Presentation
Limb pain and weakness
Patient Data
An intradural intramedullary enhancing lesion located at T1 has fairly low T2 signal and vivid contrast enhancement. There is extensive cord edema above and below it.
Multiple additional enhancing nodules are seen at the conus and along the cauda equina.
Note is made of L5 on S1 spondylolisthesis due to bilateral pars defects.
No intracranial disease.
Conclusion
In the absence of intracranial disease, the differential is primarily between metastatic disease from an extracranial primary or lymphoma. The cervical lesion could represent an ependymoma with CSF seeding, however, the appearances of the mass would be atypical.
Both the cervicothoracic and thoracolumbar cord demonstrate increased activity at the sites of enhancing lesions on MRI. No other abnormal regions.
Case Discussion
The patient went on to have a biopsy.
Histology
Sections show fragments of a highly cellular tumor. The tumor is composed of diffuse sheets of markedly atypical cells. The cells have large, irregularly shaped nuclei with vesicular chromatin and a small nucleolus. There is some crush-artefact within the specimen.
Immunohistochemistry
Positive for CD45, CD20 and Pax5. More than 90% of cells are positive for Bcl-2, and approximately 60% of cells are positive for Bcl-6. MUM1 is positive in almost all cells. cMyc stains ~20% of cells.
The tumor cells are negative for CD10, Cyclin D1, CD30, CD15, EBER-ISH, AE1/AE3, Cam5.2, GFAP, OLIG2, SOX10 and S100.
Ki67 labels approximately 80% of cells.
Final diagnosis
Intradural intramedullary conus lesion consistent with diffuse large B-cell lymphoma, non-germinal center type (LBCL of immune-privileged site, WHO 2022).
Discussion
Primary CSN lymphoma restricted to the spinal cord is uncommon.
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