AJR 2005; 184:1679-1685
© American Roentgen Ray Society
CT and MRI Findings of Intracranial Lymphoma
H. Wayne Slone1,
Joseph J. Blake,
Rajul Shah,
Sangeeta Guttikonda and
Eric C. Bourekas
1 All authors: Department of Radiology, The Ohio State University Medical Center
and The Ohio State University College of Medicine and Public Health, 629 Means
Hall, 1654 Upham Dr., Columbus, OH 43210.
Received July 26, 2004;
accepted after revision November 8, 2004.
Address correspondence to H. W. Slone
(slone-1{at}medctr.osu.edu).
Presented at the 2004 annual meeting of the American Roentgen Ray Society,
Miami Beach, FL.
Introduction
Primary CNS lymphoma is the confinement of extranodal lymphoma to the CNS.
Classically, lymphomas are divided into Hodgkin's lymphoma and non-Hodgkin's
lymphoma, with a primary extranodal presentation in 5% and 30% of cases,
respectively. With an increasing incidence in both the immunocompetent and
immunocompromised populations, primary CNS lymphoma represents 1% of all
lymphomas and as many as 16% of all primary brain tumors
[1]. This amplified prevalence
makes primary CNS lymphoma an important consideration in the differential
diagnosis of brain lesions. This pictorial essay will review the varied CT and
MRI appearances of intracranial lymphomas.
Primary CNS Lymphoma in the Immunocompetent
Most primary CNS lymphomas are of the non-Hodgkin's B-cell type
[1]. B-cell primary CNS
lymphoma typically presents when the patient is approximately 50 years old and
is more frequent in men. The most common presenting symptom is a change in
mental status followed by nausea, headache, hemiparesis, cerebellar signs,
cranial nerve palsies, and visual disturbances
[1,
2]. Cerebrospinal fluid
analysis yields a cytologic diagnosis in fewer than half of patients with
B-cell primary CNS lymphoma. Neuroimaging reveals solitary lesions that are
most commonly located supratentorially in the white matter of the frontal or
parietal lobes or in the subependymal regions, but the lesions may also
involve the deep gray matter (Figs.
1A,
1B,
1C and
2A,
2B). In 12% of B-cell primary
CNS lymphomas, the leptomeninges are involved
[1]. CT scans usually show high
attenuation, probably because of high cellularity, and virtually all lesions
show homogeneous contrast enhancement (Fig.
1A). On MRI, B-cell primary CNS lymphoma lesions are clearly
delineated masses that appear isointense to hypointense on T1-weighted images
and mostly hypointense on T2-weighted images
[1,
2]
(Fig. 1B). Nearly all lesions
show homogeneous enhancement with contrast material
(Fig. 1C). A classic
presentation is the lesion that crosses the corpus callosum in a butterfly
pattern (Fig. 3A,
3B). Rarely, necrosis, cyst
formation, calcification, and hemorrhage can be seen. If steroids are
administered, the tumor may shrink and "vanish," compromising the
ability to obtain a histologic diagnosis (Fig.
4A,
4B).
View larger version (169K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1A. —72-year-old immunocompetent woman with primary CNS
non-Hodgkin's B-cell lymphoma who presented with progressive motor weakness.
Unenhanced CT image shows classic hyperdense masses involving deep white and
gray matter.
|
|
View larger version (159K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B. —72-year-old immunocompetent woman with primary CNS
non-Hodgkin's B-cell lymphoma who presented with progressive motor weakness.
Axial FLAIR MR image shows isointensity of lesions to brain parenchyma and
surrounding edema.
|
|
View larger version (149K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1C. —72-year-old immunocompetent woman with primary CNS
non-Hodgkin's B-cell lymphoma who presented with progressive motor weakness.
Axial contrast-enhanced T1-weighted MR image shows homogeneous enhancement of
multiple bilateral tumors.
|
|
View larger version (141K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2A. —62-year-old immunocompetent woman with primary CNS
non-Hodgkin's B-cell lymphoma with leptomeningeal spread who presented with
left hemiparesis, severe headache, and confusion. Systemic workup was
negative. Cerebrospinal fluid cytology was positive for leptomeningeal spread.
