Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement.
Title: | Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement. |
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Authors: | Magaki, Shino, Satyadev, Radha, Chen, Zesheng, Yung, Kathryn S., Vinters, Harry V., Kinney, Marsha C., Said, Jonathan W. |
Source: | Brain Tumor Pathology; Jan2022, Vol. 39 Issue 1, p25-34, 10p |
Abstract: | 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. [ABSTRACT FROM AUTHOR] |
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Database: | Complementary Index |
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