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Representative images of case 3 (PTCL-NOS) (A-F) and case 8 (PTCL-NOS) (G-L). A, Brain magnetic resonance imaging of case 3 showed multiple enhancing lesions in both cerebral hemispheres (A) and the cerebellum (not shown). Brain parenchyme was diffusely infiltrated by small-to-medium-sized atypical lymphoid cells (B), which expressed CD3 (C), TCRβF1 (D), and CD4 (E). Scattered suspected reactive cells were positive for CD8 (F). G, Brain magnetic resonance imaging of case 8 showed ill-defined T2 high SI lesions in both basal ganglia, the internal capsule, and adjacent white matter. Small-sized lymphoid cells infiltrated brain parenchyme along with perivascular cuffing (H, I). Infiltrating cells were positive for CD3 (J, K) and negative for CD20 (L). PTCL-NOS indicates peripheral T-cell lymphoma, not otherwise specified.
Source publication
Primary central nervous system lymphoma (PCNSL) of peripheral T-cell lineage (T-PCNSL) is rare, and its genetic and clinicopathologic features remain unclear. Here, we present 11 cases of T-PCNSL in immunocompetent individuals from a single institute, focusing on their genetic alterations. Seven cases were subject to targeted panel sequencing cover...
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Context 1
... Robin spaces (Figs. 1B, C). The lymphoid cells had nuclear atypia with irregular or angulated nuclear contour and coarse chromatin and scanty clear cytoplasm (Figs. 1B, C). In microscopic examination of case 3 (PTCL-NOS), the biopsied tissue was hypercellular and densely infiltrated by small-to-medium-sized atypical lymphoid cells and histiocytes (Fig. 2B). Perivascular lymphocytic cuffing was frequently observed and the lymphoid cells showed nuclear atypia with irregular or angulated nuclear contour and scanty clear cytoplasm. In case 9 (PTCL of γδT-cell origin), removed spinal tumor was hypercellular and composed of diffuse and dense infiltration of atypical monomorphic lymphoid cells ...
Citations
Primary central nervous system (PCNS) extranodal NK/T‐cell lymphoma, nasal type (ENKTCL), is an exceedingly rare tumor. To the best of our knowledge, only 27 cases and only one reported aberrant CD20 expression have been documented in the literature. Here we present a second case of PCNS ENKTCL with aberrant CD20 expression in a 43‐year‐old immunocompetent Chinese female. The patient presented with tremors, weakness in the right upper limb, and a slow reaction. Magnetic resonance imaging revealed multiple brain lesions. A histological examination revealed a diffuse distribution of intermediate‐sized pleomorphic lymphocytes with angiocentric growth. The tumor cells expressed CD2, CD3, CD56, T‐cell intracellular antigen‐1, granzyme B, and Epstein–Barr virus‐encoded RNAs (EBERs), with additional partial and weak CD20 and CD30 expression. Despite a confirmatory pathological diagnosis, the patient refused treatment and was discharged, ultimately dying from the disease. In the literature review, the clinical, immunohistochemical, EBERs, treatment, and prognostic features of PCNS ENKTCL were summarized. Although PCNS ENKTCT is extremely rare, it does occur and should always be included in differential diagnoses. CD20 expression should be evaluated routinely with relevant markers. The accumulation of cases is crucial for developing an effective treatment strategy for this rare and aggressive malignancy.
Primary central nervous system lymphoma (PCNSL) is a rare extranodal non-Hodgkin lymphoma (NHL) with poor prognosis. In recent years, the emergence of genetic subtypes of systematic DLBCL has highlighted the importance of molecular genetics, but large-scale research on the molecular genetics of PCNSL is lacking. Herein, we summarize the frequent gene mutations and discuss the possible pathogenesis of PCNSL. MYD88 and CD79B mutations, which cause abnormal activation of NF-κB, are prominent genetic abnormalities in PCNSL. They are considered to play a major role in the pathogenesis of PCNSL. Other genes, such as CARD11, TNFAIP3, TBL1XR1, CDKN2A, PRDM1 and PIM1, are also frequently mutated. Notably, the pathogenesis of immune insufficiency-associated PCNSL is related to EBV infection, and its progression may be affected by different signaling pathways. The different mutational patterns in different studies highlight the heterogeneity of PCNSL. However, existing research on the molecular genetics of PCNSL is still limited, and further research into PCNSL is required to clarify the genetic characteristics of PCNSL.
