The transformation of pediatric gliomatosis cerebri to cerebellar glioblastoma multiforme presenting as supraand infratentorial acute disseminated encephalomyelitis. Case report

Article (PDF Available)inJournal of Neurosurgery 102(1 Suppl):72-7 · February 2005with27 Reads
DOI: 10.3171/ped.2005.102.1.0072 · Source: PubMed
Cerebellar glioblastoma multiforme (GBM) is a rare entity in adults and an extremely rare entity in children. Approximately 30 cases have been reported in the literature. The authors report the case of a histologically confirmed cerebellar GBM presenting initially as supra- and infratentorial gliomatosis cerebri. Acute disseminated encephalomyelitis had been diagnosed in the patient and that diagnosis remained until near the end of his treatment. This case underscores the need for recognizing the clinical presentation of gliomatosis cerebri and multifocal GBM in the pediatric subpopulation thought to harbor demyelinating disease.
E present the case of a 5-year-old boy who pre-
sented with the history
, signs, and symptoms of
acute disseminated encephalomyelitis. The initial
neuroimaging studies seemed to support this diagnosis and
the child was treated accordingly
. Later a lar
ge enhancing
lesion was found on intracranial Gd-enhanced MR imaging
and following biopsy the diagnosis was changed to GBM.
Case Repor
This previously healthy 5-year
-old boy was ad
mitted to the hospital with a medical history of mild reac-
tive airway disease and chronic constipation. For 4 days
preceding his admission he had displayed decreasing appe
tite and lethargy. he patient had not suffered any recent
febrile illness. He had experienced upper respiratory infec-
tions without sequelae during the previous month, and his
mother noted a rash on his right thigh, which was diag-
nosed as nummular eczma. The patient’s father had been ill
with a headachelike illness 2 weeks prior to the child’s ad-
wo days before admission, the patient vomited twice
and became increasingly lethar
gic. The patient’
s primary
care physician diagnosed strep throat and placed the boy
on an amoxicillin regimen. The day before admission, the
patient refused to walk downstairs, vomited twice, and ex-
perienced a fever of 101˚F
. The morning of admission the
patient was again febrile and vomited twice; his mother
observed left facial weakness. He was taken to his prima-
ry care physician who noted ataxia, left facial droop, and
dizziness when lying down.
Examination, Initial Diagnosis, and T
The pa
tient was brought to the hospital and was observed to be
suffering from a fever of 101˚F. He was somnolent but
arousable and presented with a left facial droop, nystagmus,
and ataxic gait. Magnetic resonance imaging of the brain re
vealed cerebral edema associated with abnormal deep white
matter signal changes that extended through the cerebral
peduncles, more so on the left side, with pontine and cere-
bellar involvement.
The patient was admitted to the hospital, and ADEM
J Neurosurg (Pediatrics 1) 102:72–77, 2005
The transformation of pediatric gliomatosis cerebri to
cerebellar glioblastoma multiforme presenting as supra-
and infratentorial acute disseminated encephalomyelitis
Case report
Departments of Neurological Surgery, Neuropathology, and Neuroradiology, Columbia College of
Physicians and Surgeons; Neurological Institute of New York, Columbia University Medical Center,
New York, New York; and Department of Neurological Surgery, The Johns Hopkins School of Medicine,
Baltimore, Maryland
Cerebellar glioblastoma multiforme (GBM) is a rare entity in adults and an extremely rare entity in children.
Approximately 30 cases have been reported in the literature. The authors report the case of a histologically confirmed cere-
bellar GBM presenting initially as supra- and infratentorial gliomatosis cerebri. Acute disseminated encephalomyelitis had
been diagnosed in the patient and that diagnosis remained until near the end of his treatment. This case underscores the
need for recognizing the clinical presentation of gliomatosis cerebri and multifocal GBM in the pediatric subpopulation
thought to harbor demyelinating disease.
