Eur Radiol (2003) 13:1753–1755
© Springer-Verlag 2003

I N T E R P R E TAT I O N C O R N E R

Y. De Bruecker
P. Demaerel
G. Wilms
R. Sciot
F. Ballaux
W. Robberecht

A case of hemiparesis associated
with cranial nerve lesions due to intravascular
lymphomatosis (2003:4b)

Y. De Bruecker · P. Demaerel (✉)
G. Wilms
Department of Radiology,
University Hospitals,
Catholic University of Leuven,
Herestraat 49, 3000 Leuven, Belgium
Tel.: +32-16-343782, Fax: +32-16-343765
e-mail:
philippe.demaerel@uz.kuleuven.ac.be

Abstract Intravascular lymphomatosis is a rare variant of non-Hodgkin’s
lymphoma with an unusual predilection for the central nervous system.
Most cases are not diagnosed until
postmortem because of variable clinical presentation and non-specific
laboratory findings. We studied a 41year-old lady who presented with
progressive neurological symptoms.
MR showed multifocal grey and
white matter lesions. Brain biopsy
showed an intravascular lymphoma
of the B-cell lineage. This report illustrates the ischaemic origin of the
radiological lesions, as they are all
hyperintense on the diffusion-

R. Sciot · F. Ballaux
Department of Pathology,
University Hospitals,
Catholic University of Leuven,
Leuven, Belgium
W. Robberecht
Department of Neurology,
University Hospitals,
Catholic University of Leuven,
Leuven, Belgium

Case report
A 41-year-old woman, without any relevant medical history, presented with a progressive left hemiparesis. Neurological examination revealed a left homonymous hemianopia, a central left VIIth
nerve paresis and a left XIIth nerve paresis. She had been complaining of lethargy and weight loss for months. The laboratory
data showed no significant abnormalities. Cerebrospinal fluid
analysis was normal.
T2-weighted images showed several hyperintense cerebral lesions. Grey matter lesions were seen in the basal ganglia on both
sides, the right frontoparietal cortex, anteriorly in the right frontal
cortex and in the left gyrus cinguli. White matter lesions were
found in the genu and splenium of the corpus callosum, in the centrum semi-ovale and the corona radiata on both sides and in the
right parietal lobe. All lesions were hyperintense on the FLAIRsequence (Fig. 1) and there was no enhancement after intravenous contrast medium administration. 3D-TOF MR-angiography
showed a filiform stenosis of a right middle cerebral artery branch.
Catheter angiography confirmed this stenosis (Fig. 2) and showed
additional lesions in a right posterior cerebral artery branch and a
right anterior cerebral artery branch.
Transesophageal echocardiography (TEE) was normal.

weighted images. This has, to our
knowledge, never been published before. MR did not show any enhancement after intravenous GadoliniumDTPA (parenchymal or meningeal)
which is a very uncommon finding
in this entity. Intravascular lymphomatosis should be taken into account
in the differential diagnosis of repeated cerebral ischemia of unclear
aetiology.
Keywords Vasculitis · Lymphoma ·
Diffusion-weighted

Additional blood examinations showed a normal level of antineutrophil cytoplasmatic autoantibodies (ANCA) and circulating
immune complexes.
Open brain biopsy was performed two days after the initial
MR-examination. Histological examination showed atypical cell
clusters in the intraparenchymatous and leptomeningeal blood
vessels. The atypical cells were large with vesicular nuclei, prominent nucleoli and some showed mitotic figures (Fig. 3). Furthermore, immunohistochemical examination with antibodies directed
against CD-45 and CD-20 was positive, while CD3 was negative
in the tumor cells.
Postoperative MR on day 6 showed new lesions in the grey
and white matter of the right temporal lobe. On diffusion-weighted
MR images, the existing and the new lesions were strongly hyperintense, suggesting recent infarcts (Fig. 4). FDG-PET showed no
abnormalities.

