- Title: Cloudy Vitelliform Submaculopathy as an Early Sign of Primary Vitreoretinal Lymphoma

- Key Focus: Primary Vitreoretinal Lymphoma (PVRL) is a rare intraocular malignant tumor, with diagnosis relying on cytological evidence of lymphomatous cells in the eye.

  

- Case Presentation:

  - A 51-year-old man presented with a 1-week history of blurred vision in his right eye.

  - Visual acuity was 20/100 OD (right eye).

  - Fundus examination revealed multifocal yellow-white retinal infiltrating lesions.

  - Optical Coherence Tomography (OCT) showed a homogeneously high reflective submacular vitelliform lesion.

- Diagnostic Imaging and Findings:

  - Retinal Fundus Photograph: Showed cloudy milky opacity and yellow-white infiltrating lesions.

  - SD-OCT: Revealed cloudy vitelliform submaculopathy, which regressed after oral prednisone treatment but reappeared as focal intraretinal infiltrations after discontinuation.

  - MRI of the Brain: Revealed a hyperintense lesion in the right frontal lobe, suspecting lymphoma.

  - Cytopathology: Atypical medium-sized lymphomatous cells were identified in the vitreous, staining positive for CD20.

- Treatment and Outcome:

  - Initially treated with oral prednisone for inflammatory outer retinopathy, leading to temporary vision improvement.

  - After discontinuation of prednisone, intraretinal infiltrations reappeared.

  - Diagnosed with PVRL and treated with intravitreal and systemic chemotherapy.

  - Visual acuity recovered to 20/20 in both eyes, with residual outer retinal abnormalities.

- Key Observations:

  - Cloudy vitelliform submaculopathy is an early transient manifestation of PVRL, which can subside within three months.

  - 65%-90% of PVRL patients progress to primary central nervous system lymphoma within 29 months.

  - Cerebral MRI is recommended as part of the initial workup when PVRL is suspected.