Axial FLAIR MR image shows high-signal-intensity edema in white matter around
trigone of left lateral ventricle.
|
|
View larger version (127K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2B. —62-year-old immunocompetent woman with primary CNS
non-Hodgkin's B-cell lymphoma with leptomeningeal spread who presented with
left hemiparesis, severe headache, and confusion. Systemic workup was
negative. Cerebrospinal fluid cytology was positive for leptomeningeal spread.
Contrast-enhanced coronal T1-weighted MR image shows focal enhancing lesion in
deep white matter of left parietal and posterior temporal regions with
enhancement of adjacent subependyma (arrow).
|
|
View larger version (145K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3A. —50-year-old immunocompetent man with primary CNS
non-Hodgkin's B-cell lymphoma. Axial T2-weighted MR image shows infiltrative
hyperintense mass expanding genu and splenium of corpus callosum in butterfly
pattern.
|
|
View larger version (154K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 4A. —61-year-old immunocompetent man with primary CNS
non-Hodgkin's B-cell lymphoma and "vanishing" hyperdense masses
when treated with steroids. Systemic workup was negative. Initial axial CT
image shows hyperdense masses in basal ganglia bilaterally.
|
|
View larger version (136K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 4B. —61-year-old immunocompetent man with primary CNS
non-Hodgkin's B-cell lymphoma and "vanishing" hyperdense masses
when treated with steroids. Systemic workup was negative. On follow-up CT
image obtained 10 days after initiation of steroids, lesions have nearly
resolved, consistent with "vanishing" tumor.
|
|
Primary CNS Lymphoma in the Immunocompromised
Immunocompromised patients are at increased risk for developing primary CNS
lymphoma. In fact, estimates indicate that nearly 6% of the AIDS population
will be afflicted with an intracranial lymphoma
[3]. Indeed, primary CNS
lymphoma in an HIV-seropositive patient is an AIDS-defining condition. The age
at presentation is earlier (fourth decade) in immunocompromised patients than
in the immunocompetent, but the cell type (B cell) and presenting signs and
symptoms are similar. Neuroimaging reveals a higher frequency of multiple
lesions and more often displays irregular margins, heterogeneity, and ring
enhancement [1,
3] (Figs.
5A,
5B,
5C and
6A,
6B,
6C). In the immunocompromised
population, an important dilemma is the difficulty in distinguishing primary
CNS lymphoma from the more common cerebral toxoplasmosis using CT and MRI,
because both entities can present with multiple ring-enhancing lesions.
Thallium SPECT or PET can aid in this setting, although frequently the patient
is treated for presumed toxoplasmosis, and if the patient responds the
diagnosis is established.
View larger version (154K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5A. —44-year-old HIV-positive woman with primary CNS non-Hodgkin's
B-cell lymphoma. She presented with changes in mental status and CD-4 count of
0. Contrast-enhanced CT image shows low-density infiltrating butterfly lesion
crossing corpus callosum with ring of enhancement (arrows).
|
|
View larger version (167K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5B. —44-year-old HIV-positive woman with primary CNS non-Hodgkin's
B-cell lymphoma. She presented with changes in mental status and CD-4 count of
0. Axial FLAIR MR image shows lesion isointense to gray matter
(arrows).
|
|
View larger version (137K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5C. —44-year-old HIV-positive woman with primary CNS non-Hodgkin's
B-cell lymphoma. She presented with changes in mental status and CD-4 count of
0. Contrast-enhanced T1-weighted axial MR image shows ring of enhancement
(arrows).
|
|
View larger version (139K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 6A. —42-year-old HIV-positive man with primary CNS non-Hodgkin's
B-cell lymphoma. Presenting signs were third and fourth cranial nerve palsies.
Patient underwent irradiation 1 year earlier for stage I palate cancer. Axial
FLAIR MR image shows lesion involving left cerebral peduncle.
|
|
View larger version (114K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 6B. —42-year-old HIV-positive man with primary CNS non-Hodgkin's
B-cell lymphoma. Presenting signs were third and fourth cranial nerve palsies.
