1.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
Yonsei Medical Journal 2013;54(3):791-796
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.
Adult
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Antineoplastic Agents/therapeutic use
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Biopsy
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Brain/metabolism/pathology
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Diagnosis, Differential
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Humans
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Immunohistochemistry
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Lymphoma, Large-Cell, Anaplastic/*diagnosis/drug therapy/pathology
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Male
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Meningeal Neoplasms/*diagnosis/drug therapy/pathology
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Receptor Protein-Tyrosine Kinases/*metabolism