Cerebellar Glioblastoma Multiforme in an = Adult

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Journal=20 List > J=20 Korean Neurosurg Soc > v.4= 3(4);=20 Apr 2008

PubMed articles by:

J Korean Neurosurg = Soc.=20 2008 April;=20 43(4):=20 194=E2=80=93197.

Published online 2008 = April 20.=20 doi:=20 10.3340/jkns.2008.43.4.194.

PMCID:=20 PMC2588262

Cerebellar Glioblastoma Multiforme in an = Adult

Hyuk Hur, M.D., = Shin Jung,=20 M.D.,=20 Tae-Young Jung, M.D., and In-Young Kim, M.D.

Department of Neurosurgery, Chonnam National = University=20 Hwasun Hospital & Medical School, Hwasun, Korea.

Corresponding=20 author.

Address for reprints: Shin Jung, M.D. = Department of=20 Neurosurgery, Chonnam National University Hwasun Hospital & = Medical=20 School, 160 Ilsim-ri, Hwasun-eup, Hwasun-gun, Hwasun 519-809, = Korea. Tel:=20 +82-61-379-7666, 7668, Fax: +82-61-379-7673, Email: sjung/at/chonnam.ac.kr

Received May 2, 2007; Accepted April 7,=20 2008.

Abstract

Primary cerebellar = glioblastoma multiforme (GBM) is a rare tumor in adults that = accounts for=20 just 1% of all cases of GBM. Due to their rarity, cerebellar GBMs = are not=20 yet completely understood about the pathogenesis and the = prognosis. Here,=20 we present a case of GBM in a 69-year-old man. Neurologic = examination=20 revealed the presence of cerebellar signs. Magnetic resonance = imaging=20 (MRI) showed a 4.5 =C3=97 3.6 cm-sized, ill-defined, = heterogeneously enhancing=20 mass in the left cerebellum and two patchy hyperintense lesions in = the=20 right cerebellum with minimal enhancement. After operation, = glioblastoma=20 was histologically confirmed. Postoperative radiotherapy with = concomitant=20 and adjuvant temozolomide chemotherapy was subsequently followed. = Here, a=20 case of unusual GBM in the cerebellum is reported with review of=20 literature regarding the pathogenesis, the differential diagnosis = and=20 prognosis. There was no evidence of recurrence during = postoperative one=20 year. This patient showed a good prognosis in spite of the = multiple=20 lesions.

Keywords: Cerebellum, Differential diagnosis, Glioblastoma=20 multiforme, Pathogenesis

INTRODUCTION

Glioblastoma multiforme (GBM) is the most common primary = central=20 nervous (CNS) tumor in adults comprising approximately 50% of all = primary=20 intracranial tumors. They generally occur in the fifth and sixth = decades=20 of life. These are infiltrating tumors located in the deep white = matter or=20 in the deep gray matter neighboring white matter, primarily in = cerebral=20 hemispheres. Primary cerebellar GBM is a rare tumor in adults = ²^,⁶^,⁸^,⁹^,¹⁵^-¹⁸⁾and=20 accounts for just 1% of all cases of GBM. Cerebellar GBMs are not = yet=20 completely understood about the pathogenesis and the prognosis due = to=20 their rarity. Here, we report a case of cerebellar GBM in an adult = and=20 provide a review of the literature regarding the pathogenesis, = diagnosis=20 including radiological and pathological aspects, and treatment of=20 cerebellar GBM.

