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A rare case of cerebellar glioblastoma multiforme in childhood: MR imaging
ELSEVIER
A RARE CASE OF CEREBELLAR GLIOBLASTOMA MULTIFORME IN CHILDHOOD: MR IMAGING DOUGLAS S. KATZ, MD, LARRY B. POE, JEFFREY A. WINFIELD, MD, PHD, AND ROBERT J. CORONA, JR., DO Glioblastoma multiforme is a highly malignant brain neoplasm that is very rarely discovered in childhood but accounts for approximately 17% of intracranial tumors in the adult. Only approximately 25 children with glioblastoma multiforme in the cerebellum have been described in the literature. We report on a 41/zyear-old girl with this tumor in the cerebellar hemisphere and discuss the magnetic resonance imaging findings. KEY WORDS:
Glioblastoma multiforme; Cerebellum; MR; Childhood
CASE HISTORY A 4%year-old girl presented with a &month history of “stumbling” and bumping into objects. Her parents first noticed her unsteadiness after she rode a carousel at a local fair. Physical examination at presentation was notable for a moderately wide-based gait and slow, rapidly alternating movements in the left upper and lower extremities. A magnetic resonance (MR) scan at 1.5 T revealed a large midline cerebellar and vermian mass that was mainly isointense to gray matter on long repetition time (TR), long echo time (TE) sequences. It demonstrated an enhancing periphery and a significant anterior cystic component that compressed the fourth ventricle (Figure 1). The patient underwent an
From the Departments of Radiology (D.S.K., L.B.P.) , Neurosurgery (J.A.W.), and Pathology (R.J.C.), State University of New York Health Science Center at Syracuse, Syracuse, New York. Address reprint requests to: Douglas S. Katz, MD, Department of Radiology, SUNY Health Science Center at Syracuse, 750 East Adams Street, Syracuse, NY 13210. ReceivedJanuary 10,X394; revised March IO, 1994; accepted April 27, 1994. CLINICAL IMAGING1995;19:162-164 Science Inc., 1995
o Elsevier 655 Avenue
of the Americas,
New York, NY 10010
MD,
occipital craniotomy for surgical resection of the mass. Pathological examination of the surgical specimen revealed glioblastoma multiforme (Figure 2). DISCUSSION Glioblastoma multiforme is a relatively rare intracranial tumor in children. While astrocytomas account for approximately 37% of childhood brain tumors, glioblastoma multiforme comprised only 6.5% of 4404 pediatric intracranial neoplasms in one combined series (1). In another review of 1847 patients with glioblastoma multiforme, less than 3 % were children (2). Cerebellar glioblastoma multiforme is very rare in adults (3) and extremely rare in children. Only approximately 25 well-documented cases of primary glioblastoma multiforme in the cerebellum of patients under age 20 have been described in the literature (l-2, 4-7). The reason why glioblastoma multiforme occurs so rarely in the cerebellum is unknown (3,8). One would expect 10% of malignant gliomas to occur in the cerebellum based on its weight relative to the whole brain (3). In the few cerebellar glioblastomas imaged with CT, a large hemispheric or midline mass was seen, without significant peritumoral edema. Rim enhancement, hydrocephalus, and cavitation or cyst formation were noted in several patients (3, 4, 6-9). To our knowledge, the MR appearance of a cerebellar glioblastoma multiforme has not been described. On MR scans, our patient’s tumor was mainly isointense to gray matter on long TR, long TE sequences and demonstrated an enhancing rim and a significant cystic component that compressed the fourth ventricle. We do not believe, however, that the imaging appearance alone would differentiate this lesion since
0699-7(nl/95/$9.50
SSDI 0699-7IJ1(94)00042-B
JULY-SEF'TEMBER1995
CEREBELLARGLIOBLASTOMAMULTIFORMEINCHILDHOOD
163
FIGURE 1. (A) m-weighted axial image (TR 22OO/TE80/l NEX) demonstrates a large midline complex intraaxial posterior fossa mass. The central solid part of the mass is isointense to gray matter and contains some tiny areas of hyperintense necrosis. Multiloculated cysts form the margins [arrows). The fourth ventricle is compressed and obliterated. (B) II-weighted coronal image (TR SOO/TE15/2NEX) reveals the isointensity of the majority of the tumor [arrowheads), which is separated from the tentorium by the superior vermis. Cystic components are most notable caudally. The lateral ventricles are enlarged and transependymal migration of fluid is observable (arrows). (C) After gadolinium-diethylenetriamine pentaacetic acid is given, the solid portion of this tumor takes up the contrast agent, especially in the periphery. The cystic components are apparently not necrosis as there is no marginal enhancement. This parasagittal image best demonstrates the compression of the fourth ventricle [arrow) and the relationship of the tumor to the medullary velum [arrowheads). The lateral and third ventricles as well as the cerebral aqueduct are enlarged.