Patient underwent irradiation 1 year earlier for stage I palate cancer.
Contrast-enhanced coronal T1-weighted MR image shows masslike enhancement.
|
|
View larger version (95K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 6C. —42-year-old HIV-positive man with primary CNS non-Hodgkin's
B-cell lymphoma. Presenting signs were third and fourth cranial nerve palsies.
Patient underwent irradiation 1 year earlier for stage I palate cancer.
Contrast-enhanced coronal T1-weighted MR image shows enhancement along third
and fourth cranial nerves (arrow).
|
|
Primary Leptomeningeal Lymphoma
Although extension of primary CNS lymphoma into the leptomeninges is
common, primary leptomeningeal lymphoma is rare, constituting fewer than 8% of
all cases of primary CNS lymphoma
[4]. The clinical presentation
of primary leptomeningeal lymphoma is similar to that of B-cell primary CNS
lymphoma but may also include dizziness, tinnitus, spinal neuropathies, and
meningismus. The diagnosis is often elusive because clinical findings are
often suggestive of meningoencephalitis or common conditions that cause
increased intracranial pressure. Analysis of the cerebrospinal fluid of
patients with primary leptomeningeal lymphoma has failed to show a consistent
presence of malignant cells. Neuroimaging is often unremarkable or may show
nonspecific findings such as hydrocephalus. On occasion, significant imaging
findings may include widespread meningeal calcification, discrete masses or
densities, and faint meningeal enhancement (Figs.
7A,
7B,
7C and
8). In the absence of other
findings, proton-density or FLAIR MRI revealing the presence of high signal
intensity in the subarachnoid space may support a diagnosis of primary
leptomeningeal lymphoma.
View larger version (139K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 7A. —63-year-old woman with primary meningeal lymphoma who
presented with frequent falls and vertigo. CT scan from outside institution
(not shown) showed hyerdensity along surface of parietotemporal
covexity that was incorrectly interpreted as subdural hemorrhage. Systemic
workup was negative. Axial FLAIR image shows hyperintensity (arrow)
involving sulci and leptomeninges of parietotemporal convexity.
|
|
View larger version (125K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 7B. —63-year-old woman with primary meningeal lymphoma who
presented with frequent falls and vertigo. CT scan from outside institution
(not shown) showed hyerdensity along surface of parietotemporal
covexity that was incorrectly interpreted as subdural hemorrhage. Systemic
workup was negative. Contrast-enhanced T1-weighted axial (B) and
coronal (C) MR images show focal thickening and homogeneous enhancement
of leptomeninges of parietotemporal convexity (arrows).
|
|
View larger version (128K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 7C. —63-year-old woman with primary meningeal lymphoma who
presented with frequent falls and vertigo. CT scan from outside institution
(not shown) showed hyerdensity along surface of parietotemporal
covexity that was incorrectly interpreted as subdural hemorrhage. Systemic
workup was negative. Contrast-enhanced T1-weighted axial (B) and
coronal (C) MR images show focal thickening and homogeneous enhancement
of leptomeninges of parietotemporal convexity (arrows).
|
|
View larger version (149K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 8. —39-year-old man with AIDS and primary CNS non-Hodgkin's
B-cell lymphoma of leptomeninges who presented with diplopia, facial weakness,
and eyelid droop. No parenchymal lesions were identified. Coronal
contrast-enhanced T1-weighted MR image shows enhancement of multiple cranial
nerves (arrows) bilaterally.
|
|
Metastatic CNS Lymphoma, B-Cell Type
In 5–9% of systemic non-Hodgkin's lymphoma, secondary spread involves
the CNS [5], usually in the
form of leptomeningeal infiltrates, and has a poor prognosis. Parenchymal
lesions, when present, typically result from secondary involvement from the
leptomeninges via infiltration of the perivascular spaces (Fig.
9A,
9B).
