Primary central nervous system ALK positive anaplastic large cell lymphoma with predominantly leptomeningeal involvement in an adult

Jae Sung Park, Heejung Park, Sanghui Park, Suk Jin Kim, Ho Jun Seol, Young Hyeh Ko

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11 Scopus citations

Abstract

A 31-year-old Korean male presented with altered consciousness and severe headache. Brain MRI delineated focal leptomeningeal enhancement without any intrace-rebral lesions. Diagnosis was made based on a brain biopsy showing anaplastic large cell lymphoma (ALCL), immunohistochemical stains revealing positivity for anaplastic lymphoma kinase (ALK) and an absence of involvement in any other organs; specifically, the primary central nervous system ALKALCL. Complete remission was achieved following 5 cycles of systemic chemotherapy with a high dose of Methotrexate and a simultaneous 7 cycles of intrathecal triple chemotherapy. Diagnosis of primary leptomeningeal ALK+ALCL is challenging given its rarity and non-specific symptoms along with non-pathognomonic radiologic findings. We present the first case of primary leptomeningeal ALK-positive ALCL where the clinical course, pathologic characteristics and treatment modality are described as well as a review of literature.

Original languageEnglish
Pages (from-to)791-796
Number of pages6
JournalYonsei Medical Journal
Volume54
Issue number3
DOIs
StatePublished - May 2013

Keywords

  • ALK-positive
  • Anaplastic large-cell lymphoma
  • CNS
  • Lepto-meningeal
  • Primary

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