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FIGURE2. (A)Thetumoris characterizedbypleomorphic, bizarre, and occasionally multinucleatedastrocytes (I-I&E stain,originalmagnificationx 200). (B) Not8 the abundance of atypicalmitoticfigures[arrows)(H&Estain, originalmagnification, x 200). (C) EndotheliaVpericytichyperplasiais observedcreating “glomeruloidbodies”(arrow)(H%Estain, original magnification, x 100).
the components of cysts, heterogeneous enhancement, and relative isointensity on T&weighted images have been observed with the more common tumors of the cerebellum in childhood (10). Cerebellar glioblastoma multiforme is a rapidly growing, locally aggressive tumor with a very poor prognosis. It tends to spread through cerebrospinal fluid pathways. Local recurrences also occur (1,5, 7). Whereas the addition of chemotherapy with lomustine, vincristine, and prednisone to surgery and radiotherapy may prolong the survival of children with cerebellar high-grade astrocytoma (l), no long-term cure of cerebellar glioblastoma has been reported (11).
REJTEXENCES 1. Sposto R, Ertel IJ, Jenkin RD. The effectiveness of chemotherapy for treatment of high grade astmqtoma in children: results of a randomized trial. J Neurooncol 1989;7:165-177. 2. DohrmannGJ, lkwell JR, Flannery JT. Glioblastomamultiforme in children. J Neurosurg 1976;44:442-448.
3. Levine SiA, McKeever PB, Greenberg HS. Primary cerebellar glioblastoma multiforme. J Neurooncol 19e5:231-238. 4. Aziz Tz. Cerebellar glioblastoma multiforme: a report of two cases and review of the literature. Wst Engl Med J 1999;185: 39-42. 5. Salazar OM. Primary malignant cerebellar astrocytoma in children: a signal for postoperative cranispinal irradiation. Int J Radiat Oncol Biol Phys 1981;7:1861-1865. 6. Chin I-W, Maruyama Y, GibbsP, MarkesberyW, Young B. Cerebellar glioblastoma in childhood. J Neurooncol1984;2:79-84. 7. George8 PM, Noterman J, FlamentDura.nd J. Glioblastoma of the cerebellum in childhood and adolescents. J Neurooncol 1983;1:275-278. 8. Bhimani S, Viipongse C, Spencer D, Kim J. CT appearance of cerebellar glioblastoma multiforme. J Comput Assist lbmogr 1983;7:889-891. 9. ZitoJL, Siva A, Smith TW, Leeds M, Davidson R. Glioblastoma of the cerebellum: computed tomographic and pathologic considerations. Surg Neurol 1983;19:373-378. 10. Atlas SW. I&a-axial brain tumors. In Atlas SW (ed): Magnetic Resonance Imagina of the Brain and S&e. New York: Raven Press, 1991. - 11. Pang D, Ashmead JW. Ektraneural metastasis of cerebellar glioblastoma multiforme. Neurosurgery 1982;10:252-252
A RARE CASE OF CEREBELLAR GLIOBLASTOMA MULTIFORME IN CHILDHOOD: MR IMAGING DOUGLAS S. KATZ, MD, LARRY B. POE, JEFFREY A. WINFIELD, MD, PHD, AND ROBERT J. CORONA, JR., DO Glioblastoma multiforme is a highly malignant brain neoplasm that is very rarely discovered in childhood but accounts for approximately 17% of intracranial tumors in the adult. Only approximately 25 children with glioblastoma multiforme in the cerebellum have been described in the literature. We report on a 41/zyear-old girl with this tumor in the cerebellar hemisphere and discuss the magnetic resonance imaging findings. KEY WORDS:
Glioblastoma multiforme; Cerebellum; MR; Childhood
CASE HISTORY A 4%year-old girl presented with a &month history of “stumbling” and bumping into objects. Her parents first noticed her unsteadiness after she rode a carousel at a local fair. Physical examination at presentation was notable for a moderately wide-based gait and slow, rapidly alternating movements in the left upper and lower extremities. A magnetic resonance (MR) scan at 1.5 T revealed a large midline cerebellar and vermian mass that was mainly isointense to gray matter on long repetition time (TR), long echo time (TE) sequences. It demonstrated an enhancing periphery and a significant anterior cystic component that compressed the fourth ventricle (Figure 1). The patient underwent an
