A case of primary extranodal natural killer/T-cell lymphoma in the orbit and intraocular tissues with cerebrospinal fluid involvement

Abstract

PurposeTo report a rare case of primary orbital natural killer (NK)/T-cell lymphoma without nasal lesions but with cerebrospinal fluid involvement.ObservationsA 73-year-old woman was referred to the uveitis clinic with suspected unilateral acute uveitis in her right eye and a right orbital tumor. Epstein–Barr virus DNA was detected in the aqueous humor in her right eye, and orbital biopsy revealed the presence of extranodal NK/T-cell lymphoma (ENKTL), nasal type. Positron emission tomography showed significant 18F-fluorodeoxyglucose uptake in the right orbit, with no other signs of systemic involvement. Cerebrospinal fluid analysis demonstrated lymphoma cell infiltration. She was diagnosed with stage IV ENKTL and treated with orbital radiotherapy and systemic chemotherapy, with subsequent remission. However, the lymphoma relapsed in her left vitreous at 10 months after therapy, suggesting metastasis of lymphoma cells to the contralateral eye via the vitreous and cerebrospinal fluid.Conclusions and importanceA few cases of ocular-tissue ENKTL have been reported, mostly involving invasion or dissemination of primary nasal lesions; in contrast, primary orbital and intraocular ENKTL has rarely been reported. To the best of our knowledge, this is the first report of a primary orbital ENKTL metastasizing to the vitreous of the contralateral eye. Although ENKTL is rare in the orbit and intraocular tissues, it should be considered as a possible differential diagnosis in patients with orbital tumors or intraocular inflammation resistant to steroid therapy because ENKTL has a very poor prognosis in the advanced stage.

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.…”

“…collected from the English literature and summarized. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] These patients were pathologically diagnosed as primary CNS ENK/TCL, excluding secondary and invasive NK cell leukemia. According to previously reported cases, overall survival in patients with PCNS ENK/TCL is not high.…”

“…The highest survival reported was 139 months to date, and the median overall survival was 6 months. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] There were nineteen immunocompetent (IC) cases and two immunosuppressive (IS) cases of which one was infected with HIV and the another was a posttransplant patient. Most of the patients were from Asia, Africa, and Spain, and the age of these patients was between 13 and 77 years (median, 53 years).…”

“…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.…”

Extranodal natural killer/T-cell lymphoma presenting as hypopyon panuveitis: a case report

“…Extranodal natural killer/T-cell lymphoma (ENKTL) is an extremely rare subtype of non-Hodgkin’s lymphoma (NHL), believed to be an Epstein–Barr virus-related neoplasm [ 1 – 3 ]. The incidence of NK/T-cell lymphoma (NKTL) is more common in Asian and Latin American populations [ 2 ].…”