Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature
Primary large B-cell lymphoma of immune-privileged sites (IP-LBCL) is a rare malignant hematological neoplasm. Involvement of the cerebellum is even rarer and its diagnosis is often difficult to make due to its non-specific clinical and radiological presentation. Methods: We reported 3 cases of cere...
| Main Authors: | , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2023-01-01
|
| Series: | Life |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-1729/13/1/201 |
| _version_ | 1797439629408862208 |
|---|---|
| author | Saverio Pancetti Daoud Rahal Bethania Fernades Carlo Galli Silvia Uccella Luigi Maria Terracciano Federico Pessina Lorenzo Bello Arturo Bonometti |
| author_facet | Saverio Pancetti Daoud Rahal Bethania Fernades Carlo Galli Silvia Uccella Luigi Maria Terracciano Federico Pessina Lorenzo Bello Arturo Bonometti |
| author_sort | Saverio Pancetti |
| collection | DOAJ |
| description | Primary large B-cell lymphoma of immune-privileged sites (IP-LBCL) is a rare malignant hematological neoplasm. Involvement of the cerebellum is even rarer and its diagnosis is often difficult to make due to its non-specific clinical and radiological presentation. Methods: We reported 3 cases of cerebellar IP-LBCL followed at our hospital and reviewed the medical literature to unravel the peculiarities of this poorly studied entity. Outcomes: Analyzing our cases and reviewing the literature, we could collect and study 26 cases of cerebellar IP-LBCL. To the best of our knowledge, this is the largest cohort of such patients currently published. Conclusion: Cerebellar IP-LBCL presents more often in adult females with cerebellum-related focal neurological signs such as ataxia, headache, and nausea. Histological confirmation is mandatory for a correct diagnosis and treatment and all cases feature diffuse large B-cell lymphoma histopathology. Compared to other encephalic IP-LBCL, cerebellar cases seem to include a higher number of cases with germinal center B-cell phenotype and better survival. These differences may be related to a different immune microenvironment and especially immunoregulation that distinguishes the cerebellum from other areas of the CNS. |
| first_indexed | 2024-03-09T11:55:52Z |
| format | Article |
| id | doaj.art-b7d30a4c83164ddd9bd45e91595e34ff |
| institution | Directory Open Access Journal |
| issn | 2075-1729 |
| language | English |
| last_indexed | 2024-03-09T11:55:52Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj.art-b7d30a4c83164ddd9bd45e91595e34ff2023-11-30T23:09:23ZengMDPI AGLife2075-17292023-01-0113120110.3390/life13010201Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the LiteratureSaverio Pancetti0Daoud Rahal1Bethania Fernades2Carlo Galli3Silvia Uccella4Luigi Maria Terracciano5Federico Pessina6Lorenzo Bello7Arturo Bonometti8Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, ItalyDepartment of Pathology Unit, Humanitas Clinical and Research Hospital, Via Manzoni 56, Rozzano, 20089 Milan, ItalyDepartment of Pathology Unit, Humanitas Clinical and Research Hospital, Via Manzoni 56, Rozzano, 20089 Milan, ItalyDepartment of Pathology Unit, Humanitas Clinical and Research Hospital, Via Manzoni 56, Rozzano, 20089 Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, ItalyNeurosurgical Oncology Unit, Department of Oncology and Hemato-Oncology, IRCCS Galeazzi-Sant’Ambrogio, Università degli Studi di Milano, 20161 Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, ItalyPrimary large B-cell lymphoma of immune-privileged sites (IP-LBCL) is a rare malignant hematological neoplasm. Involvement of the cerebellum is even rarer and its diagnosis is often difficult to make due to its non-specific clinical and radiological presentation. Methods: We reported 3 cases of cerebellar IP-LBCL followed at our hospital and reviewed the medical literature to unravel the peculiarities of this poorly studied entity. Outcomes: Analyzing our cases and reviewing the literature, we could collect and study 26 cases of cerebellar IP-LBCL. To the best of our knowledge, this is the largest cohort of such patients currently published. Conclusion: Cerebellar IP-LBCL presents more often in adult females with cerebellum-related focal neurological signs such as ataxia, headache, and nausea. Histological confirmation is mandatory for a correct diagnosis and treatment and all cases feature diffuse large B-cell lymphoma histopathology. Compared to other encephalic IP-LBCL, cerebellar cases seem to include a higher number of cases with germinal center B-cell phenotype and better survival. These differences may be related to a different immune microenvironment and especially immunoregulation that distinguishes the cerebellum from other areas of the CNS.https://www.mdpi.com/2075-1729/13/1/201diffuse large B-cell lymphomacentral nervous systemcerebellartherapyimmune-privileged sites |
| spellingShingle | Saverio Pancetti Daoud Rahal Bethania Fernades Carlo Galli Silvia Uccella Luigi Maria Terracciano Federico Pessina Lorenzo Bello Arturo Bonometti Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature Life diffuse large B-cell lymphoma central nervous system cerebellar therapy immune-privileged sites |
| title | Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature |
| title_full | Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature |
| title_fullStr | Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature |
| title_full_unstemmed | Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature |
| title_short | Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature |
| title_sort | primary large b cell lymphoma of immune privileged sites of the cerebellum a case series and review of the literature |
| topic | diffuse large B-cell lymphoma central nervous system cerebellar therapy immune-privileged sites |
| url | https://www.mdpi.com/2075-1729/13/1/201 |
| work_keys_str_mv | AT saveriopancetti primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT daoudrahal primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT bethaniafernades primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT carlogalli primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT silviauccella primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT luigimariaterracciano primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT federicopessina primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT lorenzobello primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature AT arturobonometti primarylargebcelllymphomaofimmuneprivilegedsitesofthecerebellumacaseseriesandreviewoftheliterature |