From the Departments of Radiology (D.S.K., L.B.P.) , Neurosurgery (J.A.W.), and Pathology (R.J.C.), State University of New York Health Science Center at Syracuse, Syracuse, New York. Address reprint requests to: Douglas S. Katz, MD, Department of Radiology, SUNY Health Science Center at Syracuse, 750 East Adams Street, Syracuse, NY 13210. ReceivedJanuary 10,X394; revised March IO, 1994; accepted April 27, 1994. CLINICAL IMAGING1995;19:162-164 Science Inc., 1995
o Elsevier 655 Avenue
of the Americas,
New York, NY 10010
MD,
occipital craniotomy for surgical resection of the mass. Pathological examination of the surgical specimen revealed glioblastoma multiforme (Figure 2). DISCUSSION Glioblastoma multiforme is a relatively rare intracranial tumor in children. While astrocytomas account for approximately 37% of childhood brain tumors, glioblastoma multiforme comprised only 6.5% of 4404 pediatric intracranial neoplasms in one combined series (1). In another review of 1847 patients with glioblastoma multiforme, less than 3 % were children (2). Cerebellar glioblastoma multiforme is very rare in adults (3) and extremely rare in children. Only approximately 25 well-documented cases of primary glioblastoma multiforme in the cerebellum of patients under age 20 have been described in the literature (l-2, 4-7). The reason why glioblastoma multiforme occurs so rarely in the cerebellum is unknown (3,8). One would expect 10% of malignant gliomas to occur in the cerebellum based on its weight relative to the whole brain (3). In the few cerebellar glioblastomas imaged with CT, a large hemispheric or midline mass was seen, without significant peritumoral edema. Rim enhancement, hydrocephalus, and cavitation or cyst formation were noted in several patients (3, 4, 6-9). To our knowledge, the MR appearance of a cerebellar glioblastoma multiforme has not been described. On MR scans, our patient’s tumor was mainly isointense to gray matter on long TR, long TE sequences and demonstrated an enhancing rim and a significant cystic component that compressed the fourth ventricle. We do not believe, however, that the imaging appearance alone would differentiate this lesion since
0699-7(nl/95/$9.50
SSDI 0699-7IJ1(94)00042-B
JULY-SEF'TEMBER1995
CEREBELLARGLIOBLASTOMAMULTIFORMEINCHILDHOOD
163
FIGURE 1. (A) m-weighted axial image (TR 22OO/TE80/l NEX) demonstrates a large midline complex intraaxial posterior fossa mass. The central solid part of the mass is isointense to gray matter and contains some tiny areas of hyperintense necrosis. Multiloculated cysts form the margins [arrows). The fourth ventricle is compressed and obliterated. (B) II-weighted coronal image (TR SOO/TE15/2NEX) reveals the isointensity of the majority of the tumor [arrowheads), which is separated from the tentorium by the superior vermis. Cystic components are most notable caudally. The lateral ventricles are enlarged and transependymal migration of fluid is observable (arrows). (C) After gadolinium-diethylenetriamine pentaacetic acid is given, the solid portion of this tumor takes up the contrast agent, especially in the periphery. The cystic components are apparently not necrosis as there is no marginal enhancement. This parasagittal image best demonstrates the compression of the fourth ventricle [arrow) and the relationship of the tumor to the medullary velum [arrowheads). The lateral and third ventricles as well as the cerebral aqueduct are enlarged.
