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Case Reports
. 2021 Jul 21:12:645-652.
doi: 10.2147/JBM.S325264. eCollection 2021.

Primary Cauda Equina Lymphoma Treated with CNS-Centric Approach: A Case Report and Literature Review

Justin J Kuhlman  1 , Muhamad Alhaj Moustafa  2 , Vivek Gupta  3 , Liuyan Jiang  4 , Han W Tun  2
Affiliations
Case Reports

Primary Cauda Equina Lymphoma Treated with CNS-Centric Approach: A Case Report and Literature Review

Justin J Kuhlman et al. J Blood Med. .

Abstract

Primary cauda equina lymphoma is an extremely rare entity previously documented in only 24 reported cases. Primary cauda equina lymphoma represents a subtype of neurolymphomatosis, which occurs when lymphoma cells with neurotropism infiltrate and destroy peripheral nerves, spinal nerve roots, nerve plexuses and cranial nerves. The cauda equina is an anatomic structure located in the lower part of the spinal canal consisting of multiple lumbar and sacral nerve roots. Herein, we report a unique case of primary cauda equina diffuse large B-cell lymphoma presenting as a tumor mass in the lower spinal canal, which was treated with a CNS-centric treatment approach followed by autologous hematopoietic stem cell transplantation.

Keywords: MATRIX chemoimmunotherapy; autologous stem cell transplant; diffuse large B cell cauda equina lymphoma; neurolymphomatosis; primary cauda equina lymphoma.

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Conflict of interest statement

Dr Han W Tun reports grants, personal fees from Acrotech pharmaceutical, grants from Celgene, grants from Bristol Myers Squibb, grants from Mundipharma, grants from TG Therapeutic, grants from Curis, grants from Zhejiang DTRM Biopharma, outside the submitted work. In addition, Dr Han W Tun has a patent 9464093 (USA), a patent 9839632 (USA), and a patent 10301273 (USA). The authors declare that there are no other conflicts of interest regarding the publication of this manuscript.

Figures

Figure 1
Figure 1
(A) Gadolinium enhanced sagittal and coronal T1 MRI showing diffuse involvement of cauda equina with encasement of conus medullaris (arrows). (B) Staging whole-body 18F-FDG PET-CT coronal and sagittal views showing hypermetabolic mass in the cauda equina and lumbar nerve root sleeves (arrows).
Figure 2
Figure 2
(A) Pathology examination by H&E staining revealing diffusely infiltrating large atypical lymphocytes dissecting through the nerve bundles (arrow indicating residual nerve). IHC studies showed that the neoplastic lymphocytes were positive for (B) CD20, (C) BCL2, (D) BCL6, (E) MUM1, (F) with a high proliferate rate (>90%) by Ki-67.
Figure 3
Figure 3
Follow up MRI (A) and PET-CT (B) after chemotherapy showing complete resolution of the hypermetabolic cauda equina lesion. Linear FDG uptake in the posterior lumbar dura (arrows) is postoperative in nature.
See this image and copyright information in PMC

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