KEY WORDS cerebellar glioblastoma multiforme gliomatosis cerebri
acute disseminated encephalomyelitis pediatric neurosurgery
J. Neurosurg. (Pediatrics 1) / Volume 102 / January, 2005
Abbreviations used in this paper: ADEM = acute dissemin-
ated encephalomyelitis; FLAIR = fluid-attenuated inversion-
recovery; GBM = glioblastoma multiforme; Ig = immunoglobulin;
MR = magnetic resonance.
Janpeds2005 12/17/04 9:04 AM Page 72
was diagnosed. He was started on a regimen of ceftriax-
one, acyclovir, and a 10-day course of intravenous deca-
dron (25 mg daily). Cerebrospinal fluid and blood serolo-
gical findings were normal. Repeated MR imaging studies
performed 5 days after admission demonstrated no change
(Fig. 1). The patient was discharged 12 days after admis-
sion, the dosage of steroid medications was slowly ta-
pered, and he demonstrated modestly improved neurolog-
ical status.
Repeated Presentations, Examinations, and Final Diag-
One month later, the patient was readmitted with a
3-week history of intermittent left-sided hearing loss, numb-
ness and tremor of the left arm, and the inability to look to
the left side. The patient responded well to a short course of
intravenous steroid agents and was discharged on a regimen
of oral prednisone therapy
. No MR imaging studies were
performed during that hospital stay.
Three weeks later, the patient suffered a second exacer-
bation of his symptoms, including dysarthria, left-sided
ataxia, diplopia, left-sided hearing loss, and dysphagia. He
was admitted for a course of 2 g/kg intravenous Ig after
which he improved modestly and was discharged. Once
again, MR images were not obtained.
One week later, he developed seizurelike symptoms con-
sisting of multiple episodes of left arm jerking associated
with a loss of consciousness, head turning to the left side,
and a right hemiparesis. Computerized tomography scans
of the head obtained at admission and subsequent MR im
aging studies demonstrated cerebellar patchy enhancement
and abnormally enhancing foci bilaterally in the frontal and
parietal subcortical white matter. Single-voxel MR spec-
troscopy was performed but the findings were nonspecific.
Electroencephalography demonstrated brief electrographic
seizures with left temporal origin and spread to the neigh-
boring cortex. The patient was treated with Cerebyx,
Cellcept, and steroid agents, which resulted in resolution of
seizure activity but no improvement in the encephalopathy
facial nerve palsy, or right hemiparesis. He became increas-
ingly lethargic and paretic despite continued therapy and
eventually required transfer to the intensive care unit.
Gadolinium-enhanced MR imaging performed 1 month
later revealed a large enhancing lesion in the left cerebellar
peduncle, vermis, and medulla oblongata. Effacement of
the fourth ventricle and obstructive hydrocephalus without
evidence of supratentorial enhancement were also demon-
strated (Fig. 2C and D). Axial FLAIR imaging document-
ed persistent, stable patches of supratentorial hyperintensi-
ty (data not shown). There was no evidence of abnormal
leptomeningeal enhancement, either linear or nodular, to
indicate a spread of disease in this manner. A neurosurgical
consultation was requested and biopsy was recommended.
Subsequent suboccipital craniectomy for biopsy and con-
current endoscopic third ventriculostomy were performed.
Frozen and permanent tissue samples confirmed the diag-
nosis of GBM (Fig. 3). A conversation among the physi-
cians and family concluded with the decision not to pursue
aggressive measures. The patient died shortly thereafter.
This patient suffered from an aggressive subacute disease
process that evolved inexorably in a period of 3 months.
During that time, the patient exhibited signs and symptoms
resembling those associated with supra- and infratentorial
lesions, including decreased mental status, seizures, ataxia,
and cranial nerve palsies. Neuroimaging revealed a diffuse,
widespread white matter disease process involving the
cerebral hemispheres, brainstem, and cerebellum. Such fin-
dings indicate a broad differential diagnosis that includes
demyelinating processes such as ADEM and multiple scle-
rosis, infectious processes such as viral encephalitis, vascu-
litic processes such as connective tissue disease vasculitis,
leukodystrophies, and neoplastic processes such as lym-
phoma and gliomatosis cerebri.
