title: Primary central nervous system ALK positive anaplastic large cell lymphoma with predominantly leptomeningeal involvement in an adult.
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.
authors.affiliation: Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul 135-710, Korea.
authors.forename: Jae Sung
authors.initials: JS
authors.lastname: Park
authors.forename: Heejung
authors.initials: H
authors.lastname: Park
authors.forename: Sanghui
authors.initials: S
authors.lastname: Park
authors.forename: Suk Jin
authors.initials: SJ
authors.lastname: Kim
authors.forename: Ho Jun
authors.initials: HJ
authors.lastname: Seol
authors.forename: Young-Hyeh
authors.initials: YH
authors.lastname: Ko
mesh_terms: D000328:Adult; D000077548:Anaplastic Lymphoma Kinase; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001706:Biopsy; D001921:Brain / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D007150:Immunohistochemistry; D017728:Lymphoma, Large-Cell, Anaplastic / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008577:Meningeal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D020794:Receptor Protein-Tyrosine Kinases / Q000378:metabolism*
chemical_list: D000970:Antineoplastic Agents; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases
keywords: ALK-positive; CNS; anaplastic large-cell lymphoma; leptomeningeal; primary
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