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- Title
Primary Central Nervous System ALK Positive Anaplastic Large Cell Lymphoma with Predominantly Leptomeningeal Involvement in an Adult.
- Authors
Jae Sung Park; Heejung Park; Sanghui Park; Suk Jin Kim; Ho Jun Seol; Young-Hyeh Ko
- Abstract
A 31-year-old Korean male presented with altered consciousness and severe headache. Brain MRI delineated focal leptomeningeal enhancement without any intracerebral 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 ALK+ALCL. 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.
- Subjects
CENTRAL nervous system; LYMPHOMAS; CANCER chemotherapy; MENINGEAL cancer; KOREANS; IMMUNOHISTOCHEMISTRY; METHOTREXATE; DISEASES
- Publication
Yonsei Medical Journal, 2013, Vol 54, Issue 3, p791
- ISSN
0513-5796
- Publication type
Article
- DOI
10.3349/ymj.2013.54.3.791