Heinemann MH, DeAngelis LM. The clinical spectrum of ocular lymphoma. Cancer. 1993 Aug 1. 72(3):843-9. [Medline].
Freeman LN, Schachat AP, Knox DL, Michels RG, Green WR. Clinical features, laboratory investigations, and survival in ocular reticulum cell sarcoma. Ophthalmology. 1987 Dec. 94(12):1631-9. [Medline].
Chan CC, Buggage RR, Nussenblatt RB. Intraocular lymphoma. Curr Opin Ophthalmol. 2002 Dec. 13(6):411-8. [Medline].
Mansour AM. Adnexal findings in AIDS. Ophthal Plast Reconstr Surg. 1993 Dec. 9(4):273-9. [Medline].
Reifler DM, Warzynski MJ, Blount WR, Graham DM, Mills KA. Orbital lymphoma associated with acquired immune deficiency syndrome (AIDS). Surv Ophthalmol. 1994 Jan-Feb. 38(4):371-80. [Medline].
Neudorfer M, Kessler A, Anteby I, Goldenberg D, Barak A. Co-existence of intraocular and orbital lymphoma. Acta Ophthalmol Scand. 2004 Dec. 82(6):754-61. [Medline].
Akpek EK, Polcharoen W, Ferry JA, Foster CS. Conjunctival lymphoma masquerading as chronic conjunctivitis. Ophthalmology. 1999 Apr. 106(4):757-60. [Medline].
Küker W, Herrlinger U, Gronewäller E, Rohrbach JM, Weller M. Ocular manifestation of primary nervous system lymphoma: what can be expected from imaging?. J Neurol. 2002 Dec. 249(12):1713-6. [Medline].
Flanders AE, Espinosa GA, Markiewicz DA, Howell DD. Orbital lymphoma. Role of CT and MRI. Radiol Clin North Am. 1987 May. 25(3):601-13. [Medline].
Sjo LD, Ralfkiaer E, Juhl BR, Prause JU, Kivela T, Auw-Haedrich C, et al. Primary lymphoma of the lacrimal sac: an EORTC ophthalmic oncology task force study. Br J Ophthalmol. 2006 Aug. 90(8):1004-9. [Medline].
Hornblass A, Jakobiec FA, Reifler DM, Mines J. Orbital lymphoid tumors located predominantly within extraocular muscles. Ophthalmology. 1987 Jun. 94(6):688-97. [Medline].
Yeo JH, Jakobiec FA, Abbott GF, Trokel SL. Combined clinical and computed tomographic diagnosis of orbital lymphoid tumors. Am J Ophthalmol. 1982 Aug. 94(2):235-45. [Medline].
Sullivan TJ, Valenzuela AA. Imaging features of ocular adnexal lymphoproliferative disease. Eye. 2006 Oct. 20(10):1189-95. [Medline].
Akansel G, Hendrix L, Erickson BA, Demirci A, Papke A, Arslan A, et al. MRI patterns in orbital malignant lymphoma and atypical lymphocytic infiltrates. Eur J Radiol. 2005 Feb. 53(2):175-81. [Medline].
Valenzuela AA, Allen C, Grimes D, Wong D, Sullivan TJ. Positron emission tomography in the detection and staging of ocular adnexal lymphoproliferative disease. Ophthalmology. 2006 Dec. 113(12):2331-7. [Medline].
Roe RH, Finger PT, Kurli M, Tena LB, Iacob CE. Whole-body positron emission tomography/computed tomography imaging and staging of orbital lymphoma. Ophthalmology. 2006 Oct. 113(10):1854-8. [Medline].
English JF, Sullivan TJ. The Role of FDG-PET in the Diagnosis and Staging of Ocular Adnexal Lymphoproliferative Disease. Orbit. 2015 Oct. 34 (5):284-91. [Medline].
Buggage RR, Chan CC, Nussenblatt RB. Ocular manifestations of central nervous system lymphoma. Curr Opin Oncol. 2001 May. 13(3):137-42. [Medline].
Coupland SE, Hummel M, Stein H. Ocular adnexal lymphomas: five case presentations and a review of the literature. Surv Ophthalmol. 2002 Sep-Oct. 47(5):470-90. [Medline].
Norton AJ. Monoclonal antibodies in the diagnosis of lymphoproliferative diseases of the orbit and orbital adnexae. Eye. 2006 Oct. 20(10):1186-8. [Medline].
Coupland SE, Krause L, Delecluse HJ, Anagnostopoulos I, Foss HD, Hummel M, et al. Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases. Ophthalmology. 1998 Aug. 105(8):1430-41. [Medline].
