Mak KS,1 Miller RC,2 Krishnan S,3 Laperriere N,4 Micke O,5 Rutten I,6 Kadish SP,7 Ozsahin M,8 and Mirimanoff RO81Harvard Medical School, Boston, MA, 2Mayo Clinic, Rochester, MN, 3University of Texas M. D. Anderson Cancer Center, Houston, TX, 4Princess Margaret Hospital, Toronto, ON, Canada, 5Franziskus Hospital, Bielefeld, Germany, 6Centre Hospitalier Universitaire de Liège, Liège, Belgium, 7University of Massachusetts Medical School, Worcester, MA, 8University Hospital Center and University of Lausanne, Switzerland

OUTCOMES AND PROGNOSTIC FACTORS IN PRIMARY INTRAOCULAR LYMPHOMA: A MULTICENTER RARE CANCER NETWORK STUDY

OBJECTIVEPrimary intraocular lymphoma (PIOL) is a rare, malignant non-Hodgkin’s lymphoma, representing a subset of primary central nervous system lymphoma (PCNSL). Its natural history and outcome are thought to be worse than that of intraorbital lymphoma (Martinet et al., IJROBP, 2003), based on very small series or case reports. The goal of this study was to assess the clinical profile, treatment outcome, and prognostic factors in patients with Ann Arbor Stage I-E PIOL treated with radiation therapy and/or chemotherapy.

MATERIALS & METHODS

CONCLUSIONSPIOL has distinct clinical features and outcome, and its overall prognosis is fair. After therapy, local control was good but the rate of CNS recurrence was high, occurring in half of cases. Vitreous involvement carries a worse prognosis. CNS prophylaxis as well as more aggressive and innovative therapies need to be considered in this rare lymphoma entity.

Male/Female casesMedian ageMedian follow-upMedian time from onset of

symptoms to diagnosis

10/1062 years

46.5 months

10 months

(n = 20)(range: 48-82)(range: 8-151)

(range: 1-26)

Common symptomsDecreased visual acuityBlurred visionFloaters

Masquerading DiagnosesUveitisVitritis

TreatmentRadiation therapyRadiation and chemotherapyChemotherapy alone

Radiation therapyMedian dose

Tumor characteristicsBilateral diseaseLocation in vitreousLocation in choroid, iris,

ciliary body, or retinaB cell origin reported

n 1442

n 9

12

816

n20156

n 127

37 Gy

%1007530

%6035

%702010

(range: 16-46)

%4560

4080

RESULTS

In a median follow-up period of 46.5 months:Cases alive without disease 45% n=9Cases alive with disease 25% n=5Cases dead with disease 25% n=5Cases dead without disease 5% n=1

Late Toxicities:Cataracts 20% n=4 Retinopathy 10% n=2Dry eye syndrome 5% n=1

Survival Rates (Figure 1):Median overall survival 79 months 5-year overall survival 55%5-year disease free survival 39%5-year local control rate 72%

Recurrence Rates: Local recurrence 20% n=4CNS recurrence (Figure 2) 50% n=10Systemic recurrence 5% n=15-year probability of CNS relapse 51%

Case report of PIOL with CNS recurrence

A 60-year old man was diagnosed with bilateral PIOL, confirmed by cytology (Figure 3A) and PCR as B cell in origin. Work-up was negative for any other lymphoma site. RT (36 Gy in 18 fractions) was delivered to both eyes, with complete remission. One year later, the patient developed a left hemiparesis. MRI disclosed a right periventricular mass (3B), consistent with lymphoma spread to the brain. High-dose methotrexate resulted in partial remission. Consolidation RT was then administered with a total dose of 50.4 Gy (3C, 3D).

Figure 3: Case report of PIOL with CNS recurrence3A: Cytology from sampling of vitreous fluid, revealing abnormal lymphoid cells with enlarged nuclei3B: Brain MRI demonstrating R periventricular mass3C, 3D: 3-D RT planning of brain lesion

Figure 1: Local control rate, overall survival, and disease-free survival for the series of twenty patients

Figure 2: Cumulative rate of CNS relapse in the series of twenty patients

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