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Fig. 3 Microscopic specimen shows an intravascular cluster of
atypical cells. This cluster fills the lumen of this intraparenchymatous vessel. The cells have a prominent vesicular nucleus

Fig. 1 Axial FLAIR image shows white matter lesions in the right
side of the genu and splenium of the corpus callosum and in the
right occipital lobe. This image also shows grey matter lesions on
both sides in the nucleus lentiformis, in the head of the right nucleus caudatus, the right frontoparietal cortex and anteriorly in the
right frontal cortex

Fig. 4 Postoperative (day 6) diffusion-weighted MR-image shows
the hyperintensity of the new lesions in the right temporal lobe.
The existing lesions in the right parietal lobe and in the right side
of the splenium of the corpus callosum are also hyperintense

Fig. 2 Catheter angiography shows a filiform stenosis of a middle
cerebral artery branch (arrow)

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Diagnosis
The microscopic features defined the lesions as an intravascular large B-cell lymphoma.

Discussion
Intravascular lymphomatosis was first described as angioendotheliomatosis proliferans systemisata in 1959 [1]. Later, immunocytochemical and cytogenetic studies revealed
that the malignant cells were of lymphoid origin [2, 3].
The initial predilection sites of this rare variant of nonHodgkin’s lymphoma, are the brain and the skin [3]. Most
cases are not diagnosed during life because of the variable
clinical presentation and non-specific laboratory findings.
Although this entity is well recognized in the literature, the imaging findings have not been characterized in
detail as only a limited number of cases have been reported until now. Multifocal hyperintense lesions on T2weighted images, probably caused by small vessel occlusion leading to ischemia and infarction, is the most common imaging finding [4]. Enhancing mass lesions should
be considered as an extraluminal spread of the tumor
cells [4]. Dural/arachnoidal enhancement found in some
cases of intravascular lymphomatosis could be used as a

diagnostic tool to differentiate this entity from vasculitis
[4]. In this case, we found several hyperintense lesions
on the T2-weighted images. Multifocal grey matter lesions were suggestive of infarction caused by emboli or
vasculitis, whereas some white matter lesions suggested
demyelination. TEE excluded a cardiac source of emboli. Multiple sclerosis was excluded from the differential
diagnosis on the clinical basis and on the basis of the cerebrospinal fluid analysis. Moreover the angiographic
findings and the presence of grey matter lesions were not
compatible with multiple sclerosis.
A normal level of immunological blood results made
the diagnosis of vasculitis unlikely.
The parenchymal lesions did not enhance and MR
showed furthermore no meningeal (dural/arachnoidal)
enhancement. In the review article of Williams et al [4],
only 1 out of 7 patients presented with the same non-enhancement characteristics.
Most of the lesions are hyperintense on the diffusion
weighted images, which, to our knowledge, has never
been described before in this entity. This supports -or
even proves- the hypothesis that the lesions are caused
by small vessel obstruction by tumoral cells.
Intravascular lymphomatosis should be taken into account in the differential diagnosis of repeated cerebral
ischaemia of unclear aetiology.

References
1. Pfleger V, Tappeiner J (1959) Zur
Kenntnis der systemisierten Endotheliomatose der cutaner Blutgefässe
(reticuloendotheliosis?). Hautarzt
10:359-363

2. Sheibani K, Battifora H, Wingberg CD,
Burke JS, Ben Ezra J, Ellinger GM,
Quigley NJ, Fernandez BB, Morrow D,
Rappaport H (1986) Further evidence
that malignant angioendotheliomatosis
is an angiotropic large cell-lymphoma.
N Engl J Med 314:943-948
3. Demirer T, Dail DH, Aboulafia DM
(1994) Four varied cases of intravascular lymphomatosis and a literature review. Cancer 73:1738-1745

There were no precisely correct answers to this case. Numerous excellent responses were obtained and the suggestions included vasculitis/angiitis, sarcoid, CADASIL,
etc. Perhaps the case was too complex – even the authors
suggested that it was a rare feature!

4. Williams RL, Meltzer CC,
Smirnitopoulos JG, Fukui MB,
Inman M (1998) Cerebral MR imaging
in intravascular lymphomatosis. Am J
Neuroradiol 19:427-431