Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Jan 10;13(1):201.
doi: 10.3390/life13010201.

Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature

Saverio Pancetti  1   2 , Daoud Rahal  2 , Bethania Fernades  2 , Carlo Galli  2 , Silvia Uccella  1   2 , Luigi Maria Terracciano  1   2 , Federico Pessina  1   3 , Lorenzo Bello  4 , Arturo Bonometti  1   2
Affiliations
Review

Primary Large B-Cell Lymphoma of Immune-Privileged Sites of the Cerebellum: A Case Series and Review of the Literature

Saverio Pancetti et al. Life (Basel). .

Abstract

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.

Keywords: central nervous system; cerebellar; diffuse large B-cell lymphoma; immune-privileged sites; therapy.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Radiological and histopathological features of the three described cases. Case 1: brain MRI showing a mass in the left cerebellar hemisphere (A); hematoxylin and eosin with sheets of large pleomorphic cells (B); immunoreactive for CD20 (C); the lymphoma exhibited a high proliferation index (70%) (D). In (C,D) is also evident a large area of necrosis. Case 2: MRI showing a mass in the right hemisphere (E). Histopathological analisys revealed sparse CD3+ (F) and Bcl2+ (G) T-cells and a B-cell lymphoproliferation expressing MUM1 (H), CD20 (I), and p53 (J); the lymphoma exhibited a high proliferation index (K). Case 3: brain MRI showing a mass in the right cerebellar hemisphere close to the dentate nucleus (L); cerebellar sample staining in HE large lymphoid cells with centralistic morphology and perivascular preservation (M); the lymphoma expressed PAX5 (N) and exhibited a high proliferation index (O).
Figure 2
Figure 2
Survival analyses of cerebellar IP-LBCL patients. Overall survival of the patients’ cohort (A). Comparison of survival of patients with and without intracranial hypertension ((B), p = 0.03) and between those with reported CD10 expression and other cases ((C), p = 0.25).
See this image and copyright information in PMC

References

    1. Schlegel U. Primary CNS Lymphoma. Ther. Adv. Neurol. Disord. 2009;2:93–104. doi: 10.1177/1756285608101222. - DOI - PMC - PubMed
    1. Houillier C., Soussain C., Ghesquières H., Soubeyran P., Chinot O., Taillandier L., Lamy T., Choquet S., Ahle G., Damaj G., et al. Management and Outcome of Primary CNS Lymphoma in the Modern Era. Neurology. 2020;94:e1027–e1039. doi: 10.1212/WNL.0000000000008900. - DOI - PMC - PubMed
    1. Schaff L.R., Grommes C. Primary Central Nervous System Lymphoma. Blood. 2022;140:971–979. doi: 10.1182/blood.2020008377. - DOI - PMC - PubMed
    1. Küker W., Nägele T., Korfel A., Heckl S., Thiel E., Bamberg M., Weller M., Herrlinger U. Primary Central Nervous System Lymphomas (PCNSL): MRI Features at Presentation in 100 Patients. J. Neurooncol. 2005;72:169–177. doi: 10.1007/s11060-004-3390-7. - DOI - PubMed
    1. Alaggio R., Amador C., Anagnostopoulos I., Attygalle A.D., de Oliveira Araujo I.B., Berti E., Bhagat G., Borges A.M., Boyer D., Calaminici M., et al. The 5th Edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022;36:1720–1748. doi: 10.1038/s41375-022-01620-2. - DOI - PMC - PubMed

LinkOut - more resources