Primary anaplastic large cell lymphoma of the central nervous system: Prognostic effect of ALK-1 expression

David H. George, Bernd W. Scheithauer, Fügen V. Aker, Paul J. Kurtin, Peter C. Burger, José Cameselle-Teijeiro, Roger E. McLendon, Joseph E. Parisi, Werner Paulus, Wolfgang Roggendorf, Cirilo Sotelo

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63 Scopus citations


Anaplastic large cell lymphoma (ALCL) rarely occurs in the central nervous system. Although defined by its composition of large, pleomorphic, CD30-positive lymphocytes, ALCL is heterogeneous. Most are T cell but some are null cell. Most but not all have a characteristic 2:5 translocation producing the fusion protein ALK-1, which is reliably detected by immunohistochemistry. In systemic ALCL, ALK-1 expression correlates with young patient age and a favorable prognosis. Herein we report four new cases of primary central nervous system ALCL from the Mayo Clinic and incorporate additional data from five previously published cases. ALK-1 expression was determined in all nine tumors. Patient age was 4-66 years (mean 29 years) with a bimodal distribution: 6 ≤ 22 years, 3 ≥ 50 years. Six were female. Tumors were mostly supratentorial, five were multifocal, and seven had involvement of dura or leptomeninges. Seven tumors were T cell, two were null cell, and five of nine were ALK-1 immunopositive. Total mortality was six of nine. Three patients, 4-18 years of age (mean 13 years), were alive at 4.8-6.1 years postdiagnosis; these tumors were all ALK positive. Five patients, 13-66 years of age (mean 43 years), died of tumor 4 days to 11 weeks postdiagnosis; four of five of these tumors were ALK negative. One 10-year-old child with an ALK-positive tumor died of sepsis, but in remission. Central nervous system ALCL is aggressive. Our study suggests that a better outcome may be associated with young age and ALK-1 positivity, prognostic parameters similar to systemic ALCL.

Original languageEnglish (US)
Pages (from-to)487-493
Number of pages7
JournalAmerican Journal of Surgical Pathology
Issue number4
StatePublished - Apr 1 2003


  • ALK-1
  • Brain
  • Lymphoma
  • Prognosis

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine


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