Orbital lymphoma
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Orbital lymphoma is a 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 in combination with chemotherapy.

Types

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 does not. 56-86% of orbital lymphomas are classified PCNSL and 15-25% are classified PCNSLO.[1][2][3][4]

PCNSLO is common in people who are severely immunosuppressed.

Symptoms of this form of ocular lymphoma include painless decreased vision, sensitivity to light, 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.[5]

Signs and symptoms

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

Pathophysiology

Recent studies 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,[6][7] although whether or not this is the case is still debated.[6]

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.

Diagnosis

Classification

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.

Treatment

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.[8][9] Radiotherapy dose in range of 30-45 Gy administered in fractions are advised in treating the local orbital lymphomas.[10]

Epidemiology

Orbital lymphoma accounts for 55% of malignant orbital tumors in adults.[11] In one study, this was 10% of patients presenting with orbital tumors or similar lesions.[12] Orbital lymphoma is more prevalent in Asia and Europe than in the United States.[13][14][15][16]

Although intraocular lymphoma is rare, the number of cases per year is rising, affecting mainly people in their seventies[17][18] and immunocompromised patients.[19][20] A recent study has shown that ocular lymphoma is more prevalent in women than men.[21]

The survival rate is approximately 60% after 5 years.

References

  1. ↑ Char, DH; Ljung, BM; Miller, T; Phillips, T (May 1988). "Primary intraocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management". Ophthalmology. 95 (5): 625–30. doi:10.1016/s0161-6420(88)33145-3. PMID 3050698.
  2. ↑ Char, DH; Margolis, L; Newman, AB (April 1981). "Ocular reticulum cell sarcoma". American Journal of Ophthalmology. 91 (4): 480–3. doi:10.1016/0002-9394(81)90236-1. PMID 7013487.
  3. ↑ Peterson, K; Gordon, KB; Heinemann, MH; DeAngelis, LM (Aug 1, 1993). "The clinical spectrum of ocular lymphoma". Cancer. 72 (3): 843–9. doi:10.1002/1097-0142(19930801)72:3<843::AID-CNCR2820720333>3.0.CO;2-#. PMID 8334638. S2CID 196366844.
  4. ↑ Freeman, LN; Schachat, AP; Knox, DL; Michels, RG; Green, WR (December 1987). "Clinical features, laboratory investigations, and survival in ocular reticulum cell sarcoma". Ophthalmology. 94 (12): 1631–9. doi:10.1016/s0161-6420(87)33256-7. PMID 3323986.
  5. ↑ Chan, CC; Buggage, RR; Nussenblatt, RB (December 2002). "Intraocular lymphoma". Current Opinion in Ophthalmology. 13 (6): 411–8. doi:10.1097/00055735-200212000-00012. PMID 12441846. S2CID 22751067.
  6. 1 2 Rosado, MF; Byrne, GE Jr.; Ding, F; Fields, KA; Ruiz, P; Dubovy, SR; Walker, GR; Markoe, A; Lossos, IS (January 15, 2006). "Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci". Blood. 107 (2): 467–72. doi:10.1182/blood-2005-06-2332. PMC 1895606. PMID 16166588. Retrieved 1 December 2012.
  7. ↑ Chanudet, E; Zhou, Y; Bacon, CM; Wotherspoon, AC; Müller-Hermelink, HK; Adam, P; Dong, HY; de Jong, D; Li, Y; Wei, R; Gong, X; Wu, Q; Ranaldi, R; Goteri, G; Pileri, SA; Ye, H; Hamoudi, RA; Liu, H; Radford, J; Du, MQ (July 2006). "Chlamydia psittaci is variably associated with ocular adnexal MALT lymphoma in different geographical regions". The Journal of Pathology. 209 (3): 344–51. doi:10.1002/path.1984. PMID 16583361. S2CID 41716903.
