{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T19:26:40Z","timestamp":1769974000042,"version":"3.49.0"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2022,9,14]],"date-time":"2022-09-14T00:00:00Z","timestamp":1663113600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,11,16]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>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\u2009years 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\u2009months later.<\/jats:p>","DOI":"10.1093\/jnen\/nlac084","type":"journal-article","created":{"date-parts":[[2022,9,14]],"date-time":"2022-09-14T15:28:47Z","timestamp":1663169327000},"page":"1002-1007","source":"Crossref","is-referenced-by-count":4,"title":["Primary Leptomeningeal Lymphoma: Clinicopathologic Features of 2 Rare Phenotypes"],"prefix":"10.1093","volume":"81","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4636-8743","authenticated-orcid":false,"given":"M Adelita","family":"Vizcaino","sequence":"first","affiliation":[{"name":"From the Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2757-6782","authenticated-orcid":false,"given":"Caterina","family":"Giannini","sequence":"additional","affiliation":[{"name":"From the Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rebecca L","family":"King","sequence":"additional","affiliation":[{"name":"From the Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Derek R","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Radiology, Mayo Clinic , Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tong","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Ochsner Medical Center , New Orleans, Louisiana, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6198-7441","authenticated-orcid":false,"given":"Aditya","family":"Raghunathan","sequence":"additional","affiliation":[{"name":"From the Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,9,14]]},"reference":[{"key":"2024032523482110700_nlac084-B1","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1212\/WNL.41.1.95","article-title":"Primary leptomeningeal lymphoma: Report of 9 cases, diagnosis with immunocytochemical analysis, and review of the literature","volume":"41","author":"Lachance","year":"1991","journal-title":"Neurology"},{"key":"2024032523482110700_nlac084-B2","doi-asserted-by":"crossref","first-page":"1690","DOI":"10.1212\/01.wnl.0000435302.02895.f3","article-title":"Primary leptomeningeal lymphoma: International Primary CNS Lymphoma Collaborative Group report","volume":"81","author":"Taylor","year":"2013","journal-title":"Neurology"},{"key":"2024032523482110700_nlac084-B3","doi-asserted-by":"crossref","first-page":"e236491","DOI":"10.1136\/bcr-2020-236491","article-title":"Primary leptomeningeal T-cell 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