Development of Extranodal NK/T-cell Lymphoma Nasal Type in Cerebrum Following Epstein-Barr Virus-positive Uveitis

Abstract

A 74-year-old woman developed bilateral uveitis with high Epstein-Barr virus (EBV) DNA load in the vitreous fluid without lymphoma cells. Four years after the onset, T2-weighted contrast-enhanced MRI revealed hyperintense lesions in the right occipital and parietal lobe. A biopsy resulted in the diagnosis of extranodal NK/T-cell lymphoma nasal type (ENKL). The repeat region of LMP1, an EBV gene, detected in the brain lesion was identical to that detected in the vitreous fluid. ENKL of the central nervous system is quite rare, and the pathogenesis has not been determined. The lymphoma in this case might have been closely associated with the EBV-positive uveitis.

Primary Intraocular Lymphoma in a Patient with Bilateral Epstein-Barr Virus Panuveitis

“…Therefore, it is wondered whether the retinitis in this patient was neoplastic. A similar case was reported by Imai et al [ 10 ] that was ultimately observed to have an intraocular natural killer (NK)/T-cell lymphoma. Although several cases of intraocular NK/T-cell lymphoma have been previously reported [ 11 ], such rare EBV-associated NK/T-cell tumors appear to be more likely to involve the orbit or ocular adnexa [ 12 ].…”

Primary Intraocular Lymphoma in a Patient with Bilateral Epstein-Barr Virus Panuveitis: A Case Report

“…Therefore, it is wondered that whether the retinitis in this patient was neoplastic. A similar case was reported by Imai et al 10 that was ultimately observed to have an intraocular natural killer (NK)/T-cell lymphoma. Although several cases of intraocular NK/T-cell lymphoma have been previously reported 11 , such rare EBVassociated NK/T-cell tumors appear to be more likely to involve the orbit or ocular adnexa 12 .…”

Successful Treatment of Primary CNS Extranodal NK/T-Cell Lymphoma with Surgery and Chemotherapy Combined with Sintilimab: A Case Report and Literature Review

“…than extracranial NK/TCL. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] PCNSLs are often accompanied by nonspecific clinical symptoms such as dizziness, headache, vomiting, and blurred vision. Our patient presented with loss of visual acuity and weakness of the limbs, which were consistent with the nonspecific symptoms of primary CNS lymphoma.…”

“…To our knowledge, only 23 cases of PCNS ENK/TCL have been reported in the English literature. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] To date, the understanding of this tumor is not deep enough. Herein, we present a case of PCNS ENK/TCL in a 50year-old immunocompetent Chinese female and review of the literature.…”