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Primary Leptomeningeal Lymphoma: Clinicopathologic Features of 2 Rare Phenotypes

Vizcaino, M Adelita ; Giannini, Caterina ; King, Rebecca L ; Johnson, Derek R ; Yang, Tong ; Raghunathan, Aditya

Journal of neuropathology and experimental neurology, 2022-12, Vol.81 (12), p.1002-1007 [Periódico revisado por pares]

England: Oxford University Press

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Título:
Primary Leptomeningeal Lymphoma: Clinicopathologic Features of 2 Rare Phenotypes
Autor: Vizcaino, M Adelita ; Giannini, Caterina ; King, Rebecca L ; Johnson, Derek R ; Yang, Tong ; Raghunathan, Aditya
Assuntos: Adult ; Anaplastic Lymphoma Kinase - genetics ; Biopsy ; Case studies ; Diagnosis ; Humans ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic - diagnosis ; Lymphoma, Large-Cell, Anaplastic - genetics ; Lymphoma, Large-Cell, Anaplastic - pathology ; Male ; Meningeal Neoplasms - diagnostic imaging ; Meningeal Neoplasms - pathology ; Meningitis ; Middle Aged ; Neoplasm Recurrence, Local ; Nervous system cancer ; Phenotype ; Receptor Protein-Tyrosine Kinases - genetics ; Receptor Protein-Tyrosine Kinases - therapeutic use ; Remission (Medicine) ; T cell lymphoma ; Young Adult
É parte de: Journal of neuropathology and experimental neurology, 2022-12, Vol.81 (12), p.1002-1007
Notas: ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
content type line 23
ObjectType-Report-1
ObjectType-Article-3
Descrição: Abstract Primary leptomeningeal lymphoma is exceedingly rare. We describe 2 rare lymphoma cases with exclusive leptomeningeal disease: 1 ALK-positive (ALK+) anaplastic large cell lymphoma (ALCL) and 1 primary effusion lymphoma (PEL). Case 1: A 19-year-old man presented with symptoms concerning for leptomeningitis. Cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis. Spine MRI demonstrated pial enhancement from T10 through the conus medullaris and cauda equina enhancement/thickening. A biopsy showed leptomeningeal involvement by large lymphoma cells with hallmark cells and brisk mitotic activity. By immunohistochemistry, cells were CD7/CD30-positive with cytoplasmic ALK staining. No systemic disease was identified. The diagnosis of primary leptomeningeal ALK+ ALCL was made. Despite 2 CSF relapses requiring systemic therapy and autologous bone marrow transplant, the patient was in complete clinical remission 9 years after the diagnosis. Case 2: A 60-year-old, human immunodeficiency virus-positive man presented with symptoms suggestive of leptomeningitis. Brain MRIs revealed multifocal, supratentorial, and infratentorial leptomeningeal enhancement. A right frontal biopsy demonstrated leptomeningeal involvement by large lymphoma cells negative for B-cell immunostains, but CD138, MUM-1, and HHV8-positive, with aberrant CD3 expression. EBV-encoded RNA in situ hybridization was positive. In absence of solid lesions/extracranial involvement, the diagnosis of leptomeningeal PEL was rendered. Despite initial complete remission after chemotherapy, the patient died 9 months later.
Editor: England: Oxford University Press
Idioma: Inglês

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Primary Leptomeningeal Lymphoma: Clinicopathologic Features of 2 Rare Phenotypes

Vizcaino, M Adelita ; Giannini, Caterina ; King, Rebecca L ; Johnson, Derek R ; Yang, Tong ; Raghunathan, Aditya

England: Oxford University Press

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