Primary Central Nervous System Anaplastic Large Cell Lymphoma, ALK Positive

Jared T. Ahrendsen, Robert Ta, Jingwei Li, Olga K. Weinberg, Judith A. Ferry, Robert P. Hasserjian, David M. Meredith, Hemant Varma, Sam Sadigh, Phillip D. Michaels

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations


OBJECTIVES: Primary central nervous system anaplastic large cell lymphoma, anaplastic lymphoma kinase positive (primary CNS ALCL, ALK+) is a rare CNS lymphoma whose description is limited to case reports. These tumors have a variable clinical course, and prognosis is primarily determined by age. We present the largest case series to date of primary CNS ALCL, ALK+, with observational data. METHODS: A retrospective search of multiple academic centers was performed to identify cases of primary CNS ALCL, ALK+. We also performed a review of published cases of primary CNS ALCL, ALK+. Clinical history, radiography, pathology, and genetic testing data were obtained to determine the prognostic implications in the context of clinical course. RESULTS: We identified three cases of primary CNS ALCL, ALK+ from our databases. A literature review identified 30 published reports of 31 individual cases. Clinical features for the combined 34 cases included a median age of 18.5 years, with a male to female ratio of 4.7:1, and the most common symptom was headache. Genetic studies demonstrated an ALK rearrangement by fluorescence in situ hybridization, and a gene fusion assay confirmed an NPM1-ALK gene fusion in one case. CONCLUSIONS: We present the largest case series to date of a rare primary CNS lymphoma with additional diagnostic and clinical information.

Original languageEnglish (US)
Pages (from-to)300-310
Number of pages11
JournalAmerican journal of clinical pathology
Issue number2
StatePublished - Aug 4 2022


  • ALK
  • Anaplastic large cell lymphoma
  • CNS lymphoma
  • NPM1-ALK

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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