Based on the initial clinical presentation and imaging re-
sults, a working diagnosis of ADEM was ascribed. A well-
J. Neurosurg. (Pediatrics 1) / Volume 102 / January, 2005
Pediatric gliomatosis cerebri to cerebellar GBM presenting as ADEM
FIG. 1. Magnetic resonance FLAIR images obtained at the time of the ADEM diagnosis. Left: Axial image demon-
strating the enlargement and masslike appearance of the left cerebellar peduncle with an abnormal signal anteriorly in the
pons and in the white matter (dentate nucleus) of the cerebellum.
Right: Coronal image revealing bilateral abnormal
hyperintensities in the subcortical and periventricular white matter as well as bilateral cerebellar hyperintensities. The
findings in each image are consistent with white matter leukoencephalopathy and ADEM.
Janpeds2005 12/17/04 9:04 AM Page 73
characterized condition, ADEM usually presents abruptly
in children and young adults, causing multifocal neu
rological disturbances that are accompanied by generalized
complaints of headache, fever, nausea, vomiting, and chang-
es in mental status ranging from somnolence to coma.
It usually follows a viral prodrome or vaccination, typical
ly 5 to 14 days after the initial exposure.
At London
Hospital, its prevalence in the pediatric population account-
ed for approximately one third of all cases of encephalitis
between 1963 and 1978.
The most common presenting
feature is ataxia, although less common presentations such
as nausea and vomiting or acute psychosis have been doc-
Although gray matter can be involved, MR
imaging generally demonstrates white matter lesions. The
ADEM lesions as demonstrated on MR images include the
following characteristics: 1) few in number; 2) localized to
the brainstem and posterior fossa; 3) asymmetrical; 4) non-
hemorrhagic; and 5) easily correlated to clinical symp-
Lesions involving the corpus callosum and periven-
tricular region are less common than those seen in multiple
In addition to targeting suspected offending
infective agents, the most widely employed therapies for
ADEM are corticosteroid agents, intravenous Ig, and Cell-
In this case, the patient presented with history, signs,
symptoms, and neuroimaging findings consistent with
ADEM. During the month before his first admission the pa
tient was noted to have had upper respiratory infections, a
rash on his thigh, and exposure to viral illness no more than
12 days prior to becoming symptomatic. In the days imme-
diately preceding admission the patient was febrile, and ex-
hibited nausea, vomiting, lethar
, and asymmetric neuro
logical findings that correlated anatomically with the
neuroimaging findings. The finding of infratentorial as well
as supratentorial white matter changes is particularly con-
sistent with ADEM as opposed to tumor
. For the afore
mentioned reasons, the presumptive diagnosis of ADEM
was therefore justified. Steroid therapy, intravenous Ig, and
treatment with Cellcept were instituted with only modest
success. Ultimately these therapies failed to arrest progres
sion of the disease process, as the clinical and neuroimag
ing examinations demonstrated. The boy was ultimately
found to harbor a cerebellar GBM and died of this disease.
In view of the histological diagnosis of cerebellar GBM,
P. B. Senatus, et al.
J. Neurosurg. (Pediatrics 1) / Volume 102 / January, 2005
FIG. 2. Gadolinium-enhanced T
-weighted MR images obtained at admission (A and B) and 1 month after diagnosis
(C and D). The initial axial (A) and sagittal (B) images reveal a mass effect in the left cerebellar peduncle without evi-
dence of abnormal enhancement. Later axial (C) and sagittal (D) images demonstrate a persisting mass effect appearing
as a bulky enhancing mass arising from the left cerebellar peduncle into the fourth ventricle. Note the additional enhance
ment in the anterior pons.
Janpeds2005 12/17/04 9:04 AM Page 74
it is more likely that the widespread supra- and infratentor-
ial white matter involvement represented gliomatosis cere-
bri that rapidly progressed to GBM of the cerebellum.
Gliomatosis cerebri, described by Nevin in 1938, is a
rare primary brain tumor, neuroepithelial in origin, typified
by diffuse infiltration of glioma cells into at least two lobes
of the brain. The lesion frequently extends into the corpus
callosum, the basal ganglia, the thalamus, the brainstem, or
the cerebellum.
