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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)...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
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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. |
format | Online Article Text |
id | pubmed-6699128 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
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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