  8. ↑ Pfeffer MR, 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. doi:10.1016/j.ijrobp.2004.03.039. PMID 15380588.
  9. ↑ Garg (2009). Instant clinical diagnosis in ophthalmology: oculoplasty & reconstructive surgery. Jaypee Brothers Publishers, 2009. p. 336. ISBN 978-81-8448-403-8.
  10. ↑ Raymond E. Lenhard; Robert T. Osteen; Ted S. Gansler (2000-12-27). Clinical oncology, Volume 1. Wiley-Blackwell. pp. 919. ISBN 978-0-944235-15-7.
  11. ↑ Valvassori, GE; Sabnis, SS; Mafee, RF; Brown, MS; Putterman, A (Jan 1999). "Imaging of orbital lymphoproliferative disorders". Radiologic Clinics of North America. 37 (1): 135–50, x–xi. doi:10.1016/S0033-8389(05)70083-X. PMID 10026734.
  12. ↑ Shields, Jerry A; Shields, Carol L; Scartozzi, Richard (May 2004). "Survey of 1264 patients with orbital tumors and simulating lesions". Ophthalmology. 111 (5): 997–1008. doi:10.1016/j.ophtha.2003.01.002. PMID 15121380. Retrieved 1 December 2012.
  13. ↑ Ohtsuka, Kenji; Hashimoto, Masato; Suzuki, Yasuo (Nov 2004). "High incidence of orbital malignant lymphoma in Japanese patients". American Journal of Ophthalmology. 138 (5): 881–882. doi:10.1016/j.ajo.2004.05.069. PMID 15531337. Retrieved 1 December 2012.
  14. ↑ Shikishima, Keigo; Kawai, Kazushige; Kitahara, Kenji (April 2006). "Pathological evaluation of orbital tumours in Japan: analysis of a large case series and 1379 cases reported in the Japanese literature". Clinical and Experimental Ophthalmology. 34 (3): 239–244. doi:10.1111/j.1442-9071.2006.01192.x. PMID 16671904. S2CID 23434526.
  15. ↑ Ko, YH; Kim, CW; Park, CS; Jang, HK; Lee, SS; Kim, SH; Ree, HJ; Lee, JD; Kim, SW; Huh, JR (Aug 15, 1998). "REAL classification of malignant lymphomas in the Republic of Korea: incidence of recently recognized entities and changes in clinicopathologic features. Hematolymphoreticular Study G roup of the Korean Society of Pathologists. Revised European-American lymphoma" (PDF). Cancer. 83 (4): 806–12. doi:10.1002/(SICI)1097-0142(19980815)83:4<806::AID-CNCR26>3.3.CO;2-8. PMID 9708949.
  16. ↑ Seregard, S; Sahlin, S (February 1999). "Panorama of orbital space-occupying lesions. The 24-year experience of a referral centre". Acta Ophthalmologica Scandinavica. 77 (1): 91–8. doi:10.1034/j.1600-0420.1999.770121.x. PMID 10071158.
  17. ↑ Moslehi, R.; Devesa, S. S.; Schairer, C.; Fraumeni, J. F. (July 2006). "Rapidly Increasing Incidence of Ocular Non-Hodgkin Lymphoma". JNCI Journal of the National Cancer Institute. 98 (13): 936–939. doi:10.1093/jnci/djj248. PMID 16818858. Retrieved 1 December 2012.
  18. ↑ Demirci, H; Shields, CL; Shields, JA; Honavar, SG; Mercado, GJ; Tovilla, JC (February 2002). "Orbital tumors in the older adult population". Ophthalmology. 109 (2): 243–8. doi:10.1016/S0161-6420(01)00932-0. PMID 11825802. Retrieved 1 December 2012.
  19. ↑ 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.
  20. ↑ Schabet, M (July 1999). "Epidemiology of primary CNS lymphoma". Journal of Neuro-Oncology. 43 (3): 199–201. doi:10.1023/A:1006290032052. PMID 10563423. S2CID 36849488.
  21. ↑ Ahmed, S; Shahid, RK; Sison, CP; Fuchs, A; Mehrotra, B (February 2006). "Orbital lymphomas: a clinicopathologic study of a rare disease". The American Journal of the Medical Sciences. 331 (2): 79–83. doi:10.1097/00000441-200602000-00013. PMID 16479179. S2CID 30013904.
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