Beneath the Retinal Pigment Epithelium: Histopathologic Findings in Metastatic Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type.

Journal Article

PURPOSE: To report a case of extranodal natural killer/T-cell lymphoma (ENKTCL), nasal type metastatic to the space beneath the retinal pigment epithelium (RPE) with coincident paraneoplastic lymphoma-associated retinopathy. METHODS: Findings of clinical and histopathologic examination are presented with differential diagnoses and a literature review. CASE REPORT: A 53-year-old man presented with bilateral blindness and had exudative retinal detachments overlying subretinal masses in both eyes. Flow cytometry of pericardial fluid revealed malignant T lymphocytes. After two cycles of chemotherapy, the patient was hospitalized and quickly expired. Autopsy revealed lymphoma involving the eyes, heart, right lung, and two subcarinal lymph nodes focally. Histopathologic examination of the eyes revealed intraocular metastases from ENKTCL, nasal type. Expression of CD3 and CD56, along with expression of Epstein-Barr virus by in situ hybridization, confirmed the diagnosis. Lymphomatous infiltrates were confined to the space beneath the neurosensory retina and between the RPE and the Bruch membrane, sparing the uveal tissue, similar to other metastatic T-cell lymphomas. Extensive RPE and photoreceptor loss in regions with and without underlying tumor was typical of a concurrent paraneoplastic lymphoma-associated retinopathy. CONCLUSION: Patients diagnosed with ENKTCL should be evaluated by an ophthalmologist, as ophthalmic involvement portends a poor prognosis.

Full Text

Duke Authors

Cited Authors

  • Thompson, AC; McCall, CM; Proia, AD

Published Date

  • November 2018

Published In

Volume / Issue

  • 4 / 6

Start / End Page

  • 388 - 394

PubMed ID

  • 30574492

Pubmed Central ID

  • PMC6288667

International Standard Serial Number (ISSN)

  • 2296-4681

Digital Object Identifier (DOI)

  • 10.1159/000487268


  • eng

Conference Location

  • Switzerland