Primary and secondary central nervous system mature T- and NK-cell lymphomas

Pang, Yifan; Chihara, Dai

doi:10.1053/j.seminhematol.2021.02.003

Cited by 3 publications

(2 citation statements)

References 86 publications

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“…The majority are B-cell lymphomas, with DLBCL comprising over 90% [13,26,27]. T-cell lymphomas account for approximately 2-4% of all primary CNS lymphomas in the West [28,29] with a higher incidence in Asia, ranging from 2 to 17% [30][31][32]. Primary CNS T-cell lymphomas (PCNSTL) include peripheral T-cell lymphoma, not otherwise specified, the most common subtype, ALCL, and extranodal NK/T-cell lymphoma [5,26,32,33].…”

Section: Discussionmentioning

confidence: 99%

“…T-cell lymphomas account for approximately 2-4% of all primary CNS lymphomas in the West [28,29] with a higher incidence in Asia, ranging from 2 to 17% [30][31][32]. Primary CNS T-cell lymphomas (PCNSTL) include peripheral T-cell lymphoma, not otherwise specified, the most common subtype, ALCL, and extranodal NK/T-cell lymphoma [5,26,32,33]. In a series of 45 PCNSTLs from multiple institutions and countries, Shenkier et al reported a wide age range of presentation from 3 to 84 years with median age of 60 years and male predominance, similar to PCNSLs overall [27,34].…”

Section: Discussionmentioning

confidence: 99%

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Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement

et al. 2021

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Anaplastic large cell lymphomas (ALCL) are mature T-cell neoplasms, approximately half of which harbor rearrangements of the ALK gene that confer a good prognosis. Recent studies have demonstrated that a significant proportion of ALK-negative ALCLs demonstrate rearrangements of the IRF4/DUSP22 locus that also are typically associated with a favorable prognosis. ALCL with primary involvement of the central nervous system (CNS) is extremely rare. We report what may be the first case of ALK-negative ALCL with IRF4/DUSP22 rearrangement involving the brain in a 55-year-old man. Magnetic resonance imaging demonstrated signal abnormalities in the periventricular region, corpus callosum and cingulate gyrus. Biopsy revealed a diffuse parenchymal and angiocentric infiltrate of CD30-positive cells that showed IRF4/DUSP22 rearrangement by fluorescence in situ hybridization. We also review the clinical and pathologic features of primary CNS ALK-negative ALCLs in the literature and highlight the need for awareness of this entity to optimize appropriate management.

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