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Primary T cell central nervous system lymphoblastic lymphoma in a child: case report and literature review

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Abstract

Purpose

Primary central nervous system lymphoma (PCNSL) of T cell origin is rare in pediatric patients. We report a case of T cell PCNSL in a 12-year-old boy and review the literature to highlight the importance of brain biopsy to definitively establish the diagnosis when PCNSL is suspected.

Case report

A 12-year-old boy presented with worsening left-sided weakness, nausea, vomiting, headache, blurred vision, and diplopia. Magnetic resonance imaging revealed right parietal gyral thickening with faint meningeal contrast enhancement. No clear diagnosis was identified after serum testing, cerebrospinal fluid analysis, and cerebral angiography. To establish the diagnosis definitively, a right craniotomy and open, frameless stereotactic biopsy were performed, which yielded the diagnosis of lymphoblastic T cell lymphoma.

Conclusions

PCNSL of T cell origin in children remains poorly studied, with only 18 detailed cases reported over the last three decades, including this case. Establishing a definitive diagnosis of PCNSL is challenging, and a brain biopsy is often required to obtain enough tissue for pathological analysis. Increasing awareness and identification of children diagnosed with T cell PCNSL is needed to better understand the molecular biology of this disease and develop more standardized treatment regimens.

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Acknowledgments

The authors thank Kristin Kraus, M.Sc., for her assistance in the preparation of this paper.

Conflict of interest

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Department of Neurosurgery, Primary Children’s Hospital, University of Utah, 100 North Mario Capecchi Drive, Suite 1475, Salt Lake City, UT, 84113-1100, USA

    Marcus D. Mazur, Vijay M. Ravindra & Robert J. Bollo

  2. Department of Pathology, University of Utah, Salt Lake City, UT, USA

    Mouied Alashari

  3. Division of Pediatric Hematology/Oncology, Primary Children’s Hospital, University of Utah, Salt Lake City, UT, USA

    Elizabeth Raetz

  4. Department of Radiation Oncology, University of Utah, Salt Lake City, UT, USA

    Matthew M. Poppe

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  1. Marcus D. Mazur
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Correspondence to Robert J. Bollo.

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Mazur, M.D., Ravindra, V.M., Alashari, M. et al. Primary T cell central nervous system lymphoblastic lymphoma in a child: case report and literature review. Childs Nerv Syst 31, 977–984 (2015). https://doi.org/10.1007/s00381-015-2633-6

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