Preservation of central nervous system
structures and the underlying cytoarchitecture is character
istic of this disease process.
Diagnosis is usually made in
the late stages of the disease and it is frequently misdiag-
nosed early in its course. Patients frequently present with
headache or seizures, but can present with findings such as
hemiparesis, nausea, vomiting, ataxia, and changes in per-
sonality or mental status.
The characteristic features of
gliomatosis cerebri are best appreciated on long repetition
time and echo time images such as T
-weighted or FLAIR
MR sequences.
Gliomatosis cerebri is classified into two types by some
pathologists: T
ype I consists of dif
fuse over
growth with
neoplastic elements without focal mass and T
ype II (which
may derive from Type I) is characterized by diffuse invasion
and manifests a focal mass, usually a high-grade glioma.
In this case, the patient presented with gliomatosis cere-
bri with both supra- and infratentorial involvement that rap-
idly progressed to a focal left cerebellar GBM. At the time
of writing this manuscript the authors are unaware of a pre-
vious report of histologically confirmed cerebellar GBM in
the pediatric population that presented simultaneously as
supra- and infratentorial glial transformation. Our report is
in keeping with a recent study of gliomatosis cerebri of the
entire neuraxis in a 7-year-old girl in whom anaplastic as-
trocytoma of the optic nerve was discovered.
We would like to underscore the rarity of cerebellar
GBM in the pediatric population. Despite the observation
that congenital GBM commonly occurs in the cerebellum
when it presents, only approximately 30 cases of cerebellar
GBM have been reported in the literature. The twofold ra-
rity of supra- and infratentorial transformation and cerebel-
lar GBM make this case unique.
Based on the MR images (Figs. 1 and 2) and on evalua-
tion of the entire series of MR imaging studies in this pa
tient, we do not think leptomeningeal spread of disease is a
serious possibility in this case because on several contrast-
enhanced images no abnormal leptomeningeal enhance-
ment, linear or nodular, was apparent. Intraventricular
spread of disease is also unlikely due to the lack of ependy-
mal or subependymal enhancement on the neuroimaging
studies. Initial MR images obtained in the patient after ad
ministration of contrast agents reveal no abnormal paren-
chymal or leptomeningeal enhancement (Fig. 2A and B).
One may question our confidence that the early mani
festations of this child’s disease process were due to glio-
matosis cerebri rather than multifocal GBM, particularly
given that the final histologically confirmed diagnosis was
cerebellar GBM and that the incidence of multifocal GBM
is significantly greater than that of gliomatosis cerebri.
Although both multifocal GBM and gliomatosis cerebri
involve multiple brain areas and are associated with poor
survival, a significant pathophysiological dif
ference exists
between the two processes. Unlike gliomatosis cerebri,
J. Neurosurg. (Pediatrics 1) / Volume 102 / January, 2005
Pediatric gliomatosis cerebri to cerebellar GBM presenting as ADEM
FIG. 3. Photomicrographs showing frozen and permanent sections. A: The tumor consists of tightly packed pleo-
morphic cells arranged in sheets that are invading into the granular layer of the cerebellar cortex
(arrows). B: The
immunohistochemical staining for MIB-1 shows a high proliferation rate of the tumor. C: The immunohistochemical
staining for glial fibrillary acidic protein reveals a coarse, fibrillar network of the tumor cell processes surrounding a
“glomeruloid” vessel
(arrow). D: Individual neoplastic cells have polygonal, pleomorphic nuclei. Frequent mitoses are
identified in the field
(arrows). Original magnifications 3 10 (A, B, and C); 3 40 (D).
Janpeds2005 12/17/04 9:04 AM Page 75
multifocal GBM typically does not preserve central ner-
vous system structures or spare the underlying cytoarchi-
tecture. An initial presentation resulting from multifocal
GBM would have most likely revealed moderate edema
and a mass effect on MR images as well as strong and het-
erogeneous enhancement;
such findings were not consis
tent with neuroimaging studies from the early stages of
this patient’s disease (Fig. 1).
