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Ventricle-predominant primary CNS lymphomas: clinical, radiological and pathological evaluation of five cases and review of the literature.

Abstract

Primary central nervous system lymphomas (PCNSLs) are typically intraparenchymal. A subset of PCNSLs predominantly arises in the ventricles, with minimal parenchymal involvement. We review the clinical, radiological, and pathological features of ventricle-predominant PCNSLs (VP-PCNSLs) in 40 previously reported cases and report 5 additional cases. Including all cases of VP-PCNSLs (n = 45), 38% were diffuse large B-cell lymphomas (DLBCL), 11% were Burkitt lymphomas, 7% were MALT lymphomas, 4% were T-cell lymphomas, and 40% were lymphomas, not otherwise classified. VP-PCNSLs show rapid clinical progression. Patients present at a median age of 60.5 years. Unique clinical and radiological features distinguish them from other intraventricular tumors, including advanced age, edema, multifocality, hyperdensity, early and avid post-contrast enhancement, restricted diffusion, and positron emission tomography (PET) hypermetabolism. Including our cases, which were all DLBCL, and all previously reported DLBCL cases (n = 10), 8 of 10 show germinal center B-cell-like (GCB) phenotype, contrasting the high prevalence of non-germinal center B-cell-like (non-GCB) phenotype of parenchymal DLBCL PCNSLs. MYD88 L265P mutation was detected in three of our five cases. Ventricle-predominant PCNSLs are clinically and radiologically distinct, and the DLBCLs may be pathologically distinct. Further recognition of this entity may help to evaluate the role of therapies, possibly including surgical resection.

Authors+Show Affiliations

Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA. ball.matthew@mayo.edu.Department of Radiology, Mayo Clinic, Rochester, MN, USA.Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA.Department of Neurosurgery, Mayo Clinic, Rochester, MN, USA.Department of Neurosurgery, Mayo Clinic, Rochester, MN, USA.Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31630277

Citation

Ball, Matthew K., et al. "Ventricle-predominant Primary CNS Lymphomas: Clinical, Radiological and Pathological Evaluation of Five Cases and Review of the Literature." Brain Tumor Pathology, 2019.
Ball MK, Morris JM, Wood AJ, et al. Ventricle-predominant primary CNS lymphomas: clinical, radiological and pathological evaluation of five cases and review of the literature. Brain Tumor Pathol. 2019.
Ball, M. K., Morris, J. M., Wood, A. J., Meyer, F. B., Kaszuba, M. C., & Raghunathan, A. (2019). Ventricle-predominant primary CNS lymphomas: clinical, radiological and pathological evaluation of five cases and review of the literature. Brain Tumor Pathology, doi:10.1007/s10014-019-00354-x.
Ball MK, et al. Ventricle-predominant Primary CNS Lymphomas: Clinical, Radiological and Pathological Evaluation of Five Cases and Review of the Literature. Brain Tumor Pathol. 2019 Oct 19; PubMed PMID: 31630277.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ventricle-predominant primary CNS lymphomas: clinical, radiological and pathological evaluation of five cases and review of the literature. AU - Ball,Matthew K, AU - Morris,Jonathan M, AU - Wood,Adam J, AU - Meyer,Fredric B, AU - Kaszuba,Megan C, AU - Raghunathan,Aditya, Y1 - 2019/10/19/ PY - 2019/09/16/received PY - 2019/10/04/accepted PY - 2019/10/21/entrez PY - 2019/10/21/pubmed PY - 2019/10/21/medline KW - B-cell lymphoma KW - Intraventricular tumor KW - Primary CNS lymphoma KW - Ventricle JF - Brain tumor pathology JO - Brain Tumor Pathol N2 - Primary central nervous system lymphomas (PCNSLs) are typically intraparenchymal. A subset of PCNSLs predominantly arises in the ventricles, with minimal parenchymal involvement. We review the clinical, radiological, and pathological features of ventricle-predominant PCNSLs (VP-PCNSLs) in 40 previously reported cases and report 5 additional cases. Including all cases of VP-PCNSLs (n = 45), 38% were diffuse large B-cell lymphomas (DLBCL), 11% were Burkitt lymphomas, 7% were MALT lymphomas, 4% were T-cell lymphomas, and 40% were lymphomas, not otherwise classified. VP-PCNSLs show rapid clinical progression. Patients present at a median age of 60.5 years. Unique clinical and radiological features distinguish them from other intraventricular tumors, including advanced age, edema, multifocality, hyperdensity, early and avid post-contrast enhancement, restricted diffusion, and positron emission tomography (PET) hypermetabolism. Including our cases, which were all DLBCL, and all previously reported DLBCL cases (n = 10), 8 of 10 show germinal center B-cell-like (GCB) phenotype, contrasting the high prevalence of non-germinal center B-cell-like (non-GCB) phenotype of parenchymal DLBCL PCNSLs. MYD88 L265P mutation was detected in three of our five cases. Ventricle-predominant PCNSLs are clinically and radiologically distinct, and the DLBCLs may be pathologically distinct. Further recognition of this entity may help to evaluate the role of therapies, possibly including surgical resection. SN - 1861-387X UR - https://www.unboundmedicine.com/medline/citation/31630277/Ventricle-predominant_primary_CNS_lymphomas:_clinical,_radiological_and_pathological_evaluation_of_five_cases_and_review_of_the_literature L2 - https://dx.doi.org/10.1007/s10014-019-00354-x DB - PRIME DP - Unbound Medicine ER -