Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature
Saverio Pancetti,
Daoud Rahal,
Bethania Fernades,
Carlo Galli,
Silvia Uccella,
Luigi Maria Terracciano,
Federico Pessina,
Lorenzo Bello,
Arturo Bonometti
Affiliations
Saverio Pancetti
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, Italy
Daoud Rahal
Department of Pathology Unit, Humanitas Clinical and Research Hospital, Via Manzoni 56, Rozzano, 20089 Milan, Italy
Bethania Fernades
Department of Pathology Unit, Humanitas Clinical and Research Hospital, Via Manzoni 56, Rozzano, 20089 Milan, Italy
Carlo Galli
Department of Pathology Unit, Humanitas Clinical and Research Hospital, Via Manzoni 56, Rozzano, 20089 Milan, Italy
Silvia Uccella
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, Italy
Luigi Maria Terracciano
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, Italy
Federico Pessina
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, Italy
Lorenzo Bello
Neurosurgical Oncology Unit, Department of Oncology and Hemato-Oncology, IRCCS Galeazzi-Sant’Ambrogio, Università degli Studi di Milano, 20161 Milan, Italy
Arturo Bonometti
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090 Milan, Italy
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.
