Orbital lymphoma

Orbital lymphoma
Orbital lymphoma
Classification and external resources
ICD-9 202.8
ICD-O: 9710–9719

Orbital lymphoma is common type of non-Hodgkin lymphoma that occurs near or on the eye. Common symptoms include decreased vision and uveitis. Orbital lymphoma can be diagnosed via a biopsy of the eye and is usually treated with Radiotherapy or with combination with chemotherapy.



Primary visible symptoms of ocular lymphoma include proptosis and a visible mass in the eye. Other symptoms are due to mass effect.


Recent studies[by whom?] have detected the presence of viral DNA in ocular lymphoma cells. This implies that pathogens play a role in ocular lymphoma. Other studies have found that the aging population, the increasing number of immunosuppressive drugs, and the AIDS epidemic have also contributed to the increased incidence of Non-Hodgkin lymphomas.

Ocular MALT lymphomas may also be associated with Chlamydia psittaci,[1][2] although whether or not this is the case is still debated.[3]

Follicular lymphoma, diffuse large B cell lymphoma, mantle cell lymphoma, B-cell chronic lymphocytic leukemia, peripheral T-cell lymphoma, and natural killer cell lymphoma have also been reported to affect the orbit.[citation needed]


55% of all lymphoma cases in adults and 10% in older patients is a form of ocular lymphoma.[4][5] In 2008, a prediction by the National Cancer Institute Surveillance, estimated that in 1,340 men and 1,050 women would be diagnosed with eye cancer and 240 people would die of the disease that year[6] Orbital lymphoma is more prevalent in Asia and Europe than in the United States.[7][8][9][10]

Although intraocular lymphoma is rare, the number of cases per year is rising, affecting mainly people in their seventies[11][12] and immunocompromised patients[13][14] A recent study has shown that ocular lymphoma is more prevalent in women than men.[15]

The survival rate is approximately 60% after 5 years.[citation needed]


There are two types of ocular lymphomas: intraocular lymphomas and adnexal lymphomas. An intraocular lymphoma occurs within the eye, while an adnexal lymphoma occurs outside, but adjoined to the eye.

Intraocular lymphoma

There are two main types of intraocular lymphomas: primary central nervous system involvement (PCNSL) and primary central nervous system with ocular involvement (PCNSLO). The difference between PCNSL and PCNSLO is that PNSCL involves the central nervous system, while PCNSLO doesn't. 56-86% of orbital lymphomas are classified PCNSL and 15-25% are classified PCNSLO.[16][17][18][19][20]

PCNSLO is common in people who are severely immunosuppressed.

Symptoms of this form of ocular lymphoma include painless decreased vision, photophobia, a red eye, and floaters. Diagnosis is difficult due to its gradual onset and the fact that the symptoms are the same as other diseases.

PCNSLO is usually bilateral, but sometimes grows unevenly. Like other metastatic tumors of the eye, it is usually confined to the choroid.[21]

Orbital and adnexal lymphoma


Radiotherapy is the most effective treatment for local disease either as the sole treatment for low-grade lymphoma or in combination with chemotherapy for intermediate- and high-grade lymphoma.[22][23] Radiotherapy dose in range of 30-45 Gy administered in fractions are advised in treating the local orbital lymphomas[24]


  1. ^ 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. Jan 15 2006;107(2):467-72.
  2. ^ 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. Jul 2006;209(3):344-51
  3. ^ 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. Jan 15 2006;107(2):467-72
  4. ^ Valvassori GE, Sabnis SS, Mafee RF, Brown MS, Putterman A. Imaging of orbital lymphoproliferative disorders. Radiol Clin North Am. Jan 1999;37(1):135-50, x-xi.
  5. ^ Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology. May 2004;111(5):997-1008.
  6. ^ "Surveillance, Epidemiology, and End Results Cancer Fact Stat Sheet, Cancer of the Eye and Orbit". National Cancer Institute. http://seer.cancer.gov/statfacts/html/eye.html. Retrieved 2008. 
  7. ^ Ohtsuka K, Hashimoto M, Suzuki Y. High incidence of orbital malignant lymphoma in Japanese patients. Am J Ophthalmol. Nov 2004;138(5):881-2.
  8. ^ 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. Apr 2006;34(3):239-44
  9. ^ 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. Aug 15 1998;83(4):806-12
  10. ^ Seregard S, Sahlin S. Panorama of orbital space-occupying lesions. The 24-year experience of a referral centre. Acta Ophthalmol Scand. Feb 1999;77(1):91-8
  11. ^ Moslehi R, Devesa SS, Schairer C, Fraumeni JF Jr. Rapidly increasing incidence of ocular non-hodgkin lymphoma. J Natl Cancer Inst. Jul 5 2006;98(13):936-9
  12. ^ Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older adult population. Ophthalmology. Feb 2002;109(2):243-8
  13. ^ 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.
  14. ^ Schabet M. Epidemiology of primary CNS lymphoma. J Neurooncol. Jul 1999;43(3):199-201
  15. ^ Ahmed S, Shahid RK, Sison CP, Fuchs A, Mehrotra B. Orbital lymphomas: a clinicopathologic study of a rare disease. Am J Med Sci. Feb 2006;331(2):79-83
  16. ^ Char DH, Ljung BM, Miller T, Phillips T. Primary intraocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management. Ophthalmology. May 1988;95(5):625-30
  17. ^ Char DH, Margolis L, Newman AB. Ocular reticulum cell sarcoma. Am J Ophthalmol. Apr 1981;91(4):480-3
  18. ^ Whitcup SM, de Smet MD, Rubin BI, Palestine AG, Martin DF, Burnier M Jr, et al. Intraocular lymphoma. Clinical and histopathologic diagnosis. Ophthalmology. Sep 1993;100(9):1399-406.
  19. ^ Peterson K, Gordon KB, Heinemann MH, DeAngelis LM. The clinical spectrum of ocular lymphoma. Cancer. Aug 1 1993;72(3):843-9
  20. ^ Freeman LN, Schachat AP, Knox DL, Michels RG, Green WR. Clinical features, laboratory investigations, and survival in ocular reticulum cell sarcoma. Ophthalmology. Dec 1987;94(12):1631-9.
  21. ^ Chan CC, Buggage RR, Nussenblatt RB. Intraocular lymphoma. Curr Opin Ophthalmology. Dec 2002;13(6):411-8
  22. ^ Pfeffer M R, Rabin T, Tsvang L, Goffman J, Rosen N, Symon Z (october 2004). "Orbital lymphoma: Is it necessary to treat the entire orbit?". International Journal of Radiation Oncology*Biology*Physics 60 (2): 527–530. 
  23. ^ Garg. Instant clinical diagnosis in ophthalmology: oculoplasty & reconstructive surgery. Jaypee Brothers Publishers, 2009. pp. 336. ISBN 9788184484038. 
  24. ^ Raymond E. Lenhard, Robert T. Osteen, Ted S. Gansler. Clinical oncology, Volume 1. Wiley-Blackwell. pp. 919. ISBN 9780944235157. 

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