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

Min and Hyeong: Clinical Manifestations of Intraocular Lymphoma

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.

References

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Figure 1.
Case 1. Right eye. Dense sheet-like vitreal infiltration.
jkos-50-78f1.tif
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).
jkos-50-78f2.tif
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).
jkos-50-78f3.tif
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).
jkos-50-78f4.tif
Figure 5.
Kaplan-Meier Survival curve for overall survival (median 43.3 months).
jkos-50-78f5.tif
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.

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