Valluri S, Moorthy RS, Khan A, Rao NA. Combination treatment of intraocular lymphoma. Retina. 1995. 15(2):125-9. [Medline].
Plowman PN, Montefiore DS, Lightman S. Multiagent chemotherapy in the salvage cure of ocular lymphoma relapsing after radiotherapy. Clin Oncol (R Coll Radiol). 1993. 5(5):315-6. [Medline].
Fishburne BC, Wilson DJ, Rosenbaum JT, Neuwelt EA. Intravitreal methotrexate as an adjunctive treatment of intraocular lymphoma. Arch Ophthalmol. 1997 Sep. 115(9):1152-6. [Medline].
Bhatia S, Paulino AC, Buatti JM, Mayr NA, Wen BC. Curative radiotherapy for primary orbital lymphoma. Int J Radiat Oncol Biol Phys. 2002 Nov 1. 54(3):818-23. [Medline].
Liao SL, Kao SC, Hou PK, Chen MS. Results of radiotherapy for orbital and adnexal lymphoma. Orbit. 2002 Jun. 21(2):117-23. [Medline].
Berenbom A, Davila RM, Lin HS, Harbour JW. Treatment outcomes for primary intraocular lymphoma: implications for external beam radiotherapy. Eye. 2007 Sep. 21(9):1198-201. [Medline].
Matsuo T, Yoshino T. Long-term follow-up results of observation or radiation for conjunctival malignant lymphoma. Ophthalmology. 2004 Jun. 111(6):1233-7. [Medline].
Eichler MD, Fraunfelder FT. Cryotherapy for conjunctival lymphoid tumors. Am J Ophthalmol. 1994 Oct 15. 118(4):463-7. [Medline].
Esmaeli B, Ahmadi MA, Manning J, McLaughlin PW, Ginsberg L. Clinical presentation and treatment of secondary orbital lymphoma. Ophthal Plast Reconstr Surg. 2002 Jul. 18(4):247-53. [Medline].
Martinet S, Ozsahin M, Belkacemi Y, Landmann C, Poortmans P, Oehlere C, et al. Outcome and prognostic factors in orbital lymphoma: a Rare Cancer Network study on 90 consecutive patients treated with radiotherapy. Int J Radiat Oncol Biol Phys. 2003 Mar 15. 55(4):892-8. [Medline].
Cheah CY, Milgrom S, Chihara D, Gombos DS, Pinnix CC, Dabaja BS, et al. Intensive chemoimmunotherapy and bilateral globe irradiation as initial therapy for primary intraocular lymphoma. Neuro Oncol. 2015 Oct 20. [Medline].
Benabid L, Desablens B, Defossez T, Malthieu D, Milazzo S, Turut P. [New treatment for orbital non-Hodgkin's lymphoma: 2 cases treated with rituximab]. J Fr Ophtalmol. 2005 Sep. 28(7):769-71. [Medline].
Savino G, Battendieri R, Gari M, Caputo CG, Laurenti L, Blasi MA. Long-term outcomes of primary ocular adnexal lymphoma treatment with intraorbital rituximab injections. J Cancer Res Clin Oncol. 2013 Apr 27. [Medline].
Bardenstein DS. Intraocular Lymphoma. Cancer Control. 1998 Jul. 5(4):317-325. [Medline].
McKelvie PA, McNab A, Francis IC, Fox R, O'Day J. Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Experiment Ophthalmol. 2001 Dec. 29(6):387-93. [Medline].
Woog JJ, Kim YD, Yeatts RP, Kim S, Esmaeli B, Kikkawa D, et al. Natural killer/T-cell lymphoma with ocular and adnexal involvement. Ophthalmology. 2006 Jan. 113(1):140-7. [Medline].
Jakobiec FA, Knowles DM. An overview of ocular adnexal lymphoid tumors. Trans Am Ophthalmol Soc. 1989. 87:420-42; discussion 442-4. [Medline].
Jenkins C, Rose GE, Bunce C, Cree I, Norton A, Plowman PN, et al. Clinical features associated with survival of patients with lymphoma of the ocular adnexa. Eye. 2003 Oct. 17(7):809-20. [Medline].
Choung H, Kim YA, Kim N, Lee MJ, Khwarg SI. Clinicopathologic Study of Chromosomal Aberrations in Ocular Adnexal Lymphomas of Korean Patients. Korean J Ophthalmol. 2015 Oct. 29 (5):285-93. [Medline].
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. [Medline].
Tang LJ, Gu CL, Zhang P. Intraocular lymphoma. Int J Ophthalmol. 2017. 10 (8):1301-1307. [Medline].

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Salmon-pink appearance of conjunctival lymphoma.
Homogeneous isodense orbital mass molding around the lateral ocular surface.

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