Vitreoretinal lymphomas misdiagnosed as uveitis: Lessons learned from a case series

Abstract

Purpose:To present challenging cases of vitreoretinal lymphoma (VRL) that was misdiagnosed as uveitis because of the apparent intraocular inflammation. At the light of the new classification of intraocular lymphomas, we detail the characteristics that masqueraded the tumors and the clinical aspects that guided us to the correct diagnosis.Materials and Methods:We retrospectively reviewed the patients referred to our uveitis service between January 2006 and December 2014.Results:Seven patients referred with a presumptive diagnosis of idiopathic uveitis received a final diagnosis of VRL. The median time between the onset of symptoms and definitive diagnosis was 25 months for these complex cases. The median time from presentation at our clinic to final diagnosis was 1 month. The described clinical features including dense vitreous cells and subretinal infiltrates were characteristic and tend to be present in all these chronically ill patients. Vitreous samples were collected, and all demonstrated the pathognomonic tumor cells, the specific immunoglobulin heavy chain gene rearrangements, and an interleukin (IL)-10 to IL-6 ratio >1.Conclusion:VRLs are severe diseases with a poor prognosis that may be misdiagnosed as idiopathic inflammatory conditions of the eye. Treatment with steroids may occult the tumors and delay the correct diagnosis. Appropriate evaluation may prompt to a timely vitreous sampling and therefore to a faster diagnosis in these peculiar cases where the correct diagnosis was delayed by several months.

Masquerade Syndrome: Diagnostic Challenges in a Case of Primary Central Nervous System Lymphoma

“…The difficulty involved in making the correct diagnosis is compounded by the fact that visual symptoms associated with ocular lymphoma will typically have a dependent response to steroid treatment in half of presentations and also that the treatment for immune-mediated uveitis (which is oft initiated) will make diagnosis by vitreous biopsy more difficult [3,5,9]. The case that we present here fits the model of steroid-dependent relief of visual symptoms.…”

“…Ocular manifestations of primary CNS lymphoma typically initiate with only vitreous inflammation, making these cases easy to misinterpret as immune-mediated [4]. Later findings include yellow, sub-retinal deposits secondary to detachment of the Retinal Pigment Epithelium (RPE), considered pathognomonic for PVRL [5]. Cases of vitreoretinal lymphoma are described in the literature masquerading as chronic uveitis, retinal artery occlusion, as well as diffuse retinal vasculitis [5][6][7][8].…”

“…Later findings include yellow, sub-retinal deposits secondary to detachment of the Retinal Pigment Epithelium (RPE), considered pathognomonic for PVRL [5]. Cases of vitreoretinal lymphoma are described in the literature masquerading as chronic uveitis, retinal artery occlusion, as well as diffuse retinal vasculitis [5][6][7][8].…”

Survival and Recurrence in Vitreoretinal Lymphoma Simulating Uveitis at Presentation: The Possible Role of Combined Chemotherapy

“…The initial 7 patients have been previously reported; their outcomes after December 2014 were further examined. 12 Patients were referred to us for suspected uveitis. Presenting symptoms included floaters and painless loss of vision.…”

The incidence of uveitis after systemic lymphoma in Taiwan

“…In cases of intraocular involvement of systemic lymphoma, anterior or posterior intraocular inflammation may occur (masquerade syndrome). [9][10][11][12][13][14][15][16] No published study has investigated the incidence of uveitis after systemic lymphoma. Therefore, we conducted a nationwide cohort study by analyzing the claims data from the Taiwan National Health Insurance Research Database (NHIRD) during a follow-up period from 2000 to 2017 with ICD-9 codes to explore the risk of uveitis in patients with systemic lymphoma and non-systemic lymphoma in the Taiwanese population.…”