Furthermore, from a clinical standpoint, the early stages
of this patient’s symptomatology, which mimicked ADEM,
are much more consistent with gliomatosis cerebri than
multifocal GBM; previously reported cases have demon-
strated the ability of gliomatosis cerebri to mimic infective
lesions such as acute diffuse encephalomyelitis, tuberculosis,
Creutzfeldt–Jakob disease, and herpes encephalitis.
though multifocal GBM can theoretically present as an in-
fective lesion, it is far less likely than gliomatosis cerebri to
present in this fashion.
Given that transformation of gliomatosis cerebri to GBM
is rare, one might speculate that the patient initially pre-
sented with multifocal GBM; however, this is not support-
ed by the literature. Although gliomatosis cerebri is rare,
multiple cases of the transformation of initial gliomatosis
cerebri to GBM have been reported.
Moreover, we
propose that this case represents an example of gliomatosis
cerebri Type II in which a focal high-grade mass lesion of
the cerebellum evolved in the context of a diffuse glial neo-
plastic process.
Given the progressive nature of this
child’s disease process and its documentation on clinical
and neuroimaging examinations, it is reasonable to infer
that the cause of the patient’s symptoms was gliomatosis
cerebri rather than multifocal GBM.
It would also have been reasonable to consider less like-
ly alternatives than ADEM, gliomatosis cerebri, and mu-
ltifocal GBM within the differential diagnosis at the initial
presentation. Multiple sclerosis was a possible diagnosis
because, like ADEM, it can mimic the early symptoms and
neuroimaging findings described earlier
. That multiple
sclerosis is exceedingly rare in young children, that the pa
s symptoms persisted despite the use of steroid medi
cations and intravenous Ig, that no oligoclonal bands were
identified in the cerebrospinal fluid, and that early lesions
did not enhance or reduce the likelihood of this diagnosis.
Lymphoma, although similar in its clinical presentation,
was considered unlikely because of the absence of en-
hancement on imaging studies and this patient’s poor
response to steroid medications. V
arious types of angitis
might have mimicked the appearance of the presenting
lesions on neuroimaging studies, but without clinical evi-
dence of sickle cell disease, connective tissue diseases (that
is, Marfan syndrome, Ehler–Danlos syndrome), homocys
teinuria, or moyamoya, these causes would be unlikely.
The absence of neuroimaging findings such as basal gan
glia flow voids, significant gray matter involvement, cere
bral atrophy, and a positive diffusion-weighted MR imag-
ing signal strengthen the argument against angitis. The
existence of a viral prodrome raises the specter of viral en-
cephalitis. The atypical anatomic distribution, however
combined with the absence of personality changes or hem-
orrhagic lesions in this patient make viral encephalitis
unlikely. Although adrenoleukodystrophy and metachro-
matic leukodystrophy occur in children within the correct
age range (3–10 years of age), they are unlikely to have
caused this patient’s symptoms because evidence of liver,
spleen, kidney damage, adrenal failure, or psychomotor
deterioration was lacking.
The diagnosis of ADEM remained with our patient for
3 months as his disease progressed. Early consideration of
gliomatosis cerebri would have likely prompted the per-
formance of a brain biopsy at an earlier period in the pa-
tient’s treatment. Such intervention would have excluded
the diagnosis of ADEM and given the family a better un-
derstanding of the diagnosis, management, and prognosis
in this child. This report underscores the importance of the
consideration of gliomatosis cerebri in the differential di-
agnosis of children presenting with signs and symptoms
of ADEM who fail to respond to conventional steroid and
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Manuscript received September 23, 2003.
Accepted in final form May 7, 2004.
