Neurosurg Rev (2009) 32:171–179

DOI 10.1007/s10143-009-0185-5

ORIGINAL ARTICLE

Tumefactive demyelinating lesions: nine cases and a review

of the literature
Lei Xia & Song Lin & Zhong-cheng Wang & Shao-wu Li &
Li Xu & Jing Wu & Shu-yu Hao & Chuan-chuan Gao

Received: 18 April 2008 / Revised: 8 November 2008 / Accepted: 6 December 2008 / Published online: 27 January 2009
# Springer-Verlag 2009

Abstract Tumefactive demyelinating lesions (TDLs) are well to steroid therapy and no relapse was found during
misdiagnosed frequently. To investigate the characteristics following up. Thus, intensive analysis of both clinical and
of TDLs, clinical and radiological data from nine cases with radiological data may provide some clues for diagnosis. For
TDLs were analyzed after admission. All cases underwent suspected cases, it is advisable to take steroid therapy or
surgery and pathological examination; some received undergo advanced radiological examinations, such as serial
postoperative steroid therapy. Onsets were mostly within magnetic resonance spectroscopy. However, in difficult
3 weeks and main presentation included intracranial cases, pathological evidence is beneficial to a final diagnosis.
hypertension, extremity weakness, epilepsy, and visual
disturbance. Symptoms in children were acute and severe, Keywords Tumefactive demyelinating lesions .
frequently including headache, vomiting, and visual distur- Demyelinating pseudotumor . Demyelinating diseases .
bance. Most intracephalic lesions were in cerebral hemi- Brain neoplasms
spheres. All intraspinal lesions were in cervical segments.
Radiological features included mass effect, perifocal edema
and enhancement (of which open-ring enhancement was Introduction
diagnostic), and decreased relative cerebral blood volume.
Intraoperative frozen section did not confirm the diagnosis, The primary demyelinating diseases of the central nervous
while postoperative paraffin section did confirm it (by system (CNS) encompass a series of entities, such as acute
evidence of macrophage infiltration). The patients responded disseminated encephalomyelitis, acute hemorrhagic leu-
koencephalopathy, and various types of multiple sclerosis
(MS). The latter entity includes chronic type (Charcot type),
L. Xia : Z.-c. Wang : L. Xu : S.-y. Hao : C.-c. Gao
acute type (Marburg type), myelinoclastic diffuse sclerosis
Beijing Neurosurgical Institute, Capital Medical University,
Beijing 100050, China (Schilder type), concentric sclerosis, and optic neuromye-
litis (Devic type), of which Charcot type (classical MS) is
S. Lin the most common and best known. But a rare demyelinat-
Neurosurgical Department, Beijing Tiantan Hospital,
ing disease presenting as a large (>2 cm) focal area of
Capital Medical University,
Beijing 100050, China demyelination associated with mass effect can mimic brain
neoplasm or abscess. This disease have been called
S.-w. Li : J. Wu “demyelinating pseudotumors,” “tumor-like demyelinating
Image Center of Neuroscience, Beijing Tiantan Hospital,
lesions (TDLs),” “tumefactive demyelinating lesions,” and
Capital Medical University,
Beijing 100050, China “tumefactive multiple sclerosis lesions” in literature. Be-
cause of the tendency to mimic tumor or abscess clinically,
S. Lin (*) radiologically, and even pathologically and because of
Beijing Neurosurgical Institute, Beijing Tiantan Hospital,
important differences in treatment, it is critical to differen-
Tiantan Xi li No. 6,
Beijing 100050, China tiate among them in diagnosis. In this study, nine cases
e-mail: linsong2005@126.com pathologically diagnosed as having TDLs are presented. By
172 Neurosurg Rev (2009) 32:171–179

