Primary Central Nervous System Anaplastic Large Cell Lymphoma, ALK Positive

Area of Science:

• Neurology
• Oncology
• Pathology

Background:

• Primary central nervous system anaplastic large cell lymphoma, anaplastic lymphoma kinase positive (primary CNS ALCL, ALK+) is a rare CNS malignancy.
• Existing literature is primarily limited to individual case reports, hindering comprehensive understanding.

Purpose of the Study:

• To present the largest case series of primary CNS ALCL, ALK+ to date.
• To gather observational data on clinical course and prognostic factors.
• To provide additional diagnostic and clinical information for this rare lymphoma.

Main Methods:

• Retrospective search across multiple academic centers for primary CNS ALCL, ALK+ cases.
• Comprehensive review of published primary CNS ALCL, ALK+ cases.
• Analysis of clinical history, radiography, pathology, and genetic testing data.

Main Results:

• The combined series includes 34 cases (3 from the authors' centers, 31 from literature).
• Median age was 18.5 years, with a male predominance (4.7:1 ratio).
• Headache was the most common presenting symptom; ALK rearrangement was confirmed genetically.

Conclusions:

• This series represents the largest compilation of primary CNS ALCL, ALK+ cases.
• The findings contribute valuable diagnostic and clinical insights into this rare CNS lymphoma.
• Further research is warranted to fully elucidate the clinical course and optimize treatment strategies.