Clinical Manifestations of Intraocular Lymphoma

Hee Suh Min; Gon Yu Hyeong

doi:10.3341/jkos.2009.50.1.78

Journal List > J Korean Ophthalmol Soc > v.50(1) > 1008561

Go to TopGo to Top Go to BottomGo to Bottom

TOOLS

Min and Hyeong: Clinical Manifestations of Intraocular Lymphoma

Original Article

J Korean Ophthalmol Soc 2009;50(1):78-84.

Published online: 7 September 2009

DOI: https://doi.org/10.3341/jkos.2009.50.1.78

Clinical Manifestations of Intraocular Lymphoma

Hee Suh Min, M.D¹, Gon Yu Hyeong, M.D, PhD^1,^2,³

¹Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea

²Institute of Sensory Organs, Medical Research Center, Seoul National University, Seoul, Korea

³Institute of Rheumatology, Medical Research Center, Seoul National University, Seoul, Korea

Address reprint requests to Hyeong Gon Yu, MD, PhD Department of ophthalmology, Seoul National University College of Medicine #28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea Tel: 82-2-760-2438, Fax: 82-2-741-3187, E-mail: hgonyu@snu.ac.kr

Received 21 October 2008 Accepted 23 April 2008

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To investigate the clinical features and prognosis of primary intraocular lymphoma (PIOL). Methods: A retrospective review of medical records was performed in 9 patients who were diagnosed and treated as PIOL in the Department of Ophthalmology, Seoul National University Hospital.

Results

Among patients who were enrolled in the study, 14 eyes were examined. Thirteen eyes (92.9%) showed yellowish subretinal or choroidal infiltrates which is a characteristic finding of PIOL in fundus examination and fluorescein angiography. Three patients presented with ocular symptoms initially, and 5 patients later presented with central nerve system (CNS) involvement. Only 1 patient showed PIOL without CNS involvement. Among 6 patients (9 eyes) that received systemic chemotherapy or ocular irradiation, 5 patients (7 eyes, 77.8%) responded. Among those patients, 3 patients (4 eyes) showed relapse of PIOL. Five patients died during the mean follow-up period of 43.3 months, and the median survival time was 47 months.

Conclusions

The most common characteristic fundus finding of PIOL is subretinal or choroidal infiltration. Ocular irradiation combined with systemic chemotherapy is the first method of treatment, although long-term prognosis is poor.

Keywords: Primary central nervous system lymphoma, Primary intraocular lymphoma, Prognosis, Subretinal or choroidal infiltration, Vitreous biopsy

References

1. Chan CC, Buggage RR, Nussenblatt RB. Intraocular lymp-homa. Curr Opin Ophthalmol. 2002; 13:411–8.

2. Whitcup SM, de Smet MD, Rubin BI. . Intraocular lymphoma. Clinical and histopathologic diagnosis. Ophthal-mology. 1993; 100:1399–406.

3. Akpek EK, Ahmed I, Hochberg FH. . Intraocular-central nervous system lymphoma: clinical features, diagnosis, and outcomes. Ophthalmology. 1999; 106:1805–10.

4. Lee SH, Kim DJ, Kim IT. A case of primary central nervous system lymphoma with ocular involvement. J Korean Ophthal-mol Soc. 2005; 46:565–71.

5. Char DH, Ljung BM, Miller T, Phillips T. Primary intra-ocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management. Ophthalmology. 1988; 95:625–30.

6. Peterson K, Gordon KB, Heinemann MH, DeAngelis LM. The clinical spectrum of ocular lymphoma. Cancer. 1993; 72:843–9.

7. Augustein WG, Buggage RR, Smith JA. . Fluorescein angiogram interpretation in the diagnosis of primary intra-ocular lymphoma. Invest Ophthalmol Vis Sci. 2001; 42:S462.

8. Gill MK, Jampol LM. Variations in the presentation of primary intraocular lymphoma: Case reports and a review. Surv Ophthalmol. 2001; 45:463–71.

9. Levy-Clarke GA, Chan CC, Nussenblatt RB. Diagnosis and management of primary intraocular lymphoma. Hematol Oncol Clin North Am. 2005; 19:739–49.

10. Karma A, von Willebrand EO, Tommila PV. . Primary intraocular lymphoma: improving the diagnostic procedure. Ophthalmology. 2007; 114:1372–7.

11. Jahnke K, Korfel A, Komm J. . Intraocular lymphoma 2000-2005: results of a retrospective multicentre trial. Graefes Arch Clin Exp Ophthalmol. 2006; 244:663–9.

12. Zaldivar RA, Martin DF, Holden JT, Grossniklaus HE. Primary intraocular lymphoma: clinical, cytologic, and flow cytometric analysis. Ophthalmology. 2004; 111:1762–7.

13. Tuaillon N, Chan CC. Molecular analysis of primary central nervous system and primary intraocular lymphomas. Curr Mol Med. 2001; 1:259–72.

14. Hoffman PM, McKelvie P, Hall AJ, Stawell RJ, Santamaria JD. Intraocular lymphoma: a series of 14 patients with clinicopathological features and treatment outcomes. Eye 2003; 17. 513–21.

15. Coupland SE, Bechrakis NE, Anastassiou G. . Evaluation of vitrectomy specimens and chorioretinal biopsies in the diagnosis of primary intraocular lymphoma in patients with masquerade syndrome. Graefes Arch Clin Exp Ophthalmol. 2003; 241:860–70.

