Two cases of primary vitreoretinal lymphoma: a diagnostic challenge : The supporting role of multimodal imaging in the diagnosis of primary vitreoretinal lymphoma

Int Ophthalmol. 2018 Feb;38(1):353-361. doi: 10.1007/s10792-016-0422-1. Epub 2016 Dec 30.

Abstract

Purpose: To report two cases of primary vitreoretinal lymphoma (PVRL), which presented as intermediate and posterior uveitis.

Methods: Combined clinical assessment, multimodal imaging with spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, brain magnetic resonance imaging and vitreous and retinal biopsy. Case 1 was a 48-year-old woman who complained of visual loss in her right eye secondary to a diffuse vitreous opacification and multiple chorioretinal lesions. Case 2, a 74-year-old man, presented with low vision in his right eye due to a wide chorioretinal lesion at the posterior pole, vitreous opacification and posterior uveitis in both eyes.

Results: Diffuse large B cell lymphoma was histologically diagnosed in the cerebellum in the first case and in chorioretinal tissue in the second patient. Atypical lymphoid cells were detected and allowed to make a diagnosis of primary central nervous system lymphoma in case 1 and PVRL in case 2.

Conclusion: PVRL often masquerades ad intermediate or posterior uveitis. The management of the patients needed a team of pathologists, haematologists and ophthalmologists to achieve the correct diagnosis and choose the more appropriate therapy. Some peculiar characteristics on multimodal imaging, even in atypical cases of PVRL, should raise suspicious for PVRL and lead to a diagnostic vitrectomy and/or retinal biopsy.

Keywords: Optical coherence tomography; Primary central nervous system lymphoma; Primary vitreoretinal lymphoma; Retinal biopsy; Vitreous biopsy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Biopsy
  • Diagnosis, Differential
  • Female
  • Fluorescein Angiography / methods*
  • Follow-Up Studies
  • Fundus Oculi
  • Humans
  • Lymphoma, Large B-Cell, Diffuse / complications
  • Lymphoma, Large B-Cell, Diffuse / diagnosis*
  • Lymphoma, Large B-Cell, Diffuse / surgery
  • Male
  • Middle Aged
  • Multimodal Imaging / methods*
  • Retina / pathology*
  • Retinal Neoplasms / complications
  • Retinal Neoplasms / diagnosis*
  • Retinal Neoplasms / surgery
  • Tomography, Optical Coherence
  • Uveitis, Posterior / diagnosis
  • Uveitis, Posterior / etiology*
  • Uveitis, Posterior / surgery
  • Visual Acuity
  • Vitrectomy
  • Vitreous Body / pathology*