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Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports

BACKGROUND: Primary central nervous system (CNS) small lymphocytic lymphoma (SLL), as a type of low-grade lymphoma, is extremely rare. The diagnosis of CNS SLL is challenging due to its variable clinical and radiological features, which may overlap with those of diffuse large B-cell lymphoma (DLBCL)...

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Autores principales: Guo, Rongjing, Zhang, Xiaolong, Niu, Chunxiao, Xi, Yibin, Yin, Hong, Lin, Hong, Chang, Ting
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6699128/
https://www.ncbi.nlm.nih.gov/pubmed/31426757
http://dx.doi.org/10.1186/s12883-019-1430-3
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author Guo, Rongjing
Zhang, Xiaolong
Niu, Chunxiao
Xi, Yibin
Yin, Hong
Lin, Hong
Chang, Ting
author_facet Guo, Rongjing
Zhang, Xiaolong
Niu, Chunxiao
Xi, Yibin
Yin, Hong
Lin, Hong
Chang, Ting
author_sort Guo, Rongjing
collection PubMed
description BACKGROUND: Primary central nervous system (CNS) small lymphocytic lymphoma (SLL), as a type of low-grade lymphoma, is extremely rare. The diagnosis of CNS SLL is challenging due to its variable clinical and radiological features, which may overlap with those of diffuse large B-cell lymphoma (DLBCL). Primary CNS SLL differs from DLBCL in that it has an indolent clinical course and a good prognosis. Thus, it is important to distinguish SLL from DLBCL. By reviewing the literature, only two cases of low-grade SLL, primarily located in the CNS and involving the brain parenchyma and dura, have been reported. To our knowledge, primary CNS SLL in the bilateral ventricles has never been reported. Interestingly, the two cases in our report are identical in terms of the clinical presentations, magnetic resonance imaging (MRI) features, pathological results and prognoses. CASE PRESENTATION: Both patients presented with headaches. MRI suggested solid lesions located in the bilateral ventricles that were isointense on T1-weighted images and hypointense on T2-weighted images. After the injection of contrast agent (gadolinium, Gd), the intraventricular lesions were homogeneously enhanced and hyperperfused. CSF cytology revealed malignant cells. Brain biopsy revealed diffuse proliferation of small lymphocytes with positive labelling of B-cell immunomarkers. The primary origin in the CNS was confirmed with no evidence of systemic lymphoma. Two patients were given high doses of methotrexate-based chemotherapy and were free from symptoms and progression for more than 1-year of follow-up. CONCLUSIONS: The presence of homogeneously enhanced intraventricular MRI lesions should raise the suspicion of primary CNS SLL.
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spelling pubmed-66991282019-08-26 Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports Guo, Rongjing Zhang, Xiaolong Niu, Chunxiao Xi, Yibin Yin, Hong Lin, Hong Chang, Ting BMC Neurol Case Report BACKGROUND: Primary central nervous system (CNS) small lymphocytic lymphoma (SLL), as a type of low-grade lymphoma, is extremely rare. The diagnosis of CNS SLL is challenging due to its variable clinical and radiological features, which may overlap with those of diffuse large B-cell lymphoma (DLBCL). Primary CNS SLL differs from DLBCL in that it has an indolent clinical course and a good prognosis. Thus, it is important to distinguish SLL from DLBCL. By reviewing the literature, only two cases of low-grade SLL, primarily located in the CNS and involving the brain parenchyma and dura, have been reported. To our knowledge, primary CNS SLL in the bilateral ventricles has never been reported. Interestingly, the two cases in our report are identical in terms of the clinical presentations, magnetic resonance imaging (MRI) features, pathological results and prognoses. CASE PRESENTATION: Both patients presented with headaches. MRI suggested solid lesions located in the bilateral ventricles that were isointense on T1-weighted images and hypointense on T2-weighted images. After the injection of contrast agent (gadolinium, Gd), the intraventricular lesions were homogeneously enhanced and hyperperfused. CSF cytology revealed malignant cells. Brain biopsy revealed diffuse proliferation of small lymphocytes with positive labelling of B-cell immunomarkers. The primary origin in the CNS was confirmed with no evidence of systemic lymphoma. Two patients were given high doses of methotrexate-based chemotherapy and were free from symptoms and progression for more than 1-year of follow-up. CONCLUSIONS: The presence of homogeneously enhanced intraventricular MRI lesions should raise the suspicion of primary CNS SLL. BioMed Central 2019-08-19 /pmc/articles/PMC6699128/ /pubmed/31426757 http://dx.doi.org/10.1186/s12883-019-1430-3 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Guo, Rongjing
Zhang, Xiaolong
Niu, Chunxiao
Xi, Yibin
Yin, Hong
Lin, Hong
Chang, Ting
Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports
title Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports
title_full Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports
title_fullStr Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports
title_full_unstemmed Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports
title_short Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports
title_sort primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6699128/
https://www.ncbi.nlm.nih.gov/pubmed/31426757
http://dx.doi.org/10.1186/s12883-019-1430-3
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