{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T05:13:31Z","timestamp":1776230011766,"version":"3.50.1"},"reference-count":15,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T00:00:00Z","timestamp":1776211200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, highly aggressive hematologic malignancy characterized by frequent involvement of the skin and bone marrow. Central nervous system (CNS) infiltration is uncommon and may be clinically occult, thereby posing diagnostic challenges. We report the case of a 74 year-old man who initially presented with progressively enlarging cutaneous masses on the trunk. Histopathological examination of skin biopsy demonstrated BPDCN with a characteristic immunophenotype, and subsequent bone marrow studies confirmed extensive marrow involvement. The patient was treated with a combination of azacitidine and low-dose cytarabine to achieve sustained hematologic remission with marked regression of skin lesions and splenomegaly, despite intermittent treatment-related myelosuppression. After several months of stable disease, he was readmitted with new-onset paroxysmal occipital headache and blurred vision. Peripheral blood counts, bone marrow evaluation, and systemic imaging were unremarkable, and brain and orbital magnetic resonance imaging showed no abnormalities. Cerebrospinal fluid (CSF) analysis revealed marked pleocytosis with abundant atypical cells. Flow cytometry identified BPDCN cells accounting for 97.6% of nucleated cells, establishing the diagnosis of CNS involvement. Intrathecal methotrexate combined with cytarabine led to the rapid resolution of neurologic and visual symptoms. This case study demonstrates that BPDCN may lead to isolated CNS involvement despite ongoing systemic remission, highlighting the importance of cerebrospinal fluid evaluation in patients with unexplained neurologic manifestations.<\/jats:p>","DOI":"10.3389\/fonc.2026.1818268","type":"journal-article","created":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T04:39:37Z","timestamp":1776227977000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Isolated central nervous system involvement in blastic plasmacytoid dendritic cell neoplasm: a rare case of relapse during systemic remission with diagnostic and therapeutic implications"],"prefix":"10.3389","volume":"16","author":[{"given":"Haocheng","family":"Zhao","sequence":"first","affiliation":[{"name":"Department of Hematology, Yueqing People\u2019s Hospital","place":["Yueqing, Zhejiang, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wenye","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yueqing People\u2019s Hospital","place":["Yueqing, Zhejiang, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shanshan","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Yueqing People\u2019s Hospital","place":["Yueqing, Zhejiang, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yeping","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yueqing People\u2019s Hospital","place":["Yueqing, Zhejiang, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,4,15]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019001144","article-title":"Multicenter analysis of outcomes in blastic plasmacytoid dendritic cell neoplasm offers a pre-targeted therapy benchmark","volume":"134","author":"Taylor","year":"2019","journal-title":"Blood"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2024024262","article-title":"How I treat blastic plasmacytoid dendritic cell neoplasm","volume":"145","author":"Pemmaraju","year":"2025","journal-title":"Blood"},{"key":"B3","doi-asserted-by":"publisher","first-page":"6106","DOI":"10.1182\/blood-2024-206941","article-title":"Blastic plasmacytoid dendritic cell neoplasm are not characterized by two distinct subgroups, but by a transcriptomic and phenotypic gradient","volume":"144","author":"Renosi","year":"2024","journal-title":"Blood"},{"key":"B4","doi-asserted-by":"publisher","first-page":"1664","DOI":"10.1182\/blood-2016-06-723536","article-title":"Blastic plasmacytoid dendritic cell neoplasm associated with chronic myelomonocytic leukemia","volume":"128","author":"Hu","year":"2016","journal-title":"Blood"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccell.2016.10.002","article-title":"A druggable TCF4- and BRD4-dependent transcriptional network sustains Malignancy in blastic plasmacytoid dendritic cell neoplasm","volume":"30","author":"Ceribelli","year":"2016","journal-title":"Cancer Cell"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2021011817","article-title":"Central nervous system involvement in blastic plasmacytoid dendritic cell neoplasm","volume":"138","author":"Pemmaraju","year":"2021","journal-title":"Blood"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1255","DOI":"10.1182\/blood.2021014657","article-title":"Isolated CNS relapse of blastic plasmacytoid dendritic cell neoplasm","volume":"139","author":"Hilal","year":"2022","journal-title":"Blood"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2025-6914","article-title":"Plasmacytoid dendritic cell in a setting of acute myeloid leukaemia: a single-center case series from a tertiary cancer hospital","volume":"146","author":"Fareed","year":"2025","journal-title":"Blood"},{"key":"B9","doi-asserted-by":"publisher","first-page":"547","DOI":"10.1182\/blood-2023-178734","article-title":"Durable outcomes with manageable safety leading to prolonged survival with tagraxofusp for treatment-naive patients with blastic plasmacytoid dendritic cell neoplasm: updated results from a European named patient program","volume":"142","author":"Angelucci","year":"2023","journal-title":"Blood"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-09-726653","article-title":"Can reduced-intensity conditioning regimen cure blastic plasmacytoid dendritic cell neoplasm","volume":"129","author":"Leclerc","year":"2017","journal-title":"Blood"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2025-6974","article-title":"Single-center retrospective evaluation of CHOP-based regimens in older patients with treatment-naive blastic plasmacytoid dendritic cell neoplasm","volume":"146","author":"Fealko","year":"2025","journal-title":"Blood"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-RW2016-226","article-title":"BET inhibitors target blastic plasmacytoid dendritic cell neoplasms","volume":"7","year":"2017","journal-title":"Cancer Discov"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2025-4605","article-title":"Real-world study of treatment landscape and safety outcomes of US patients with blastic plasmacytoid dendritic cell neoplasm","volume":"146","author":"Pemmaraju","year":"2025","journal-title":"Blood"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01538-9","article-title":"Bone marrow clonal hematopoiesis is highly prevalent in blastic plasmacytoid dendritic cell neoplasm and frequently sharing a clonal origin in elderly patients","volume":"36","author":"Khanlari","year":"2022","journal-title":"Leukemia"},{"key":"B15","doi-asserted-by":"publisher","first-page":"1543","DOI":"10.1182\/blood-2023-188546","article-title":"Blastic plasmacytoid dendritic cell neoplasm (BPDCN): multi-center collaboration and global registry program","volume":"142","author":"Voskanyan","year":"2023","journal-title":"Blood"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1818268\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T04:39:50Z","timestamp":1776227990000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1818268\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,15]]},"references-count":15,"alternative-id":["10.3389\/fonc.2026.1818268"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1818268","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,15]]},"article-number":"1818268"}}