reviewing related reports, clinical, radiological, and patho- Location and number of lesions (by CT/MRI scan)
logical characteristics of a TDL diagnosis are summarized.
Of the six intracephalic lesions, four were in cerebral
hemisphere; one was in pons and one was in the basal
ganglia. All three intraspinal lesions were found in the
Materials and methods
cervical cord and involved multiple segments. Two addi-
tional intracephalic lesions were found in two cases on MRI
The criteria for patient enrollment included clinical
scans: one was a left frontoparietal mass (accompanying
presentation mimicking neoplasm and biopsy confirming
lesions in left frontal lobe, corpus callosum, and cerebellar
the TDLs. After excluding one case of astrocytoblastoma
hemisphere); the other was a left brachium pontis mass
at repeat pathological investigation, nine patients were
accompanying a lesion in left frontal lobe.
included at Tiantan hospital (Beijing, China) from Jan.
2005 to Mar. 2007 (Table 1). Routine admission examina- Lesion appearance on imaging
tions, including physical examination, laboratory investiga- All lesions appeared as hypodensities on CT, hypointen-
tion, electrocardiogram, and X-ray, were performed in all sities on T1-weighted MRI, and hyperintensities on T2-
cases. Preoperative scans (computed tomography (CT) or weighted MRI. The lesions all occurred together with
magnetic resonance imaging (MRI)) were examined. All perifocal edema. The edema was remarkable in the
patients underwent operations to biopsy or remove the lesions. cases that had shorter histories (less than 3 weeks).
In some cases, intraoperative frozen sections were investigat- Also, there were marked mass effects in the acute cases.
ed. TDLs were confirmed by hematoxylin and eosin (H&E) or In all cases with intraspinal lesions, spinal cord
immunohistochemical staining with glial fibrillary acidic swellings were found on MRI. The intracephalic lesions
protein, neurofilament, synaptophysin, myelin basic protein, displayed patchy enhancement in the children, ring
N-ethylmaleimide sensitive fusion protein, Vimtin, CD68, enhancement in three adults, and nodular enhancement
and CD34 in all cases. Some patients also received medical in one adult (Figs. 1, 2, 3, and 4). The intraspinal lesions
treatment and postoperative follow-up scans. were all irregularly enhanced (Figs. 5 and 6). Decreased
relative cerebral blood volume (rCBV) and prolonged
mean transit time (MTT) were seen in one intracephalic
Results lesion (Fig. 4).

Clinical features were as follows:

Preoperative diagnosis
Age
The age of the patients at onset ranged from 4 to 65 years. Eight were preoperatively diagnosed as gliomas and one
Two patients were children aged 4 and 11 years. Three was metastasis.
patients were in their 30s. Two patients were older than 40.
The other patient was 65 years old. Intraoperative findings

Sex Of the nine total lesions, five appeared grayish red; three
were grayish yellow in three patients and one was
Four of the patients were female. Of the six patients with yellowish green (with a 1.5-cm cyst and yellowish-green
intracephalic lesions, three were females. Of the three fluid). Eight of the lesions were soft and one was tough.
patients with intraspinal lesions, two were male. Eight of the lesions had obscure boundaries, while one had
Duration from onset to admission a distinct boundary. Six lesions appeared to be moderately
vascularized; two were well vascularized and one had
The duration from onset of symptoms to admission to minimal vascularity. Intracephalic lesions were completely
hospital ranged from 1 week to about 1 year; it was less resected in one patient, almost completely resected in
than 10 days for both children and was about 1 to 2 months another, mostly resected in three patients, and biopsied
in five of the adults. only in the case of one patient with a pontile lesion.
Chief symptoms and signs Intraspinal lesions were partly resected in two patients and
biopsied in one patient.
Headache and vomiting occurred only in the two children.
Results of intraoperative frozen sectioning and postoperative
Six of the patients presented with weakness in the extremities,
paraffin sectioning
and two patients had epilepsy. General numbness/loss of
sensation was only found in the three patients with intraspinal Five lesions underwent intraoperative frozen sectioning.
lesions. One patient complained of decreased visual acuity. Two of these lesions were gliosis; one was an astrocytoma;
Table 1 Summary of patients

Patient Age Sex Onset of clinical Neuroimaging features Intraoperative findings Intraoperative Postoperative Medical treatment and follow-up
symptoms and signs frozen events
sections

1 4 Male Headache and Well-defined right frontotemporal mass, Gray red, soft, fish-like, well Astrocytoma Fever for Receiving steroid and transported to pediatric
vomiting for hypodensity and hypointense T1 vascularity, obscure 8 days, department, doing well after steroids, the
1 week, hemiparesis hyperintense T2, vasogenic edema, marked boundary, largely resection hemiparesis lesion disappeared on MRI 22 months later
in left lower mass effect, patchy enhancement resolved
extremity for 2 days
2 11 Female Headache and Poorly defined left frontoparietal mass, Yellow green, soft, obscure Gliosis Visual acuity Transported to pediatric department and
Neurosurg Rev (2009) 32:171–179

