Primary Leptomeningeal Lymphoma: Clinicopathologic Features of 2 Rare Phenotypes. Issue 12 (14th September 2022)

• Record Type: Journal Article
• Authors: Vizcaino, M Adelita; Giannini, Caterina; King, Rebecca L; Johnson, Derek R; Yang, Tong; Raghunathan, Aditya
• Is Part Of: Journal of neuropathology and experimental neurology. Volume 81:Issue 12(2022)
• Page Start: 1002
• Page End: 1007
• Publication Date: 2022-09-14
• Subjects: ALK-positive anaplastic large cell lymphoma -- Central nervous system lymphoma -- Immunodeficiency -- Immune-privileged sites -- Leptomeninges -- Primary effusion lymphoma; Neurology -- Diseases -- Periodicals; Neurology -- Diseases -- Physiopathology -- Periodicals; 616.8047
• DOI: 10.1093/jnen/nlac084
• Languages: English
• ISSNs: 0022-3069

Abstract

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.