Address reprint requests to: Patrick Senatus M.D., Ph.D., De
partment of Neurological Surgery, Columbia College of Physicians
and Surgeons, Neurological Institute of New York, 710 West 168th
Street, PH 176, New York, New York 10032. email: pps19@
J. Neurosurg. (Pediatrics 1) / Volume 102 / January, 2005
Pediatric gliomatosis cerebri to cerebellar GBM presenting as ADEM
Janpeds2005 12/17/04 9:04 AM Page 77
  • [Show abstract] [Hide abstract] ABSTRACT: Gliomatosis cerebri (GC) is a rare fatal glial neoplasm of the central nervous system. Neuroimaging, histological, and clinical outcome data were reviewed of 17 consecutive patients, 8 males and 9 females aged 15-68 years (median, 37 years), treated for GC between April 1992 and October 2007. All patients received cranial irradiation to include the hyperintense area on T2-weighted magnetic resonance imaging. The total dose of the radiotherapy was 50-72 Gy (median, 60). Intravenous nimustine hydrochloride was administered in all patients, combined with temozolomide in four patients. The median survival time was 23.3 months, with a median follow-up of 23.3 months. Kaplan-Meier analysis demonstrated the overall survival rate which was 70.6% for 1 year, 23.5% for 3 years, and 17.7% for 5 years. Spinal enhanced lesions and nodular malignant transformation to glioblastoma were observed during follow-up in two patients each. Poor survival showed correlation with higher Ki-67 labeling index, higher choline/N-acetylaspartate ratio on magnetic resonance spectroscopy, tumor volumes, lower Karnofsky performance status on admission, cognitive/behavioral deterioration, poor response to the initial radiochemotherapy, and emergence of paraventricular enhanced lesions during the clinical course. The prognosis for patients with GC is unfavorable, but radiochemotherapy may prolong survival.
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  • [Show abstract] [Hide abstract] ABSTRACT: Opinion statement: Dolichoectasia is a dilatation and elongation of the arteries, usually affecting intracranial basilar and vertebral arteries. It may cause posterior circulation stroke or transient ischemic attacks independent of atherosclerotic disease. Compression of cranial nerves or brainstem leads to hemifacial spasm, trigeminal neuralgia, or brainstem dysfunction (temporary or permanent). Intracranial bleeding is another possible manifestation. In general, the mortality and morbidity is higher in patients with dolichoectasia. Progression of arterial dilatation or elongation is an ominous sign for poor prognosis. Optimal treatment for vertebrobasilar dolichoectasia is uncertain. Adequate control of blood pressure may prevent ischemic or hemorrhagic stroke. In case of ischemic stroke, antiplatelet treatment is indicated for the prevention of recurrent stroke. However, the risk of hemorrhagic stroke should be considered in patients with severe forms of dolichoectasia. Close observation and follow-up neuroimaging is recommended to monitor the progression of the disease and appearance of new ischemic or hemorrhagic lesions. Surgical decompression may be reserved for the medically intractable patients with trigeminal neuralgia or hemifacial spasm.
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  • [Show abstract] [Hide abstract] ABSTRACT: Gliomatosis confined to the cerebellum is most unusual. We report such a case in a 20-month-old male who presented with unsteadiness. Magnetic resonance imaging revealed a diffuse area of abnormal signal intensity within both cerebellar hemispheres, which did not enhance after contrast administration. The patient underwent a biopsy, which revealed a diffuse glioma infiltrating the cerebellum. Overall, the tumor cells had oligodendroglioma-like features and exhibited only focal vimentin immunoreactivity. They were negative for glial fibrillary acidic protein, synaptophysin, βIII-tubulin, and neurofilament protein. Immunofluorescence, performed on primary biopsy explants maintained in cell culture without exposure to growth factors or differentiation-promoting agents, revealed widespread nestin immunoreactivity and immunolabeling of occasional cells with antibodies to platelet-derived growth factor-α and O1/O4, markers of oligodendrocyte precursor-cells and immature oligodendrocytes, respectively. Fluorescent in situ hybridization performed on explants, touch preparations, and paraffin sections failed to reveal loss of heterozygosity for either 1p36 or 19q13. The patient was treated with temozolomide and remains stable, albeit with residual quiescent tumor, more than 3 years after surgery. This report calls attention to an unusual presentation of gliomatosis confined to the cerebellum of a toddler and addresses salient aspects of clinical and radiological differential diagnosis, as well as therapeutic challenges encountered.
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