Background: A recent report has documented some cases of primary intraocular lymphoma (PIOL) with transient subretinal vitelliform deposits observed in the macular region in the early stage of the disease. Here, we report a case of PIOL-associated paraneoplastic cloudy vitelliform submaculopathy in which changes of diverse macular lesions were observed over time from the early stage of the disease with an optical coherence tomography (OCT).
Case description: The patient was a 49-year-old man who revealed chief complaints of decreased visual acuity and visual field defect in his left eye. In the early stage of the PIOL, findings of transient vitelliform maculopathy were noted, and OCT revealed a large amount of submacular deposits and a rippled and thickened line of the retinal pigment epithelium (RPE). Subsequently, emergence and extended fusion of multiple nodular lesions were noted in the peripheral retina and diverse retinal lesions were observed. A vitreous biopsy was thus performed and showed atypical lymphocytes in cytology and a markedly elevated interleukin (IL) 10/IL-6 ratio in a quantitative measurement of ILs. A diagnosis of PIOL was made as there were no abnormal intracranial magnetic resonance imaging findings. While the intraocular lesions regressed after intravitreal injections of methotrexate (MTX), an intracranial lesion was found in the course of treatment. Systemic high-dose MTX therapy and intracranial radiation therapy were thus performed and the mass was successfully reduced in size. The patient is currently on chemotherapy for the residual mass.
Conclusion: The present case suggests that transient vitelliform maculopathy findings and OCT findings of rippled and thickened RPE line are important for the diagnosis of the early stage of PIOL.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 121:871-877, 2017.