View larger version (102K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 9A. —71-year-old woman with metastatic leptomeningeal CNS
non-Hodgkin's B-cell lymphoma who presented with left facial droop. She was
previously diagnosed with systemic stage IV non-Hodgkin's lymphoma (B-cell
type). Axial contrast-enhanced T1-weighted MR image shows linear
leptomeningeal enhancement (arrows). Enhancement of fifth cranial
nerve (arrowhead) is evident as well.
|
|
View larger version (114K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 9B. —71-year-old woman with metastatic leptomeningeal CNS
non-Hodgkin's B-cell lymphoma who presented with left facial droop. She was
previously diagnosed with systemic stage IV non-Hodgkin's lymphoma (B-cell
type). Coronal contrast-enhanced T1-weighted MR image shows enhancement of
fifth, seventh, and eighth cranial nerves (arrows).
|
|
Intravascular Lymphomatosis
With fewer than 50 reported cases, intravascular lymphomatosis is an
extraordinarily rare form of large B-cell non-Hodgkin's lymphoma that is
characterized by aggressive, intravascular proliferation of lymphoid cells.
Proclivity for involvement and subsequent occlusion of CNS vessels often leads
to nonlocalizing neurologic deficits and changes in mental status. Because no
specific clinical or laboratory findings are associated with intravascular
lymphomatosis, the diagnosis is rarely established before histologic
examination during autopsy. MRI findings in intravascular lymphomatosis
include high-signal deep white matter lesions and infarctlike, high-signal
lesions in vascular territories on T2-weighted images. After the
administration of contrast material, enhancement can be masslike
[6] (Fig.
10A,
10B,
10C). Various patterns of
parenchymal and meningeal enhancement may also be seen.
View larger version (91K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 10B. —48-year-old man with intravascular non-Hodgkin's B-cell
lymphoma who presented with left leg weakness for 1 year. Diffusion-weighted
axial MR image shows restricted diffusion of lesion.
|
|
View larger version (114K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 10C. —48-year-old man with intravascular non-Hodgkin's B-cell
lymphoma who presented with left leg weakness for 1 year. Contrast-enhanced
axial T1-weighted MR image shows nodular enhancement.
|
|
Primary CNS Lymphoma, T-Cell Type
Primary T-cell lymphoma of the CNS constitutes a small fraction of all
primary CNS lymphomas in the immunocompetent population. A threefold higher
incidence of T-cell primary CNS lymphoma in Japan compared with the United
States has been reported. In a review of 25 cases of T-cell primary CNS
lymphoma, Liu et al. [7]
reported that T-cell primary CNS lymphoma is similar to B-cell primary CNS
lymphoma in clinical presentation and imaging features. Unlike B-cell primary
CNS lymphoma, involvement of the cerebrospinal fluid in T-cell primary CNS
lymphoma is uncommon. CT and MRI typically show one or more homogeneous masses
that uniformly enhance with contrast material
[7] (Fig.
11A,
11B). Association with AIDS or
other types of immunodeficiency has only rarely been reported.
View larger version (159K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 11A. —40-year-old man with primary CNS non-Hodgkin's T-cell
lymphoma who presented with seizure. Axial FLAIR MR image shows hyperintense
signal in tectum and posterior aspect of midbrain (arrow).
|
|
Intracranial Hodgkin's Lymphoma
Fewer than 0.5% of patients with Hodgkin's lymphoma have CNS involvement,
and most of these cases are late manifestations of disseminated disease
outside the CNS. Primary intracranial Hodgkin's lymphoma, with only a few case
reports, is perhaps the rarest of all intracranial lymphomas. In the reported
cases, neuroimaging usually shows meningeal involvement. Intracranial
Hodgkin's lymphoma may mimic meningioma, although parenchymal lesions without
meningeal attachment have been reported
[8] (Figs.
12A,
12B and
13A,
13B,
13C).
View larger version (151K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 12A. —48-year-old woman with primary Hodgkin's lymphoma who
presented with progressive left-sided weakness. Systemic workup was negative.
