CD20-positive extranodal NK/T cell lymphoma: clinicopathologic and prognostic features

• Published in: Virchows Archiv : an international journal of pathology 2020-12, Vol.477 (6), p.873-883
• Main Authors: Huang, Yuhua, Chen, Shilu, Wei, Rong, Guo, Xinjian, Yang, Xia, Cao, Qinghua, Yang, Yu, Yun, Jingping
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Antigens, CD20 - immunology; Antigens, CD20 - metabolism; Butterflies & moths; CD19 antigen; CD20 antigen; Female; Humans; Literature reviews; Lymphocytes; Lymphocytes B; Lymphocytes T; Lymphoma; Lymphoma, Extranodal NK-T-Cell - immunology; Lymphoma, Extranodal NK-T-Cell - pathology; Male; Medicine; Medicine & Public Health; Middle Aged; Mutation; Natural killer cells; Next-generation sequencing; Nose; Nucleotides; Original Article; Pathology; Pax5 protein; Prognosis; T-cell lymphoma; Tumor cells; Tumors
• ISSN: 0945-6317; 1432-2307
• DOI: 10.1007/s00428-020-02776-x

Extranodal natural killer (NK)/T cell lymphoma (ENKTCL) with aberrant CD20 expression is extremely rare. Here, we describe the clinicopathologic features of 11 CD20-positive ENKTCLs from three institutions in China along with a literature review. Membranous expression of CD20 was identified in 1.29% (11/851) of ENKTCLs. CD20-positive ENKTCLs primarily occurred in extra-nasal sites (72.2%, 13/18) rather than in the nasal cavity (27.8%, 5/18). Most evaluated patients (71.4%, 10/14) presented ENKTCL at advanced stage IV. The percentage of CD20-positive tumor cells ranged from 20 to 90%, and the CD20 staining intensity was dimmer in tumor cells than in normal B cells. Among four cases with multiple biopsies, three cases showed discordant expression of CD20 between the disseminated and primary lesions. All evaluated cases were negative for other B cell markers, including PAX5, CD79a, and CD19, except for one case that showed focally positive for CD79a. Patients with CD20-positive ENKTCL more frequently had advanced diseases (stage III/IV: 70% vs 17%, p  = 0.001), with older age (median age at diagnosis: 60 years vs. 43.5 years, p  = 0.006) and had inferior outcome (median survival: 18.7 moths vs 36.0 moths, p  = 0.017) compared with CD20-negative cases. Four nonsynonymous single nucleotide variants (C > T) and one stop-gain mutation (C > T) in the exonic region of CD20 gene (MS4A1) were detected in one of seven cases with target region next-generation sequencing. Thus, ENKTCL with aberrant CD20 expression is rare, tends to occur in older patients, and is characterized by a highly aggressive clinical course and poor outcomes. The mechanism underlying the expression of CD20 in ENKTC still remains unknown.