CASE REPORT

A 69-year-old man presented with headache, dizziness and gait=20 disturbance. Neurological examination revealed the presence of = cerebellar=20 signs including cerebellar ataxia, tandem gait, dysmetria and=20 dysdiadochokinesia. He did not have any past medical history. = Magnetic=20 resonance imaging (MRI) showed a hypointense and hyperintense = lesion in=20 the left cerebellum on T1- and T2-weighted images, respectively = (Fig.=20 1A, B).=20 The mass had poorly defined borders and mild peritumoral edema. = After the=20 gadolinium administration, a 4.5=C3=973.6 cm-sized, ill-defined,=20 heterogeneously ring-enhanced mass was observed with one = additional patchy=20 enhancing lesion (Fig.=20 1C). The MR Spectroscopy of the lesion demonstrated a high = choline=20 peak (choline/creatinine ratio=3D3.3) in contrast to the decreased = N-acetil-aspartate (NAA) peak and markedly increased lactate peak, = suggesting the presence of a high-grade tumor (Fig.=20 1D). The provisional diagnosis was metastatic lesion or = glioblastoma=20 multiforme (GBM) in the cerebellum. To rule out the metastatic = lesion,=20 chest x-ray and abdominal sonography were done but there studies = did not=20 show any tumorous lesions.

Fig.=20 1

Magnetic = resonance=20 (MR) imaging revealed the presence of hypointense and = hyperintense=20 lesions in the left cerebellum in T1- and in T2-weighted = images,=20 respectively (A, B). The mass had poorly defined borders, = mild=20 peritumoral edema, and large areas of=20 (more ...)

We performed the operation using the midline suboccipital = approach in=20 the prone position. There was a yellowish tumor with necrotic = change that=20 had indistinct margins from adjacent normal white matter. A gross = total=20 resection was performed. The postoperative course was uneventful = and his=20 symptoms and neurologic signs gradually improved. Histological = examination=20 showed a cellular tumor, consistent with glioblastoma, composed of = elongated, spindle-shaped cells with irregular, moderately = pleomorphic=20 nuclei, as well as proliferative blood vessels and necrosis (Fig.=20 2A, B).=20 The tumor cells showed cytoplasmic positivity for glial fibrillary = acidic=20 protein (GFAP) (Fig.=20 2C). About 30-40% of the nuclei were positive for Ki-67 (Fig.=20 2D). The patient received radiotherapy with concomitant and = adjuvant=20 temozolomide beginning 24 days after surgery. Irradiation was = given 54-Gy=20 dose in total per 27 fractions. There was no evidence of = recurrence during=20 postoperative one year.

Fig.=20 2

Histological=20 examination of the removed tumor specimens showed a cellular = tumor=20 composed of elongated, spindle-shaped cells with irregular,=20 moderately pleomorphic nuclei, as well as proliferative = blood=20 vessels and necrosis (A : original magnification=20 (more ...)

DISCUSSION

Glioblastoma (GBM) is the most common primary intracranial = tumor in=20 adults. It can be seen in people of all ages. However, = approximately=20 two-thirds of the cases occur in adult patients with a peak = incidence=20 around the sixth decade¹⁶⁾=20 and a second peak in the first decade¹⁸⁾.=20 The male to female ratio was 2:1⁷^,⁸^,¹⁶⁾.

Infratentorial GBM, however, is a rare entity. Thus, GBM of the = cerebellum only comprises a small proportion of all GBM of the = brain (0.24=20 to 1.00%)¹⁰⁾.=20 Patients typically present with increased pressure, and the most = frequent=20 symptoms are headache, nausea, vomiting and ataxia²^,⁶^,⁹^,¹⁵^,¹⁶^,¹⁸⁾.=20 Dizziness, neck pain, and mental confusion can also be = present⁹^,¹⁷^,¹⁸⁾.=20 The diagnosis of GBM of the cerebellum is not usually suspected=20 preoperatively, although there are certain computed tomography = (CT) and=20 MRI features which may point towards it. Cerebellar metastasis and = anaplastic astrocytoma are the common differential diagnosis in an = adult.=20 Although most authors failed to fully explain the rarity of = cerebellar=20 GBM²^,⁶^,¹⁶⁾,=20 an explanation for the lesser tendency of cerebellar astrocytes to = become=20 malignant has been proposed¹⁸⁾.

