A 29-year-old man presented with tonic-clonic seizures. Initial MRI showed a lesion centered on the white matter of the left frontal lobe, with restricted diffusion and contrast enhancement on its margins and low rCBV and hypometabolismon PET-CT, suggestive of a tumefactive demyelination lesion (Figure 1). Patient underwent surgical biopsy, with no signs of malignancy (Figure 2). Two months later, control MRI showed a new lesion on the brainstem, with solid enhancement and hypermetabolism on PET-CT, compatible with lymphoma (Figures 3 and 4).
Demyelinating sentinel lesions preceding CNS lymphomas are a rare entity and its pathophysiology is not fully understood11. Kvarta MD, Sharma D, Castellani RJ, Morales RE, Reich SG, Kimball AS, et al. Demyelination as a harbinger of lymphoma: a case report and review of primary central nervous system lymphoma preceded by multifocal sentinel demyelination. BMC Neurol. 2016 May 21;16:72. https://doi.org/10.1186/s12883-016-0596-1
https://doi.org/10.1186/s12883-016-0596-...
,22. Kuhlmann T, Schröter A, Dechent P, Weber F, Rustenbeck HH, Füzesi L, et al. Diagnosis of a multifocal B cell lymphoma with preceding demyelinating central nervous system lesions by single voxel proton MR spectroscopy. J Neurol Neurosurg Psychiatry. 2001 Feb 1;70(2):259-62. https://doi.org/10.1136/jnnp.70.2.259
https://doi.org/10.1136/jnnp.70.2.259...
.
A: Axial diffusion weighted imaging (DWI), showing a left frontal lesion with restricted diffusion on the lesion’s free margin, oriented towards the white matter, suggestive of demyelinating nature. B: Axial post-gadolinium T1, showing contrast enhancement on the lesions free margin. C: Coronal post-gadolinium T1 shows the left frontal lesion, insinuating towards the corpus callosum, but with no frank signs of invasion. Notice the spared brainstem.
Histopathological findings from surgical biopsy. A: Hematoxicilin-eosin 100x, amplified on B, shows a diffuse inflammatory infiltrate composed by T-lymphocytes, confirmed by immunohistochemistry for CD3 marker on C, plasmacytes (CD138 on D) and foamy macrophages (CD68 on E). The sample was negative for malignancy and markers for B cells were negative (not shown).
Control MRI two months later, demonstrates persistent restricted diffusion (A), but less enhancement of the left frontal lesion (B). C (coronal post-gadolinium T1): Its caudal aspect extends and invades the corpus callosum. Notice the development of a new and solid-enhancing lesion on the brainstem, extending along the cerebral peduncles and the postoperative changes on the left frontal lobe.
References
- 1. Kvarta MD, Sharma D, Castellani RJ, Morales RE, Reich SG, Kimball AS, et al. Demyelination as a harbinger of lymphoma: a case report and review of primary central nervous system lymphoma preceded by multifocal sentinel demyelination. BMC Neurol. 2016 May 21;16:72. https://doi.org/10.1186/s12883-016-0596-1
» https://doi.org/10.1186/s12883-016-0596-1 - 2. Kuhlmann T, Schröter A, Dechent P, Weber F, Rustenbeck HH, Füzesi L, et al. Diagnosis of a multifocal B cell lymphoma with preceding demyelinating central nervous system lesions by single voxel proton MR spectroscopy. J Neurol Neurosurg Psychiatry. 2001 Feb 1;70(2):259-62. https://doi.org/10.1136/jnnp.70.2.259
» https://doi.org/10.1136/jnnp.70.2.259
Publication Dates
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Publication in this collection
29 Nov 2021 -
Date of issue
Jan 2022
History
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Received
12 July 2021 -
Reviewed
09 Aug 2021 -
Accepted
15 Aug 2021