164
KATZ ET AL.
CLINICAL IMAGINGVOL. 19, NO. 3
FIGURE2. (A)Thetumoris characterizedbypleomorphic, bizarre, and occasionally multinucleatedastrocytes (I-I&E stain,originalmagnificationx 200). (B) Not8 the abundance of atypicalmitoticfigures[arrows)(H&Estain, originalmagnification, x 200). (C) EndotheliaVpericytichyperplasiais observedcreating “glomeruloidbodies”(arrow)(H%Estain, original magnification, x 100).
the components of cysts, heterogeneous enhancement, and relative isointensity on T&weighted images have been observed with the more common tumors of the cerebellum in childhood (10). Cerebellar glioblastoma multiforme is a rapidly growing, locally aggressive tumor with a very poor prognosis. It tends to spread through cerebrospinal fluid pathways. Local recurrences also occur (1,5, 7). Whereas the addition of chemotherapy with lomustine, vincristine, and prednisone to surgery and radiotherapy may prolong the survival of children with cerebellar high-grade astrocytoma (l), no long-term cure of cerebellar glioblastoma has been reported (11).
REJTEXENCES 1. Sposto R, Ertel IJ, Jenkin RD. The effectiveness of chemotherapy for treatment of high grade astmqtoma in children: results of a randomized trial. J Neurooncol 1989;7:165-177. 2. DohrmannGJ, lkwell JR, Flannery JT. Glioblastomamultiforme in children. J Neurosurg 1976;44:442-448.
3. Levine SiA, McKeever PB, Greenberg HS. Primary cerebellar glioblastoma multiforme. J Neurooncol 19e5:231-238. 4. Aziz Tz. Cerebellar glioblastoma multiforme: a report of two cases and review of the literature. Wst Engl Med J 1999;185: 39-42. 5. Salazar OM. Primary malignant cerebellar astrocytoma in children: a signal for postoperative cranispinal irradiation. Int J Radiat Oncol Biol Phys 1981;7:1861-1865. 6. Chin I-W, Maruyama Y, GibbsP, MarkesberyW, Young B. Cerebellar glioblastoma in childhood. J Neurooncol1984;2:79-84. 7. George8 PM, Noterman J, FlamentDura.nd J. Glioblastoma of the cerebellum in childhood and adolescents. J Neurooncol 1983;1:275-278. 8. Bhimani S, Viipongse C, Spencer D, Kim J. CT appearance of cerebellar glioblastoma multiforme. J Comput Assist lbmogr 1983;7:889-891. 9. ZitoJL, Siva A, Smith TW, Leeds M, Davidson R. Glioblastoma of the cerebellum: computed tomographic and pathologic considerations. Surg Neurol 1983;19:373-378. 10. Atlas SW. I&a-axial brain tumors. In Atlas SW (ed): Magnetic Resonance Imagina of the Brain and S&e. New York: Raven Press, 1991. - 11. Pang D, Ashmead JW. Ektraneural metastasis of cerebellar glioblastoma multiforme. Neurosurgery 1982;10:252-252