접수번호: 2008-103 Korean Journal of Ophthalm ology 2009;23:210-214 ISSN : 1011-8942 DOI : 10.3341/kjo.2009.23.3.210 A Case of Primary Intraocular Lymphoma Treated by Intravitreal Methotrexate Eunah Kim, MD1, Changhyun Kim, MD1, Jiwoong Lee, MD2, Youngwook Cho, MD1 1Department of Ophthalmology, Daegu Fatima Hospital, Daegu, Korea 2Department of Ophthalmology, School of Medicine, Pusan National University, Busan, Korea A 40-year-old female visited our clinic for visual disturbance of the right eye, in which a few creamy-yellow retinal lesions and visual field constrictions were noted. She had been treated for primary CNS lymphoma and was in complete remission. After failure to follow-up for three months, she lost vision in the right eye, at which time active panuveitis was seen. Decreased vision and field constriction was observed in the left eye. Her left eye showed a granular pattern and dye leakage from the vessels and disc on fluorescein angiography and small RPE humps were seen in optical coherence tomography (OCT). Diffuse large malignant B-cells with strong immunoreactivities with CD20 immunostaining were seen in the epiretinal membrane biopsy specimen. Intravitreal injections of metho- trexate (MTX) (800 μg/0.1 ml in the right eye, 400 μg/0.05 ml in the left eye) were performed twice weekly for one month, once weekly for the following month, once every two weeks for the next month, followed by nine monthly injections. Both eyes were free from malignant cells on vitreous biopsy six months later. There was no leakage seen by angiography, but the granular pattern persisted. Visual field constriction was slightly improved, and the small RPE humpsdetachments seen in OCT disappeared. EOG Arden ratio was decreased in both eyes, and b wave amplitude of scotopic ERG was decreased in the left eye. She was free from recurrence until six months later. No ocular complications except minimal opacity of the crystalline lenses were noted in both eyes. Korean J Ophthalmol 2009;23:210-214 ⓒ 2009 by the Korean Ophthalmological Society. Key Words: Intravitreal injection, Masquerade syndrome, Methotrexate, Primary intraocular lymphoma, Uveitis Primary intraocular lymphoma (PIOL) is considered to be a subset of primary CNS lymphoma (PCNSL).1 One-fifth of patients with PCNSL may demonstrate ocular involvement at the time of diagnosis, and the eye can be involved prior of the onset of CNS disease.2,3 The exact incidence of PIOL is un- known, but it is reported to be in the range of 30 to 50 cases per 10 million people in the United States.4,5 Only one case has been reported in Korea.6 So far, there are three main treatment options for PIOL; sys- temic high-dose MTX injection, orbital irradiation, and intra- vitreal chemotherapy with MTX injections. These may be com- bined or used separately.7,8 Initial approaches for the treatment of PIOL/PCNSL centered on radiotherapy, but it had a minimal effect on survival and may have been associated with radiation retinopathy.9 Isolated radiation therapy is currently less com- monly used,10 and treatment can be limited to the eye in pa- tients with PIOL limited to the eye.11 There are some reports of PIOL effectively treated with intravitreal chemotherapy, and Received: October 13, 2008 Accepted: August 7, 2009 Reprint requests to Youngwook Cho, MD. Department of Ophthalmology, Fatima Hospital, #576-31 Sinam-dong, Dong-gu, Daegu 701-600, Korea. Tel: 82-53-940-7143, Fax: 82-53-954-7417, E-mail: vitreo-retina@hanmail.net * This study was presented as a poster at the 99th annual meeting of the Korean Ophthalmological Society in April, 2008. 210 it has been often recommended as a first-line therapy for initial and recurrent PIOL.11-13 To our knowledge, this is the first report of PIOL treated by intravitreal chemotherapy, and the first to describe fluorescein angiographic findings, visual field exams, OCT findings and the electrophysiological characteristics of PIOL in Koreas. Case Repo rt A 40-year-old female visited our clinic for agradual visual disturbance in her right eye in January 2007. Her best-corrected visual acuity (BCVA) was 20/160 OD and 20/20 OS. A few barely detectable creamy-yellow retinal lesions were seen in the right eye. Eighteen months prior, she had been diagnosed with PCNSL in the right temporo-parietal lobe which had been pathologically confirmed as malignant large B-cells. Systemic chemotherapy with MTX was used initially, but it failed. Com- plete remission was achieved by whole brain irradiation. A gra- nular pattern of mixed hyperfluorescent and hypofluorescent spots, along with leakage from the retinal vessels and the optic disc, were observed in fluorescein angiography of the right eye. Her visual field was considerably constricted and OCT (STRA- TUS OCT model 3000, Zeiss Instruments INC. San Leandro, CA) showed small irregular RPE detachments in the right eye (Fig. 1). P1 amplitude and latency of the right eye were mar- kedly decreased compared with those of the left eye, as deter-