vomiting for hypodensity, vasogenic edema, marked mass boundary, moderate progressively receiving steroid, doing well and visual acuity
10 days, visual effect, patching enhancement; left frontal, vascularity, with a 1.5-cm decreasing and decreasing resolved after steroids, remnant
acuity decreasing at corpus callosum and cerebellar hemisphere cyst with yellow green diagnosed as lesion shrunk on MRI 12 months later
the fifth day after abnormal enhancement fluid; largely resection optic neuritis
admission
3 27 Female Dizzy and ataxia, Poorly defined left brachium pontis lesion, Gray, soft, obscure Cerebellar Fever for Facial palsy maintained as usual
aphagia left facial hypointense T1 hyperintense T2, vasogenic boundary, moderate tissue 10 days
palsy for 5 years edema, minimal mass effect, irregular ring vascularity, biopsy
enhancement; left frontal lesion
(demyelination)
4 32 Female Right lower extremity Well-defined left parieto-occipital round Part gray red and part light Tumor tissue Fever for 6 days Doing well, lesion disappeared on MRI
weakness for lesion, hypointense T1 hyperintense T2, yellow, soft, obscure and 8 months later
20 days and seizure vasogenic edema, marked mass effect, ring boundary, moderate hemorrhagic
once enhancement vascularity, near total necrosis
resection
5 45 Male General seizure once Poorly defined right frontoparietal lesion, mix Dark yellow, soft, obscure The left upper Transport to neurological department to receive
2 weeks ago, left intense, marked vasogenic edema, marked boundary, well vascularity, extremity steroids for l month; the muscle strength
extremities mass effect, irregular nodular enhancement, total resection paralyzed, the recovered to grade 5, the lesion disappeared
weakness rCBV decreasing muscle on MRI 14 months later
strength was
grade 1
6 42 Female Left hemianesthesia Poorly defined C3–4 lesion, hypointense T1 Gray yellow, soft, distinct Gliosis General seizure Discharging and receiving antiepileptic
and right upper hyperintense T2, spinal swelling, moderate boundary, moderate one time treatment, no neurological deficit and the
extremity weakness patching enhancement vascularity, partly resection lesion disappeared on MRI 12 months later
for 20 days
7 44 Male Upper extremity Poorly defined C2–6 lesion, hypointense T1 Gray red, soft, obscure Fever for 7 days Receiving steroid and vitamin B1, B12, and
anesthesia for hyperintense T2, spinal swelling, moderate boundary, moderate transported to neurological department for
2 months and lower mixed strip-like enhancement vascularity, partly resection steroid treatment, anesthesia persists, no other
extremity for neurological deficit, the lesion disappear on
20 days MRI 32 months later
8 65 Male Lower extremity Poorly defined C1–7 lesion, hypointense T1 Gray, firm, minimal Smooth Receiving steroid and vitamin B1, B12; the
paraplegia and hyperintense T2, spinal swelling, irregular vascularity, obscure patient aggravated to quadriplegia for 2 years,
anesthesia for strip-like enhancement boundary, biopsy and the relatives gave up positive treatment
12 months
9 35 Male Right extremities Poorly defined left basal ganglia lesion, Gray red, soft, moderate Smooth Transported to neurological department and
weakness for hypointense T1 hyperintense T2, irregular vascularity, obscure doing well after steroids, died from
1 month open-ring enhancement boundary, largely resection pneumonia 1 year later
173
174 Neurosurg Rev (2009) 32:171–179

Fig. 1 a, b CT scan showed left

parietal lobe hypodense lesion
with perifocal edema (a). After
contrasting, the lesion displayed
open-ring enhancement

another was cerebellar tissue and the last one was tumor Follow-up
tissue with hemorrhagic necrosis.
Eight patients underwent follow-up examination up to
Postoperative paraffin pathological findings showed the
32 months following the surgical procedure (mean =
diffuse presence of infiltrating macrophages with finely
17 months). The lesion disappeared on MRI later in five
vacuolated cytoplasms. A final diagnosis of TDLs was
cases and shrank in one case. Overall, Six cases received
rendered in all cases (Fig. 7).
postoperative steroid therapy. Five patients responded well
to steroids and one progressed to quadriplegia.
Postoperative course

Five cases presented with postoperative fever which lasted Discussion

for 5–10 days. One patient presented with progressively
decreasing visual acuity, which was diagnosed as optic TDLs are rare disease of the CNS. It has been documented that
neuritis and was treated with steroid therapy. (Visual acuity TDLs are most commonly seen in the initial background of MS
remarkably improved 10 days later.) One patient had a [4, 9, 11, 14, 20, 23, 28, 30, 36, 37, 40, 45]. Other primary
general seizure and was given an antiepileptic agent. One demyelinating diseases, such as myelinoclastic diffuse sclero-
patient suffered from facial palsy and another’s hemi- sis (Schilder’s disease) [1, 44] and acute disseminated
paralysis was relieved. encephalomyelitis [26], can also manifest as tumefactive

