Multimodal Imaging and Clinicopathologic Correlation in Primary Uveal Lymphoma

Abstract

Purpose: We report a rare case of primary uveal lymphoma and characterize it using histopathology and multimodal imaging.

Patient and methods: A 41-year-old male presented with a 2-year history of increasingly blurry vision in his right eye and no systemic symptoms. Examination revealed a retinal detachment and mass lesion in the right eye. Radiologic and histologic testing was performed.

Results: Multimodal imaging localized the lesion to the choroid, and fine needle aspiration biopsy diagnosed the lesion as a low-grade B-cell lymphoma. The patient was treated with external beam radiation, resulting in regression of the mass and resolution of the retinal detachment.

Conclusions: Primary uveal lymphoma is a rare, usually indolent tumor that carries a good prognosis. In this case, we show that primary uveal lymphoma has distinct findings via histopathology and multimodal imaging, and that imaging after radiation treatment documents disease regression.

Keywords: Fluorescein angiography; Histopathology; Optical coherence tomography; Primary uveal lymphoma.

Fig. 1

a–e Initial presentation. a OCT showing subretinal fluid, elevated choroid, and atrophic ONL (horizontal black bar in d shows corresponding area of fundus). b Axial T1 MRI showing hyperintense, thickened choroid and detachment. c Transverse B-scan showing bullous retinal detachment and irregular choroidal thickening. d Color fundus image showing choroidal mass lesion (arrowheads) and retinal detachment (arrows). e Sub-RPE and choroidal mass are highlighted with increased autofluorescence in the RPE. f–h 4.5 months after EBRT. f Color fundus showing mass regression, as well as prominent choroidal vasculature, RPE hyperplasia, and residual scarring. g OCT showing regression of mass and absence of subretinal fluid, along with some RPE atrophy and subretinal scar (horizontal black bar in f shows corresponding area of fundus). h Transverse B-scan showing mass regression and resolution of retinal detachment.

Fig. 2

a Fine needle aspirate showing small lymphocytes, scattered pigmented and nonpigmented cells, and proteinaceous material (cell block HE; magnification ×1,000). b Immunohistochemical stain showing CD20 B lymphocytes (magnification ×1,000). c Immunohistochemical stain showing few CD3 T lymphocytes (magnification ×1,000).