Orbital lymphoma

Short description: Human disease of the eye

Orbital lymphoma

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.

## Contents

* 1 Types
* 2 Signs and symptoms
* 3 Pathophysiology
* 4 Diagnosis
* 4.1 Classification
* 5 Treatment
* 6 Epidemiology
* 7 References
* 8 External links

## 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[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,[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.[citation needed]

## 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.[citation needed]

## 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.
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.
6. ↑ 6.0 6.1 Rosado, MF; Byrne, GE Jr.; Ding, F; Fields, KA; Ruiz, P; Dubovy, SR; Walker, GR; Markoe, A et al. (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. PMID 16166588. PMC 1895606. http://bloodjournal.hematologylibrary.org/content/107/2/467.long. Retrieved 1 December 2012.
7. ↑ Chanudet, E; Zhou, Y; Bacon, CM; Wotherspoon, AC; Müller-Hermelink, HK; Adam, P; Dong, HY; de Jong, D et al. (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.
8. ↑ "Orbital lymphoma: Is it necessary to treat the entire orbit?". International Journal of Radiation Oncology, Biology, Physics 60 (2): 527–530. October 2004. 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. pp. 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. https://archive.org/details/americancancerso00lenh/page/919.
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. http://www.aaojournal.org/article/S0161-6420(03)01495-7/abstract. 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. http://www.ajo.com/article/S0002-9394(04)00650-6/abstract. 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.
15. ↑ Ko, YH; Kim, CW; Park, CS; Jang, HK; Lee, SS; Kim, SH; Ree, HJ; Lee, JD et al. (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". Cancer 83 (4): 806–12. doi:10.1002/(SICI)1097-0142(19980815)83:4<806::AID-CNCR26>3.3.CO;2-8. PMID 9708949. http://kumel.medlib.dsmc.or.kr/bitstream/2015.oak/35280/1/oak-aaa-00731.pdf.
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. http://jnci.oxfordjournals.org/content/98/13/936.long. 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. http://www.aaojournal.org/article/S0161-6420(01)00932-0/abstract. 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.
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.

## External links

Classification|

* ICD-10: C85.8
* ICD-9-CM: 202.8
* ICD-O: 9710–9719

|

* v
* t
* e

* Diseases of the human eye (H00–H59
* 360–379)

Adnexa

| Eyelid|

| Inflammation|

* Stye
* Chalazion
* Blepharitis

|

* Entropion
* Ectropion
* Lagophthalmos
* Blepharochalasis
* Ptosis
* Blepharophimosis
* Xanthelasma

Eyelash|

* Trichiasis
* Madarosis

Lacrimal apparatus|

* Dacryoadenitis
* Epiphora
* Dacryocystitis
* Xerophthalmia

Orbit|

* Exophthalmos
* Enophthalmos
* Orbital cellulitis
* Orbital lymphoma
* Periorbital cellulitis

Conjunctiva|

* Conjunctivitis
* allergic
* Pterygium
* Pinguecula
* Subconjunctival hemorrhage

Globe

| Fibrous tunic|

| Sclera|

* Scleritis
* Episcleritis

|

Cornea|

* Keratitis
* herpetic
* acanthamoebic
* fungal
* Corneal ulcer
* Photokeratitis
* Thygeson's superficial punctate keratopathy
* Corneal dystrophy
* Fuchs'
* Meesmann
* Corneal ectasia
* Keratoconus
* Pellucid marginal degeneration
* Keratoglobus
* Terrien's marginal degeneration
* Post-LASIK ectasia
* Keratoconjunctivitis
* sicca
* Corneal neovascularization
* Kayser–Fleischer ring
* Haab's striae
* Arcus senilis
* Band keratopathy

Vascular tunic|

* Iris
* Ciliary body

* Uveitis
* Intermediate uveitis
* Hyphema
* Rubeosis iridis
* Persistent pupillary membrane
* Iridodialysis
* Synechia

|

Choroid|

* Choroideremia
* Choroiditis
* Chorioretinitis

Lens|

* Cataract
* Congenital cataract
* Childhood cataract
* Aphakia
* Ectopia lentis

Retina|

* Retinitis
* Chorioretinitis
* Cytomegalovirus retinitis
* Retinal detachment
* Retinoschisis
* Ocular ischemic syndrome / Central retinal vein occlusion
* Central retinal artery occlusion
* Branch retinal artery occlusion
* Retinopathy
* diabetic
* hypertensive
* Purtscher's
* of prematurity
* Bietti's crystalline dystrophy
* Coats' disease
* Macular degeneration
* Retinitis pigmentosa
* Retinal haemorrhage
* Central serous retinopathy
* Macular edema
* Epiretinal membrane (Macular pucker)
* Vitelliform macular dystrophy
* Leber's congenital amaurosis
* Birdshot chorioretinopathy

Other|

* Glaucoma / Ocular hypertension / Primary juvenile glaucoma
* Floater
* Leber's hereditary optic neuropathy
* Red eye
* Globe rupture
* Keratomycosis
* Phthisis bulbi
* Persistent fetal vasculature / Persistent hyperplastic primary vitreous
* Persistent tunica vasculosa lentis
* Familial exudative vitreoretinopathy

Pathways

| Optic nerve
Optic disc|

* Optic neuritis
* optic papillitis
* Papilledema
* Foster Kennedy syndrome
* Optic atrophy
* Optic disc drusen

Optic neuropathy|

* Ischemic
* anterior (AION)
* posterior (PION)
* Kjer's
* Leber's hereditary
* Toxic and nutritional

Strabismus
Extraocular muscles
Binocular vision
Accommodation|

| Paralytic strabismus|

* Ophthalmoparesis
* Chronic progressive external ophthalmoplegia
* Kearns–Sayre syndrome

palsies|

* Oculomotor (III)
* Fourth-nerve (IV)
* Sixth-nerve (VI)

Other strabismus|

* Esotropia / Exotropia
* Hypertropia
* Heterophoria
* Esophoria
* Exophoria
* Cyclotropia
* Brown's syndrome
* Duane syndrome

Other binocular|

* Conjugate gaze palsy
* Convergence insufficiency
* Internuclear ophthalmoplegia
* One and a half syndrome

Refraction|

* Refractive error
* Hyperopia
* Myopia
* Astigmatism
* Anisometropia / Aniseikonia
* Presbyopia

Vision disorders
Blindness|

* Amblyopia
* Leber's congenital amaurosis
* Diplopia
* Scotoma
* Color blindness
* Achromatopsia
* Dichromacy
* Monochromacy
* Nyctalopia
* Oguchi disease
* Blindness / Vision loss / Visual impairment

Anopsia|

* Hemianopsia
* binasal
* bitemporal
* homonymous
* Quadrantanopia

subjective|

* Asthenopia
* Hemeralopia
* Photophobia
* Scintillating scotoma

Pupil|

* Anisocoria
* Argyll Robertson pupil
* Marcus Gunn pupil
* Adie syndrome
* Miosis
* Mydriasis
* Cycloplegia
* Parinaud's syndrome

Other|

* Nystagmus
* Childhood blindness

Infections

* Trachoma
* Onchocerciasis

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