Fig. 2 a, b Postcontrast T1-

weighted image showed left
basal ganglia lesion with
irregular open-ring enhancement
Neurosurg Rev (2009) 32:171–179 175

Fig. 3 MRI showed that the lesion was in the left brachium pontis and hypointense in T1 (a), hyperintense in T2 (b), with irregular ring
enhancement (c)

lesions. It has been suggested that TDLs may represent either the involvement of an autoimmune mechanism [26]. The
a variant of MS [38] or a unique form of isolated clinical, radiological, and pathological presentations of TDLs
demyelinating disease [26]. Some patients were found to have resemble brain tumors, leading to unnecessary biopsy, surgery,
developmentally immature myelin basic protein, pointing to a or even radiotherapy which usually causes aggravation.
genetic predisposition to more severe demyelination [46]. TDLs have no specific clinical presentation and lesions
Others have antecedent vaccination or infection, suggesting can occur at any age (a range of 2 to 71 years has been

Fig. 4 MRI T2-weighted image

showed the right parietal hyper-
intense lesion with perifocal
edema (a). Postcontrast T1-
weighted image showed that the
lesion displayed heterogeneous
nodular enhancement (b).
Perfusion-weighted image
showed decreased rCBV (c) and
prolonged MTT (d) within
lesions and perifocal tissue as
compared with that within the
contralateral normal-appearing
white matter
176 Neurosurg Rev (2009) 32:171–179

Fig. 5 MRI showed that the lesion was in the cervical cord and hypointense in T1 (a), hyperintense in T2 (b), with irregular enhancement (c)

reported [26, 34, 48]). A history of relapsing–remitting MS or pons. In cases with spinal lesions, multiple cervical
symptoms or preceding vaccination or viral infection may segments appear to be most often involved [5, 6, 18, 29].
provide the diagnostic clue of demyelinating diseases. Indeed, in this study, the lesions of all three intraspinal
TDLs have a relatively acute onset (mostly less than cases were confined in cervical segments. This observation
3 weeks in the present study) with a progressive aggrava- is helpful in diagnosis. The presence of other demyelinating
tion in symptoms. Onset time for children is typically changes in the CNS, especially in periventricular areas or
within 1 week [34, 48]. In children, symptoms are acute spinal cord, may be indicative of further demyelinating
and severe and usually include headache and vomiting lesions [12, 34]. In two cases in this study, multiple lesions
resulting from intracranial hypertension [34, 48]. Moreover, were found in the frontal lobe, corpus callosum, and
it is notable that pediatric cases frequently present with cerebellum on MRI. An additional characteristic of TDLs
symptoms of optic neuritis, such as visual acuity and field is their relative lack of mass effect and less substantial
changes [2, 34]. In this study, one child developed perifocal edema compared to brain neoplasm [12]. The
decreased visual acuity 1 week after admission. Having pattern of enhancement in the lesions is an important
gone unnoticed, the child’s visual acuity decreased abruptly identifying feature. It consists of “open-ring” enhancement,
thereafter, resulting in an examination of the optic fundi and as compared to the complete ring of enhancement seen in
a diagnosis of optic neuritis. abscesses and tumors. The open-ring sign was present in
There are several important radiological hallmarks that 70% of cases with demyelinating disease [33]. In some
favor the detection of demyelination. TDLs are mainly cases, the open portion of the partial ring abuts the cortical
localized in the subcortical hemispheric white matter [12, gray matter or basal ganglia and the area of enhancement
26] and may also be found in the corpus callosum [25, 39] sweeps through the white matter [32]. The area of

Fig. 6 MRI showed another intraspinal lesion which involved multiple cervical segments and caused spinal swelling
Neurosurg Rev (2009) 32:171–179 177

