Atypical presentation of primary intraocular lymphoma

Abstract

BackgroundIn 2014, Pang et al. reported three cases with vitelliform submaculopathy as a preceding lesion of primary intraocular lymphoma (PIOL). Here, we report a case with an atypical presentation of PIOL who initially presented with vitelliform submaculopathy, vitreous haze and preripheral retinal focus.Case presentationA 73-year-old female initially visited another hospital with a chief complaint of acute reduced vision in the right eye. Funduscopic examination of the right eye showed a yellowish retinal lesion at the fovea with vitreous haze and retinal foci scattered in the peripheral region. Spectral-domain optic coherence tomography (SD-OCT) revealed a hyperreflective subretinal debris above the retinal pigment epithelium (RPE) at the fovea, suggesting vitelliform submaculopathy. Vitrectomy was performed to improve visualization of the retinal lesions and for examination of PIOL. Vitreous cytology was class III and cytokine analysis of vitreous fluid showed increased IL-10 and an IL-10/IL-6 ratio >1, suggesting PIOL. Thereafter, there was a sub-RPE infiltration of presumed lymphoma in the nasal retina, and PCR analysis of anterior chamber fluid indicated IgH gene rearrangement, leading to diagnosis of PIOL. Three months later, there was complete disappearance of the vitelliform submacular lesion, with resultant disruption and thinning of the outer retinal layers on SD-OCT images.ConclusionsClinicians should be aware of atypical manifestations of PIOL such as vitelliform submaculopathy and peripheral retinal foci with vitreous haze. The patient’s unusual funduscopic changes are findings that have not reported in patients with PIOL.

Survival in B-Cell Primary Ocular Lymphoma 1997–2014: A Population-Based Study

“…[1][2][3] Primary intraocular lymphoma (PIOL) accounts for 1.86% of malignant ocular tumors; B-cell PIOL is most common, though some malignancies are derived from T cells and natural killer cells. 4 Based on the tumor site, PIOLs include vitreoretinal lymphoma, choroidal lymphoma, iris lymphoma, and ciliary body lymphoma. According to the Surveillance, Epidemiology, and End Results (SEER) program, the incidence of PIOL was approximately 0.48 per 100,000 individuals in the USA .…”

“…POL includes many pathological types with varying degrees of malignancy and may be difficult to distinguish from other ocular diseases such as retinal choroidal reactive lymphoid hyperplasia and orbital inflammatory pseudotumor 1–3. Primary intraocular lymphoma (PIOL) accounts for 1.86% of malignant ocular tumors; B-cell PIOL is most common, though some malignancies are derived from T cells and natural killer cells 4. Based on the tumor site, PIOLs include vitreoretinal lymphoma, choroidal lymphoma, iris lymphoma, and ciliary body lymphoma.…”

Electroretinographic and Optical Coherence Tomographic Evaluations of Eyes with Vitreoretinal Lymphoma

“…OCT is an important and helpful noninvasive method in diagnosing and managing retinal diseases including VRL. Although several studies have been published that illustrated the OCT manifestations of primary VRLs or VRLs associated with CNS lymphomas [16][17][18][19][20]24,28,29,[32][33][34][35][36][37][38][39][40][41][42][43][44][45], most of the morphologic features were nonspecific [16,32,33]. Our cases also had some of the features found in other studies; however, these features were labelled differently by different authors(Supplemental Table S1) [16][17][18][19][20]24,28,29,[32][33][34][35][36][37][38][39][40][41][42][43][44]…”

“…OCT is an important and helpful noninvasive method in diagnosing and managing retinal diseases including VRL. Although several studies have been published that illustrated the OCT manifestations of primary VRLs or VRLs associated with CNS lymphomas [ 16 , 17 , 18 , 19 , 20 , 24 , 28 , 29 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 ], most of the morphologic features were nonspecific [ 16 , 32 , 33 ]. Our cases also had some of the features found in other studies; however, these features were labelled differently by different authors ( Supplemental Table S1 ) [ 16 , 17 , 18 , 19 , 20 , 24 , 28 , 29 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 ].…”

Presumed intraocular lymphoma masquerading as age-related macular degeneration: A case report

“…In 2016, Komatsu et al 9 reported a case of primary intraocular lymphoma (PIOL), in which vitelliform maculopathy was accompanied with vitreous organization and sub-RPE infiltration in the peripheral retina. Pang et al 4 pointed out the importance of vitelliform maculopathy as a preceding lesion in PIOL in three cases.…”