Case report: diagnostic challenges of primary central nervous system anaplastic large cell lymphoma, ALK-positive
Weaver, Abigail E., Williamson, Timothy J., Iyengar, Krishnan R., Campbell, Robert A., and Stuart, Michael J. (2025) Case report: diagnostic challenges of primary central nervous system anaplastic large cell lymphoma, ALK-positive. Child's Nervous System, 41 (1). 241.
|
PDF (Published Version)
- Published Version
Available under License Creative Commons Attribution. Download (1MB) | Preview |
Abstract
Introduction: Primary central nervous system lymphoma in children is rare, and the highly variable radiological presentation presents a significant diagnostic challenge. The reported subtype: anaplastic large cell lymphoma, kinase positive (ALCL, ALK+) is an exceedingly rare entity, with the largest case series comprising only 34 cases. Involvement of the choroid plexus is particularly uncommon, with only one previously reported case arising in the lateral ventricle. This case report highlights the diagnostic and management challenges associated with this unusual presentation and lesion location. Case report: A 16-year-old male presented with a 3-week history of fever and vomiting followed by progressive neurological deterioration, including confusion and collapse. Brain computed tomography (CT) revealed significant vasogenic edema and a hyperdense lesion in the right lateral ventricle. Magnetic resonance imaging (MRI) demonstrated a lobulated contrast-enhancing choroid plexus lesion with entrapment of the temporal horn and midline shift. Biopsy confirmed the diagnosis of PCNS ALK + ALCL. Subsequent chemotherapy resulted in remission of the lesion by the time of last follow-up, and aggressive surgical resection was not required. The rarity of this lesion posed diagnostic difficulties clinically and radiologically. Conclusion: This case illustrates the challenges of diagnosing childhood PCNSL, particularly when the lesion arises in an atypical location such as the choroid plexus. The excellent response to adjuvant therapy highlights the importance of considering this diagnosis in young patients with atypical brain lesions in order to avoid unnecessarily aggressive and potentially morbid surgical approaches.
| Item ID: | 87694 |
|---|---|
| Item Type: | Article (Case Study) |
| ISSN: | 1433-0350 |
| Keywords: | Cerebrospinal fluid, Hydrocephalus, Pediatric, Ventricular, Ventriculoperitoneal |
| Copyright Information: | © The Author(s) 2025. This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. |
| Date Deposited: | 19 Jan 2026 06:23 |
| FoR Codes: | 32 BIOMEDICAL AND CLINICAL SCIENCES > 3209 Neurosciences > 320903 Central nervous system @ 100% |
| SEO Codes: | 20 HEALTH > 2001 Clinical health > 200105 Treatment of human diseases and conditions @ 100% |
| Downloads: |
Total: 1 Last 12 Months: 1 |
| More Statistics |
Actions (Repository Staff Only)
 |
Item Control Page |