these lesions. If serial proton MRS shows no obvious

changes, which is inconsistent with the course of acute
demyelinating plaque, it supports the diagnosis of neo-
plasms [7]. Decreased magnetization transfer value (MTV)
has been shown in MS plaques. A similar decline in MTV
within TDLs was also observed [17], with MTV decline
around or below 30% in the chronic TDLs [15, 35].
Positron emission tomography shows a low or no uptake of
glucose, methionine, or choline [27] and a marked increase
in the uptake of tyrosine, which is nonspecific [19].
Diagnostic therapy is critical for the final diagnosis of
TDLs. In whom the diagnosis of TDLs is suspected, repeat
MRI may be performed during or shortly after completion of
corticosteroid therapy [34]. Although perifocal edema asso-
Fig. 7 H&E staining showed the diffuse presence of infiltrating ciated with tumor or abscess may respond to corticosteroids,
macrophages with finely vacuolated cytoplasm. Original magnifica- a rapid reduction in the size of the lesion associated with
tion ×400 significant clinical improvement favors demyelination.
Noteworthy is the fact that MS can coexist with gliomas
enhancement is regarded as the border of demyelination and lymphomas [13, 42]. The concurrence of MS and
and therefore localized at the site of whiter matter [22], gliomas could be purely coincidental or the result of
while the nonenhancing center of the lesion represents neoplastic transformation of reactive glial cells in the areas
chronic inflammation. of demyelination. A nonenhancing tumefactive lesion in a
Some advanced radiological techniques have been patient with MS is highly suspicious for the presence of
applied to TDLs. It was noted from several MRI perfusion low-grade glioma since TDLs typically display contrast
studies that dilated veins drain toward distended subepen- enhancement. Some cases of TDLs preceding the appear-
dymal veins, running centrally within TDLs on T2 images ance of primary cerebral lymphoma have been reported [3].
[8]. It was reported that the mean rCBV within TDLs was Many MS patients receive corticosteroids and other
lower than that within the contralateral normal-appearing immunosuppressant medications and develop an immuno-
white matter and substantially less than that found in high- compromised state; this might contribute to the occurrence
grade gliomas [8, 17]. In this study, decreased rCBV was of a primary CNS lymphoma [47] and an elevated risk of
found in a case which was misdiagnosed as glioma. infection and abscess [34].
Therefore, decreased rCBV is an important clue. Apparent Overall, when differentiation cannot be confidently
diffusion coefficients within TDLs are mildly increased, established, a biopsy is advisable—especially paraffin-
which is helpful in differentiating ring-enhancing TDLs embedded tissue sections followed by staining. However,
from cerebral abscesses—the latter being associated with the histologic features observed on intraoperative frozen-
restricted diffusion centrally within the lesions. Neoplasms section preparations can be deceptive and difficult to
with central necrosis may also display increased diffusion interpret, especially when neoplasms have been previously
coefficients centrally within the lesion, which make it less diagnosed by neurosurgeons or radiologists. As seen in this
helpful in differentiating from neoplasms [21]. Proton study, no frozen sections suggested a diagnosis of TDLs.
magnetic resonance spectroscopy (MRS) is a powerful The histologic tableau consisting of astrocytosis, atypical-
noninvasive tool. Although several nonneoplastic brain appearing reactive astrocytes with mitotic figures, and
lesions (including TDLs) may produce an magnetic lymphocytic infiltrates can closely mimic gliomas. The
resonance spectrum mimicking a neoplastic process [17, key to avoiding misdiagnosis of demyelinating disease is to
41], the mean central-region NAA to Cr ratio in gliomas identify the macrophage infiltrate. But macrophages in
was significantly lower than that of TDLs [41]. Also, the brain tissue are frequently mistaken for infiltrating neoplas-
size of the glutamate/glutamine peaks, which are typically tic astrocytes or oligodendroglia. The immunohistochemi-
not seen in aggressive intra-axial neoplastic processes, may cal staining for histiocytes (anti-HAM 56 and CD68
be useful [10]. It should be mentioned, however, that MRS antibody) is helpful [16].
findings in patients with TDLs vary with disease stage. Most patients with TDLs usually respond well to high
Acute demyelinating plaques show an MRS spectrum doses of corticosteroids [26], especially children (as seen in
similar to that of low-grade gliomas, with reduction in this study and others), while a poor response to such
NAA and an increase in choline. Therefore, the use of serial therapy occurs mainly in adults when associated with MS.
proton MRS has proven to be beneficial in distinguishing It has been reported that these steroid-unresponsive cases
178 Neurosurg Rev (2009) 32:171–179

