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Case Reports
. 2016 May 21:16:72.
doi: 10.1186/s12883-016-0596-1.

Demyelination as a harbinger of lymphoma: a case report and review of primary central nervous system lymphoma preceded by multifocal sentinel demyelination

Mark D Kvarta  1   2 , Deva Sharma  3 , Rudolph J Castellani  4 , Robert E Morales  5 , Stephen G Reich  6 , Amy S Kimball  7 , Robert K Shin  8
Affiliations
Case Reports

Demyelination as a harbinger of lymphoma: a case report and review of primary central nervous system lymphoma preceded by multifocal sentinel demyelination

Mark D Kvarta et al. BMC Neurol. .

Abstract

Background: Primary central nervous system lymphoma (PCNSL) may rarely be preceded by "sentinel demyelination," a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). Interpreting the overlapping radiologic and clinical characteristics associated with each of these conditions-contrast-enhancing demyelination of white matter and relapsing and remitting steroid-responsive symptoms respectively-can be a significant diagnostic challenge.

Case presentation: We describe a 57-year-old woman with an unusual clinical course who presented with multi-focal enhancing white matter lesions demonstrated to be inflammatory demyelination by brain biopsy. Despite a good initial response to steroids and rituximab for treatment of presumed tumefactive multiple sclerosis, the patient's condition rapidly deteriorated, and a repeat brain biopsy six months later was consistent with a diagnosis of diffuse large B-cell lymphoma.

Conclusions: Early clinical suspicion for PCNSL and awareness that biopsied lesions may initially show sentinel demyelination suggestive of alternate diagnoses may be essential for early initiation of appropriate therapies and mitigation of disease progression. Clinical, pathophysiological, and diagnostic aspects of sentinel demyelination and PCNSL are discussed.

Keywords: Demyelination; Multiple Sclerosis; Pre-operative steroids; Primary CNS Lymphoma.

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Figures

Fig. 1
Fig. 1
Brain MRI (October 2009): axial FLAIR (left) and axial T1 post contrast (right) revealing right frontal and left parieto-occipital enhancing lesions with surrounding edema
Fig. 2
Fig. 2
Histopathology (October 2009): H&E 60x showing reactive perivascular astrocytosis and macrophages (left), CD68 60x immunostain for histiocytes (center), NF 180x showing relative preservation of axons (right)
Fig. 3
Fig. 3
Brain MRI (November 2009): axial FLAIR (left), axial T1 post contrast (right) demonstrating an increase in the size of the left-sided lesions, vasogenic edema, and mass effect leading to subfalcine herniation (notice left parietal burr hole from the biopsy)
Fig. 4
Fig. 4
Brain MRI (April 2010): axial FLAIR (left), axial T1 post contrast (right) reveal progression of nodular enhancement of the persisting left-sided lesions
Fig. 5
Fig. 5
Histopathology (May 2010): H&E 180x showing large malignant perivascular lymphoid cells (left), CD20 180x pan B-cell immunostain (right)
See this image and copyright information in PMC

References

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