As stated above, cerebellar astrocytes have lesser tendency to = become=20 malignant, but several factors like radiotherapy causing the = anaplastic=20 progression of cerebellar astrocytic cells have been proposed. The = role of=20 radiotherapy in causing malignant astrocytoma of the cerebellum is = uncertain¹⁴⁾.=20 But, Maat-Schieman et al.¹⁴⁾=20 reported a midline cerebellar astrocytoma that occurred after = radiotherapy=20 following craniopharyngioma. The development of GBM following = pilocytic=20 astrocytoma, medulloblastoma and other posterior fossa midline = tumors has=20 also been described¹⁹^,²¹^,²²⁾.=20 However, it is difficult to prove their malignant transformation = since=20 most of those cases were submitted to radiation therapy=20 postoperatively.

One of the important diagnostic method for cerebellar GBM is CT = with=20 contrast enhancement. However, the image of diseases such as = metastases,=20 abscess and cerebellar infract is similar with GBM, therefore the=20 differential diagnosis from these diseases is difficult¹⁰^,¹⁵^,²⁰⁾.=20 Metastatic tumor is the most common differential diagnosis for = posterior=20 fossa brain masses in adults. Solid tumors with contrast = enhancement or=20 those with central hypodensity suggesting necrosis were described = in=20 cerebellar GBM. Occhiogrosso et al.¹⁶⁾=20 found little peritumoral edema in patients with cerebellar GBM, = and Zito=20 et al.²³⁾=20 stated that CT was helpful in differentiating GBM from metastasis = of the=20 cerebellum via peritumoral edema or mass effect. The use of MRI = has=20 increased the accuracy of the definition of the nature of the = lesion=20 because of its good tissue resolution and lack of major bone=20 artifacts²^,⁵⁾.=20 The differences in vascularity and metabolite levels in the = periphery of a=20 tumor have been found to be significant for the differentiation of = metastases and GBMs. The relative cerebral blood volumes in the=20 peritumoral areas calculated with perfusion-weighted MR imaging = are=20 clearly higher in gliomas than in metastases. In the spectroscopic = MR=20 examination, choline levels are high in the peritumoral areas of=20 gliomas⁴^,¹²⁾.=20 A significant reduction in NAA due to the loss of neuronal = elements, an=20 elevation of the choline peak due to increased membrane synthesis = and the=20 presence of a lactate peak all correlate with the higher degree of = malignancy commonly observed in GBM.

Abscess shows well-defined, thin-walled enhancing rim on = contrast=20 enhanced T1 weighted images. Diffusion weighted image shows = increased=20 signal intensity in abscess and ADC map demonstrates markedly = decreased=20 signal centrally within abscess. But, primary or metastatic = neoplasm is=20 frequently seen as low signal on diffusion weighted images¹¹⁾.=20 The contrast enhancement 1 to 2 weeks after infarction is so = called=20 "fogging effect" that is probably due to a reduction in edema and = leakage=20 of proteins from cell lysis¹⁾.=20 However, this lesion shows a virtually normal appearing T2 = weighted=20 images.

The biological behavior of cerebellar and supratentorial GBM is = similar¹³^,¹⁸⁾.=20 On the other hand, it is possible that cerebellar GBM found in = young aged=20 patients has a better prognosis as in anaplastic astrocytomas = (AAs), which=20 have a longer survival time than supratentorial GBM. The median = survival=20 for cerebellar GBM is known to be approximately 19 months⁹⁾.

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CONCLUSION

We report an experience of an unusual case of cerebellar GBM. = The=20 differential diagnosis with metastases, abscess and cerebellar = infract=20 with contrast enhancement is necessary

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<= /DIV>

Articles=20 from Journal of Korean=20 Neurosurgical Society are provided here courtesy of =
The=20 Korean Neurosurgical Society

