Primary central nervous system extranodal nasal‐type natural killer/T‐cell lymphoma with CD20 expression

Abstract

We report a unique case of primary CNS extranodal natural killer/T-cell lymphoma (PCNS ENKTCL) with CD20 expression and the monoclonal rearrangement of Ig heavey chain (IgH) gene. Resection specimens were evaluated using HE-stained sections, immunohistochemistry, in situ hybridization and PCR. Histopathologic examination, immunohistochemistry and molecular studies showed the intermediate-sized lymphoma cells expressing CD2, CD3ε, granzyme B, TIA-1, CD20 and Epstein-Barr virus-encoded RNA, with germline T-cell receptor gene and the monoclonal rearrangement of IgH gene. Clinicopathologic and radiographic evaluation indicated the lesion was of exclusive left cerebellar localization. Thus, PCNS ENKTCL with the CD20 expression was diagnosed. ENKTCL with both CD20-positive expression and the monoclonal rearrangement of IgH gene may be mistaken for B-cell lymphoma; thus, the comprehensive evaluation of histomorphology, more extensive immunoprofiles and molecular studies is essential to reach the correct diagnosis. The rare PCNS ENKTCL shows a highly aggressive nature and a poorer prognosis.

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.…”

An Unusual Case of EBV-Negative Primary CNS Lymphoma of Natural Killer/T-Cell Lineage

“…The diagnoses were established either on biopsy or by the detection of malignant cells in the cerebrospinal fluid. 12 - 25 …”