16. Batchelor TT, Kolak G, Ciordia R. . High-dose methotrexate for intraocular lymphoma. Clin Cancer Res. 2003; 9:711–5.

17. Batchelor T, Carson K, O'Neill A. . Treatment of primary CNS lymphoma with methotrexate and deferred radiotherapy: a report of NABTT 96–07. J Clin Oncol. 2003; 21:1044–9.

18. Hayabuchi N, Shibamoto Y, Onizuka Y. Primary central nervous system lymphoma in Japan: a national survey. Int J Radiat Oncol Biol Phys. 1999; 44:265–72.

Figure 1.

Case 1. Right eye. Dense sheet-like vitreal infiltration.

Figure 2.

Multiple dots of yellowish chorioretinal infiltration (A) and multiple hypofluoresecent dots intermingling with hyperfluorescent dots at the level of the retinal pigment epithelium, suggestive of an aggregate of tumor cells at fluorescein angiogram (B).

Figure 3.

Pretreatment fundus photograph demonstrates large infiltrative subretinal mass involving inferior retina and diffuse vitreous infiltration (A). Subretinal mass and vitreous infiltrationdisappeared 4 months after combined chemo-radiation therapy, and atrophy of optic disc and retina remained as a sequaele (B).

Figure 4.

Case 1 right eye. Wright-Giemsa stain of vitreous specimen shows immature pleomorphic lymphocytes with microcystic nucleus and ill-defined nucleus membrane (black arrows). Nectrotic atypical lymphocytes cells were also found (red arrows)(×200).

Figure 5.

Kaplan-Meier Survival curve for overall survival (median 43.3 months).

Table 1.

Demographics

No	Age/Sex	later ality	Ocular features	Sympto m-Diag nosis¹⁾	PIOL -PCN SL²⁾	Involve ment³⁾	No. & Result of ^‡PPVs	f Diagnosis based on	Patho-logy	-Treatment in chronological order (treatment on PCNSL) T R	Treatme nt Repons e⁴⁾	Outcome (months after diagnosis)	Ref 5)
1	M/56	^∗ R	panuveitis	10	-3	1,2	1, PIOL	PPV, Brain ^§ MRI	^П NA	(^‗‗ brRT)-PPV-^†† o cRT	Yes, Yes	^ПП AND 35	3
		^† L	panuveitis	10	-3	1,2	0	Brain MRI	NA	(brRT)-ocRTx-P PV	Yes, Yes	AWD (ocular) 35	3
2	F/57	R	Vitreitis with chorioretinal lesion	7	0	1,2,1	0	Brain biopsy	^# DLBL	L ^‡‡HDMTX, brRT	Yes, Yes	^## AWD (ocular) 14	3
		L	Vitreitis with chorioretinal lesion	7	0	1,2,1	0	Brain biopsy	DLBL	HDMTX, brRT	Yes, Yes	AWD (ocular) 14	3
3	M/63	R	panuveitis	7	0.5	1,2	0	Brain biopsy	DLBL	HDMTX	NA⁶⁾, No	Died 7.8	3
		L	panuveitis	7	0.5	1,2	0	Brain biopsy	DLBL	HDMTX	NA⁶⁾, No	Died 7.8	3
4	M /63	R	panuveitis	15	NA	11	1, Necroti atypical cells	ic PPV	NA	PPV-HDMTX	Yes, NA	AND 15	2
		L	panuveitis	15	NA	1 1	1,Necrotic atypical cells	c PPV	NA	PPV-HDMTX	Yes, NA	AND 15	2
5	F/64	R	panuveitis	9	-12	1 2,1	1, Necroti atypical cells	ic Brain biopsy, PPV	DLBL	(HDMTx,brRT)-PPV-ocRT	Yes, Yes	AND 47	1
		L	panuveitis	9	-12	2,1	0	Brain biopsy	DLBL	(HDMTx,brRT)-ocRT	Yes, Yes	AWD (ocular) 47	1
6	F/64	R	panuveitis	1	-57	2,1,2	0	Brain biopsy	DLBL	(CHOP,brRT)-H DMTX,ocRT	Yes, No	Died 84	1
7	F/71	R	posterior chorioretinal lesion	0.5	-24.5	2,1	0	Brain biopsy	DLBL	(HDMTx,brRT)	NA, Yes	AWD (ocular) 48.5	1
8	M/57	L	Vitreitis without chorioretinal lesion	NA	-48	2,1,2	0	Brain biopsy	DLBL	(HDMTx,brRT)	NA, No	Died 55	1
9	M/62	R	panuveitis	0.5	-60.5	2,1,2	0	Brain biopsy	DLBL	(^§§ CHOP,brRT)	NA, No	Died 83.8	1

1) Time (mo) from onset of ocular symptoms to diagnosis.
2) Time from the diagnosis of primary intraocular lymphoma (PIOL) to that of primary central nervous system lymphoma (PCNSL).
3) Choronological relationship of ocular (1), CNS (2) involvement.
4) Best response to treatment (ocular /systemic).
5) Status of the referral: 1 represents previously diagnosed of PCNSL, 2 represents referred from first order medical service; 3, secondary or tertiary order medical service.
6) Died 0.5 month after the initial chemotherapy due to progression of PCNSL.

^∗ R=right

^† L=left

^‡ PPV=pars plana vitrectomy

^§ MRI=magnetic resonance imaging

^П NA=not applicable

^# DLBL=diffuse large B-cell lymphoma

^‗‗ brRT=brain radiation

^†† ocRT=ocular radiation

^‡‡ HDMTX=high dose methotrexate

^§§ CHOP=doxorubicin vincristine, prednisolone and cyclophosphamide

^ПП AND=alive with no disease

^## AWD=alive with disease.

TOOLS

Similar articles