can be successfully treated with plasma exchange and 12. Dagher AP, Smirniotopoulos J (1996) Tumefactive demyelinating
lesions. Neuroradiology 38:560–565
mitoxantrone [24, 31].
13. De LA, Gomez PA, Boto GR, Lagares A, Ricoy JR, Alen JF,
Most patients with TDLs have a single acute clinical Lobato RD (2004) Oligodendroglioma and multiple sclerosis. A
attack and generally do not undergo recurrence. However, case report. Neurocirugia 15:378–383
some of these patients may evolve to MS [43]. 14. Di PP, Castillo V, Delavelle J, Vuillemoz S, Picard F, Landis T
(2003) “Tumor-mimicking” multiple sclerosis. Clin Neuropathol
22:235–239
Conclusions 15. Enzinger C, Strasser-fuchs S, Ropele S, Kapeller P, Kleinert R,
Fazekas F (2005) Tumefactive demyelinating lesions: conventional
The diagnosis of TDLs is a challenge for neurosurgeons, and advanced magnetic resonance imaging. Mult Scler 11:135–139
16. Erana-rojas IE, Barboza-quintana A, Ayala AG, Fuller GN (2002)
neuroradiologists, and neuropathologists. First of all, neuro- Demyelinating pseudotumor. Ann Diagn Pathol 6:265–271
surgeons should be aware of the existence of nonneoplastic 17. Ernst T, Chang L, Walot I, Huff K (1998) Physiologic MRI of a
masses in CNS and should be rid of traditional ideas, which tumefactive multiple sclerosis lesion. Neurology 51:1486–1488
may help to avoid the thought processes leading to 18. Fazekas F, Barkhof F, Filippi M, Grossman RI, Li DK, Mcdonald
WI, Mcfarland HF, Paty DW, Simon JH, Wolinsky JS, Miller DH
misdiagnosis. Comprehensive information from the (1999) The contribution of magnetic resonance imaging to the
patient’s history and results of steroid therapy may provide diagnosis of multiple sclerosis. Neurology 53:448–456
clues for the final diagnosis. Open-ring enhancement is 19. Floeth FW, Pauleit D, Sabel M, Reifenberger G, Stoffels G,
relatively characteristic and mild mass effect and perifocal Stummer W, Rommel F, Hamacher K, Langen KJ (2006) F-18-
FET PET differentiation of ring-enhancing brain lesions. J Nucl
edema is also useful. Decreased rCBV in the lesion should Med 47:776–782
be noticed and emphasized. While it is advisable to observe 20. Friedman DI (2000) Multiple sclerosis simulating a mass lesion. J
suspected cases dynamically by serial MRS, pathological Neuro-Ophthalmol 20:147–153
data are still beneficial to the final diagnosis in difficult 21. Given CA, Stevens BS, Lee C (2004) The MRI appearance of
tumefactive demyelinating lesions. Am J Roentgenol 182:195–199
cases, especially paraffin sections and special staining. 22. He J, Grossman RI, Ge Y, Mannon LJ (2001) Enhancing patterns
in multiple sclerosis: evolution and persistence. AJNR Am J
Neuroradiol 22:664–669
References
23. Iwamoto K, Oka H, Utsuki S, Ozawa T, Fujii K (2004) Late-onset
multiple sclerosis mimicking brain tumor: a case report. Brain
1. Afifi AK, Bell WE, Menezes AH, Moore SA (1994) Myelino- Tumor Pathol 21:83–86
clastic diffuse sclerosis (Schilder’s-disease)—report of a case and 24. Jaffe SL, Minagar A (2005) Demyelinating pseudotumor. Arch
review of the literature. J Child Neurol 9:398–403 Neurol 62:1466–1467
2. Akiyama H, Yanagisawa A, Yamamoto H, Nagashima T, 25. Kalyan-raman UP, Garwacki DJ, Elwood PW (1987) Demyelin-