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.scale-pmc A.selected-8 { BACKGROUND-POSITION: -37px -207px; CURSOR: default } .scale-pmc A.selected-8:hover { BACKGROUND-POSITION: -37px -207px; CURSOR: default } ------=_NextPart_000_0000_01C96F3E.E92B14E0 Content-Type: application/octet-stream Content-Transfer-Encoding: quoted-printable Content-Location: http://www.pubmedcentral.nih.gov/corehtml/pmc/js/common.js // Called from many places to handle links.=0A= // Params:=0A= // link: URL. 'this' to reuse same window, else ""=0A= // windowname: Reference name for window=0A= // additional: Attributes for window.open, from:=0A= // width, height, resizable, scrollbars, toolbar, location, = directories,=0A= // status, menubar, copyhistory.=0A= // Notes: Book version always provides menubar, toolbar, etc.=0A= //=0A= function startTargetBook(link,windowname,width,height,additional)=0A= {=0A= startTarget(link,windowname,width,height,additional)=0A= }=0A= =0A= =0A= //=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=0A= function startTarget(link,windowname,width,height, additional)=0A= {=0A= if(! window.focus)=0A= return;=0A= =0A= var sizestring =3D ",width=3D" + width + ",height=3D" + height;=0A= var opt =3D = "menubar=3Dno,toolbar=3Dno,status=3Dno,scrollbars=3Dyes,resizable=3Dyes,d= ependent=3Dyes,location=3Dno";=0A= var allOptions =3D opt + sizestring;=0A= if (additional) { allOptions =3D allOptions + ',' + additional; }=0A= =0A= windowname =3D String(windowname).replace(/-/g, "_")=0A= var moveToXDefault =3D 75=0A= var moveToYDefault =3D 50=0A= =0A= var moveToX =3D moveToXDefault=0A= var moveToY =3D moveToYDefault=0A= =0A= try {=0A= if (window.screen.width && width > 0)=0A= moveToX =3D Math.ceil((window.screen.width - width)) - 15=0A= moveToX =3D (moveToX > 0 ? moveToX : moveToXDefault)=0A= =0A= if (window.screen.height && height > 0)=0A= moveToY =3D Math.ceil((window.screen.height - height)) - 50=0A= moveToY =3D (moveToY > 0 ? moveToY : moveToYDefault)=0A= }=0A= catch (e) {}=0A= =0A= var wLeftTopCornerOptions =3D ',left=3D' + moveToX + ',top=3D' + = moveToY + ',screenX=3D' + moveToX + ',screenY=3D' + moveToY=0A= =0A= allOptions =3D allOptions + wLeftTopCornerOptions=0A= =0A= w =3D window.open ("", windowname, allOptions)=0A= w.focus()=0A= =0A= link.target=3Dwindowname=0A= }=0A= =0A= =0A= //=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=0A= function focuswin(windowname)=0A= {=0A= windowname =3D String(windowname).replace(/-/g, "_")=0A= w =3D = window.open("",windowname,"menubar=3Dyes,scrollbars=3Dyes,toolbar=3Dyes,l= ocation=3Dyes,status=3Dyes,directories=3Dyes,resizable=3Dyes");=0A= w.focus();=0A= =0A= return true;=0A= }=0A= =0A= =0A= //=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=0A= function reverseString (inStr)=0A= {=0A= var outStr =3D ''=0A= =0A= for (i =3D0; i <=3D inStr.length; i++)=0A= {=0A= outStr =3D inStr.charAt(i) + outStr=0A= }=0A= =0A= return outStr=0A= }=0A= =0A= =0A= //=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=0A= function reverseAndReplaceString (inStr, findStr, newStr)=0A= {=0A= return reverseString(inStr).replace(findStr, newStr)=0A= }=0A= =0A= //=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=0A= function initUnObscureEmail (className, innerHTML)=0A= {=0A= =0A= try{=0A= if (window.addEventListener)=0A= window.addEventListener('load', function() {unObscureEmail = (className, innerHTML)}, false)=0A= else if (window.attachEvent)=0A= window.attachEvent('onload', function() {unObscureEmail = (className, innerHTML)})=0A= }catch(e){=0A= }=0A= }=0A= =0A= //=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D=3D= =3D=3D=3D=3D=3D=0A= function unObscureEmail (className, innerHTML)=0A= {=0A= try {=0A= if (typeof(unObscuredEmails) =3D=3D 'undefined')=0A= unObscuredEmails =3D new Array()=0A= =0A= if (! 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