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

Abstract

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.

Clinical Features of Primary and Systemic Metastatic Intraocular Lymphomas in Spanish Patients

“…Nevertheless, demographical data in our study matched very well with previously published PVRL series [4, 5, 11]. As classically described, main ocular signs found in our PVRL patients were vitritis (76%), retinal (29%), and/or subretinal (29%) yellowish infiltrates [12]; anterior chamber cells greater than 1+ (SUN scale) was only seen in one eye. We also found one eye with hemorrhagic pseudohypopyon, and 17% of the eyes showed some degree of retinal vascular involvement.…”

First observation of intraocular extranodal natural killer/T-cell lymphoma secondary to a retroperitoneal tumour: a case report and comparative review

“…In this study, a case of vitreoretinal ENKTL secondary to the retroperitoneum, without nasal lesions, was described. Similar to a published report, metastatic lymphomatous infiltrates were confined to the space between the RPE and Bruch's membrane, which remained intact [ 6 ]. Based on the monophyletic diagnostic theory, researchers have proposed that vitreoretinal and retroperitoneal tumours are homologous.…”

“…Metastatic intraocular tumours commonly develop secondarily to invasion from the nasopharynx [ 33 – 35 ]. Only two cases of metastatic intraocular ENKTL without infiltration of the nasal cavity or paranasal sinuses have been reported previously [ 6 , 36 ]. In this study, a case of vitreoretinal ENKTL secondary to the retroperitoneum, without nasal lesions, was described.…”

“…Moreover, the positive EBV testing result and oncogenic mutations related to T-cell-derived lymphomas supported the diagnosis. Lymphomatous infiltrates are commonly confined to the space between the RPE and Bruch's membrane in metastatic intraocular ENKTL [ 6 ]. Her nasopharyngeal area, a common site for ENKTL, was specifically examined to exclude possible lesions.…”

Extranodal natural killer/T-cell lymphoma invading a patient’s heart: A rare case report and literature review

“…Whole-body PET-CT with echocardiography is indispensable in the diagnostic process of such diseases. Moreover, this type of patient has poor prognosis, often with death as the clinical outcome (Table 2) (1,(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). These features were also seen in our reported cases.…”