Maruyama A, Souma O, Yoshida M (2005) A pediatric case of ating disease of corpus callosum presenting as glioma on magnetic
multiple sclerosis mimicking malignant brain tumor. No Shinkei resonance scan: a case documented with pathological findings.
Geka 33:1007–1012 Neurosurgery 21:247–250
3. Alarcia R, Ara JR, Marta E, Barrena MR, Gimenez-mas JA, 26. Kepes JJ (1993) Large focal tumor-like demyelinating lesions of
Capablo JL, Serrano M (2000) Demyelinating pseudotumoral lesion the brain: intermediate entity between multiple sclerosis and acute
prior to a primary cerebral lymphoma. Rev Neurol 31:955–958 disseminated encephalomyelitis? A study of 31 patients. Ann
4. Braverman DL, Lachmann EA, Tunkel R, Nagler W (1997) Neurol 33:18–27
Multiple sclerosis presenting as a spinal cord tumor. Arch Phys 27. Kwee SA, Coel MN, Lim J, Ko JP (2004) Combined use of F-18
Med Rehabil 78:1274–1276 fluorocholine positron emission tomography and magnetic resonance
5. Brinar M, Rados M, Habek M, Poser CM (2006) Enlargement of spectroscopy for brain tumor evaluation. J Neuroimaging 14:285–289
the spinal cord: inflammation or neoplasm? Clin Neurol Neurosur 28. Lolekha P, Kulkantrakorn K (2006) Tumor-like manifestation,
108:284–289 uncommon form of multiple sclerosis: report of a patient. J Med
6. Brinar VV (2004) Non-MS recurrent demyelinating diseases. Clin Assoc Thai 89:721–726
Neurol Neurosurg 106:197–210 29. Lycklama G, Thompson A, Filippi M, Miller D, Polman C,
7. Butteriss DJ, Ismail A, Ellison DW, Birchall D (2003) Use of Fazekas F, Barkhof F (2003) Spinal-cord MRI in multiple
serial proton magnetic resonance spectroscopy to differentiate low sclerosis. Lancet Neurol 2:555–562
grade glioma from tumefactive plaque in a patient with multiple 30. Maezawa H, Takano M, Nagai S, Iida H, Tachibana S (1995)
sclerosis. Br J Radiol 76:662–665 Spinal multiple sclerosis mimicking a spinal cord tumor: a case
8. Cha S, Pierce S, Knopp EA, Johnson G, Yang C, Ton A, Litt AW, report. No Shinkei Geka 23:1007–1010
Zagzag D (2001) Dynamic contrast-enhanced T2*-weighted MR 31. Mao-draayer Y, Braff S, Pendlebury W, Panitch H (2002)
imaging of tumefactive demyelinating lesions. AJNR Am J Treatment of steroid-unresponsive tumefactive demyelinating
Neuroradiol 22:1109–1116 disease with plasma exchange. Neurology 59:1074–1077
9. Checrallah A, Geha S, Rizk T, Koussa S (2005) Multiple sclerosis 32. Masdeu JC, Quinto C, Olivera C, Leslie D, Tenner M, Visintainer
mimicking brain tumor: an unusual presentation. J Med Liban P (1998) The open ring or white-matter-crescent pattern of
53:45–49 enhancement differentiates demyelinating brain disease with
10. Cianfoni A, Niku S, Imbesi SG (2007) Metabolite findings in tumor-like presentation from infection and neoplasm. Riv Neuro-
tumefactive demyelinating lesions utilizing short echo time proton radiol 11:15–16
magnetic resonance spectroscopy. Am J Neuroradiol 28:272–277 33. Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer
11. Comi G (2004) Multiple sclerosis: pseudotumoral forms. Neurol P (2000) Open-ring imaging sign—highly specific for atypical
Sci 25(Suppl 4):374–379 brain demyelination. Neurology 54:1427–1433
Neurosurg Rev (2009) 32:171–179 179