Contrast-enhanced CT image shows enhancement along frontoparietal
convexity.
|
|
View larger version (149K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 12B. —48-year-old woman with primary Hodgkin's lymphoma who
presented with progressive left-sided weakness. Systemic workup was negative.
Contrast-enhanced T1-weighted coronal MR image shows dura-based enhancing
lesion with "dural tail" (arrows) causing compression of
frontal lobe and subfalcine herniation.
|
|
View larger version (147K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 13A. —35-year-old HIV-positive man with systemic nodular sclerosing
Hodgkin's lymphoma who presented with mental status change. Last CD-4 count
was more than 1,200 per cubic millimeter with undetectable viral load. Patient
did not respond to antitoxoplasmosis treatment and underwent biopsy. Axial
unenhanced CT image shows hypodense vasogenic edema around subtle hyperdense
lesion (arrow) along medial margin of left parietooccipital lobe.
|
|
View larger version (152K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 13B. —35-year-old HIV-positive man with systemic nodular sclerosing
Hodgkin's lymphoma who presented with mental status change. Last CD-4 count
was more than 1,200 per cubic millimeter with undetectable viral load. Patient
did not respond to antitoxoplasmosis treatment and underwent biopsy. Axial
T2-weighted MR image shows low signal intensity of lesion
(arrow).
|
|
View larger version (130K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 13C. —35-year-old HIV-positive man with systemic nodular sclerosing
Hodgkin's lymphoma who presented with mental status change. Last CD-4 count
was more than 1,200 per cubic millimeter with undetectable viral load. Patient
did not respond to antitoxoplasmosis treatment and underwent biopsy. Axial
T1-weighted contrast-enhanced MR image shows leptomeningeal enhancement
(arrows).
|
|
Conclusion
The CT and MRI findings of intracranial lymphomas can be nonspecific or
share common features with other diseases such as demyelinating disorders,
other neoplasms, sarcoid, tuberculosis, and toxoplasmosis. Therefore, a
definitive diagnosis of primary CNS lymphoma requires histologic assessment.
However, a high index of suspicion and the presence of features similar to
those illustrated in this article can aid in the diagnosis of intracranial
lymphoma.
References
- Koeller KK, Smirniotopoulos JG, Jones RV. Primary central nervous
system lymphoma: radiologic-pathologic correlation.
RadioGraphics1997; 17:1497
–1526[Abstract]
- Coulon A, Lafitte F, Hoang-Xuan K, et al. Radiographic findings in
37 cases of primary CNS lymphoma in immunocompetent patients. Eur
Radiol 2002;12:329
–340[Medline]
- Thurnher MM, Rieger A, Kleibl-Popov C, et al. Primary central
nervous system lymphoma in AIDS: a wider spectrum of CT and MRI findings.
Neuroradiology2001; 43:29
–35[Medline]
- Lachance DH, O'Neil BP, Macdonald DR, et al. Primary leptomeningeal
lymphoma: report of 9 cases, diagnosis with immunocytochemical analysis, and
review of the literature. Neurology1991; 41:95
–100[Abstract/Free Full Text]
- Goetz P, Lafuente J, Revesz T, Galloway M, Dogan A, Kitchen N.
Primary low-grade B-cell lymphoma of mucosa-associated lymphoid tissue of the
dura mimicking the presentation of an acute subdural hematoma. J
Neurosurg 2002;96:611
–614[Medline]
- Martin-Duverneuil N, Mokhtari K, Behin A, Lafitte F, Hoang-Xuan K,
Chiras J. Intravascular malignant lymphomatosis.
Neuroradiology2002; 44:749
–754[Medline]
- Liu D, Schelper RL, Carter DA, et al. Primary central nervous
system cytotoxic/suppressor T-cell lymphoma. Am J Surg
Pathol 2003;27:682
–688[Medline]
- Deckert-Schluter M, Marek J, Setlik M, et al. Primary manifestation
of Hodgkin's disease in the central nervous system. Virchows
Arch 1998;432:477
–481[Medline]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
Top
Introduction
Primary CNS Lymphoma in...
