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

Shino Magaki, Radha Satyadev, Zesheng Chen, Kathryn S. Yung, Harry V. Vinters, Marsha C. Kinney, Jonathan W. Said

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)25-34
Number of pages10
JournalBrain Tumor Pathology
Issue number1
StatePublished - Jan 2022


  • ALK
  • Anaplastic large cell lymphoma
  • Central nervous system
  • IRF4/DUSP22

ASJC Scopus subject areas

  • Clinical Neurology
  • Oncology
  • Cancer Research


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