34. Mcadam LC, Blaser SI, Banwell BL (2002) Pediatric tumefactive appropriately managed simply by medication. Actually, unnecessary
demyelination: case series and review of the literature. Pediatr biopsy or even resective surgery has been applied by the authors based
Neurol 26:18–25 on preoperative misdiagnosis, although pathologic diagnosis may be
35. Metafratzi Z, Argyropoulou MI, Tzoufi M, Papadopoulou Z, required only on limited occasions. Considering the clinical similar-
Efremidis SC (2002) Conventional MRI and magnetisation ities, utmost care should be taken interpreting the neuroradiologic data
transfer imaging of tumour-like multiple sclerosis in a child. to differentiate neoplastic conditions from this kind of nonneoplastic
Neuroradiology 44:97–99 inflammatory disease. Further investigation using advanced magnetic
36. Meurice A, Flandroy P, Dondelinger RF, Reznik M (1994) A resonance (MR) techniques such as MR diffusion and perfusion
single focus of probable multiple sclerosis in the cervical spinal sequences and, especially, MR spectroscopy together with analysis of
cord mimicking a tumour. Neuroradiology 36:234–235 the cerebrospinal fluid may obviate the need for brain biopsy in many
37. Pakos EE, Tsekeris PG, Chatzidimou K, Goussia AC, Markoula S, circumstances.
Argyropoulou MI, Pitouli EG, Konitsiotis S (2005) Astrocytoma-
like multiple sclerosis. Clin Neurol Neurosurg 107:152–157 Jin-song Wu, Liang-fu Zhou, Shanghai, China
38. Poser S, Luer W, Bruhn H, Frahm J, Bruck Y, Felgenhauer K Primary central nervous system (CNS) demyelinating lesions,
(1992) Acute demyelinating disease. Classification and non- including multiple sclerosis, are a group of disorders with unknown
invasive diagnosis. Acta Neurol Scand 86:579–585 etiology. Classical CNS primary demyelination is often made on
39. Rieth KG, Di CG, Cromwell LD, Mckeever PE, Kornblith PL, clinical ground. Histological diagnosis is uncommon except when
Kufta CV, Pleet AB (1981) Primary demyelinating disease atypical clinical or imaging features are present. Tumefactive
simulating glioma of the corpus callosum: report of three cases. demyelinating lesions (TDLs) pose a diagnostic challenge as they
J Neurosurg 55:620–624 simulate neoplasm. Dr. Xia et al. reported nine cases of tumefactive
40. Rusin JA, Vezina LG, Chadduck WM, Chandra RS (1995) demyelinating lesions mimicking CNS malignant neoplasm and a nice
Tumoral multiple sclerosis of the cerebellum in a child. AJNR review of the literature. When a single large or multiple small
Am J Neuroradiol 16:1164–1166 tumefactive demyelinating lesions are present, the correct presurgical
41. Saindane AM, Cha S, Law M, Xue X, Knopp EA, Zagzag D diagnosis is often difficult, as the MRI appearance, comprising
(2002) Proton MR spectroscopy of tumefactive demyelinating enhanced T1-weighted images, T2-weighted images, and even proton
lesions. AJNR Am J Neuroradiol 23:1378–1386 MR spectroscopy, is not specific for these lesions. The diffusion
42. Sega S, Horvat A, Popovic M (2006) Anaplastic oligodendro- imaging of MRI was proven to be a useful tool in differentiating ring-
glioma and gliomatosis type 2 in interferon-beta treated multiple enhancing tumefactive demyelinating lesions from cerebral abscesses,
sclerosis patients—report of two cases. Clin Neurol Neurosur while the perfusion imaging of MRI was reported to be showing
108:259–265 decreased perfusion within tumefactive demyelinating lesions, as
43. Selkirk SM, Shi J (2005) Relapsing–remitting tumefactive compared with high-grade gliomas and lymphomas (1). However,
multiple sclerosis. Mult Scler 11:731–734 these imaging features are not specific, given that the degree of
44. Stachniak JB, Mickle JP, Ellis T, Quisling R, Rojiani AM (1995) overlap is most likely with other pathological processes. Therefore, an
Myelinoclastic diffuse sclerosis presenting as a mass lesion in a image-guided biopsy is recommended for suspected patients. The
child with Turner’s syndrome. Pediatr Neurosurg 22:266–269 initial frozen-section specimen should be sent to neuropathologist. In
45. Tilbery CP, Guidugli-neto J (1995) Multiple sclerosis simulating our opinion, it is still feasible for the experienced neuropathologist to
cerebral tumor: report of a case with histopathological confirma- differentiate this lesion from malignant gliomas. In short, the neuro-
tion. Arq Neuropsiquiatr 53:302–306 surgeons should be aware of the broad and heterogeneous spectrums
46. Wood DD, Bilbao JM, O’Connors P, Moscarello MA (1996) of TDLs in clinical, imaging, and pathological manifestations. The
Acute multiple sclerosis (Marburg type) is associated with exact perioperative diagnosis is essential to avoid unnecessary
developmentally immature myelin basic protein. Ann Neurol resection and improper adjunctive therapy.
40:18–24
47. Yang JH, Wu SL (2007) Multiple sclerosis preceding CNS References
lymphoma: a case report. Acta Neurol Taiwan 16:92–97 1. Given CA 2nd, Stevens BS, Lee C (2004) The MRI appearance
48. Yapici Z, Eraksoy M (2002) Bilateral demyelinating tumefactive of tumefactive demyelinating lesions. AJR Am J Roentgenol
lesions in three children with hemiparesis. J Child Neurol 17:655–660 182:195–199

Takamasa Kayama, Yamagata, Japan

The authors described a pathologically proven series of tumefac-
Comments tive demyelinating lesions with nine cases. It is important that
clinician be of aware demyelinating lesions mimicking brain neo-
Chang Jin Kim, Seoul, Korea plasm. Actually, radiological findings including rCBV are very useful,
The authors reported on a pathologically proven series of but they are not able to replace pathological diagnosis so far. It is
tumefactive demyelinating lesions with nine cases, and conducted a noteworthy that intraoperative frozen section did not confirm the
comprehensive review on clinical, radiological, and therapeutic diagnosis. We must avoid unnecessary respective surgery. This report
consideration. Awareness of demyelinating lesions mimicking brain makes an important contribution for the differential diagnosis and the
neoplasm is important for neurosurgeons in that they can be preoperative planning of the lesions mimicking brain neoplasm.