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Gliomatosis cerebri: findings with computed tomography and magnetic resonance imaging
European Journal of Radiology 28 (1998) 226 – 229
Gliomatosis cerebri: findings with computed tomography and magnetic resonance imaging Pedro Ponce *, M. Victoria Alvarez-Santullano, Emilio Otermin, Miguel Angel Santana, MaVicenta Garcı´a Luden˜a Department of Diagnostic Radiology, Hospital Nuestra Sen˜ora del Pino, c/ Angel Guimera no. 93, Las Palmas de GC. E 35005, Canary Islands, Spain Received 11 August 1997; received in revised form 14 November 1997; accepted 17 November 1997
Abstract Gliomatosis cerebri (GC), is a rare neoplastic disease (less than 150 cases reported in the literature) with a diffuse, widespread proliferation of neoplastic glial cells in the brain, generally affecting both hemispheres and involving the gray and white matter [1–3]. Less commonly, the cerebellum, the brain stem and the medulla can be affected. Histologic evaluation reveals neoplastic astrocytes with varying levels of differentiation. Perineuronal and perivascular spread of tumor infiltration is observed. Demyelination can be found in the affected areas. A well-preserved underlying neuroanatomic architecture is considered characteristic [2]. Clinical signs vary and are non-specific, including changes in the mental state and headaches, followed by focal motor deficits and convulsive episodes [4]. The prognosis is poor, ranging from weeks to some years after the manifestation of the symptoms. Steroids may be useful in the short term, but chemotherapy is of little value and radiotherapy of questionable benefit. The literature was reviewed and the radiological pattern of three new cases of GC is reported. In two cases the diagnosis was achieved ante-mortem. © 1998 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Gliomatosis cerebri; Magnetic resonance; Computerized tomography
1. Patients
1.1. Case 1 A 43-year-old woman was admitted to our hospital for the evaluation of convulsive seizures. Computed tomography (CT) was normal. In magnetic resonance imaging (MRI) showed diffuse and poorly delineated infiltrating hypo- to isointense lesions on T1 weighted (T1WI) and hyperintense to gray matter on T2 weighted (T2WI), located in both thalami and temporal lobes (Fig. 1a), affecting the cortical as well as subcortical white matter with ventricular compression. After administration of contrast media the lesions did not * Corresponding author. Tel.: + 34 28 441087; fax: + 34 28 441062; e-mail: [email protected]
enhance. The patient continued to deteriorate, developing progressive memory disturbances and 14 months later CT and MRI showed a progression of the lesions with increasing mass effect (Fig. 1b). Given these findings, the diagnosis of gliomatosis cerebri (GC) was suspected and confirmed by stereotactic biopsy (thalamus and right uncus). Repeat CT and MRI studies some months later showed an extensively enhancing lesion in the left parietal lobe (Fig. 1c).
1.2. Case 2 A 64-year-old male presented with a 6 months history of progressive neurological deterioration with loss of short-term memory and behavior disorders. This was accompanied by convulsive episodes. An initial CT showed a generalized hypodensity of the white matter,
0720-048X/98/$ - see front matter © 1998 Elsevier Science Ireland Ltd. All rights reserved. PII S0720-048X(97)00172-1
P. Ponce et al. / European Journal of Radiology 28 (1998) 226–229
predominantly on the left side and basal ganglia with effacement of the basal cisterns and sulci (Fig. 2a). On post-contrast studies no enhancement was observed. T1WI MR images were normal. Proton density and T2WI images revealed hyperintense lesions, more marked in the white matter of the left hemisphere (Fig. 2b), associated with small lesions in gray matter—left frontal, temporal lobes and lenticular nucleus—No enhancement was observed. The patient presented clinical increased deterioration and he died 28 days after admission. The autopsy yielded a diagnosis of GC.
227
white matter tracts is felt to represent tumor spread alone or in conjunction with secondary destruction of myelin fibers. [7,9–11].
1.3. Case 3 A 58-year-old woman, was admitted to the hospital with epileptic seizures, loss of short-term memory and headaches. Initial CT revealed a hypodensity of left semioval center and corpus callosum, involvement of the frontal and occipital parasagittal cortex and mass effect with shift of the midline, without postcontrast enhancement (Fig. 3a). T1WI MR images revealed hypointense signal intensity in the paramedial cortex of both frontal lobes and the hippocampus. On T2WI MR images there was increased signal in both medial temporal and left parietal regions, the parasagittal cortex, the left lenticular nucleus, corpus callosum and in the white matter of the left hemisphere. Midline shift and sulcal effacement were observed without postcontrast enhancement (Fig. 3b). The suspicion diagnosis of GC was confirmed by stereotactic biopsy.
2. Discussion GC is an uncommon entity. Controversy surrounding its etiology led to a number of appellations being applied to it in the literature. The World Health Organization has considered this form of glioma as a clinic-pathologic entity, in which the criterion for diagnosis is the infiltration of at least two cerebral lobes, without any histologic presence of central cellular necrosis [5]. The radiological appearance has been described by several authors. CT studies show areas of structural enlargement, focal or diffuse, with hypodensity reflecting the widespread nature of the infiltrative process. Enhancement is usually minimal or absent, suggesting that the blood-brain barrier is relatively preserved [6– 9]. However enhancement can occur in late stages of the disease [8]. The MRI examinations show extensive parenchymal involvement, especially of the white matter, as manifested by ill-defined regions isointense or slightly hypointense on T1WI and of uniformly high signal on T2WI. Extension to corpus callosum, basal ganglia and thalamus is common. Hyperintensity in
Fig. 1. (a) Axial T2-weighted SE (2190/105) MR scan at cerebral peduncle level, shows asymmetrical hyperintense to gray matter lesions in both medial temporal lobes (arrows). (b) Axial T2-weighted SE (2000/80) MR scan 14 months later. The progression of the disease can be seen with extension to both thalami, frontal and occipital lobes (arrows). Observe the round lesion in right thalamus due to biopsy (open arrow). (c) Axial postcontrast CT scan shows edema that surrounds a thick, shaggy, irregular rim-enhancing mass (arrow).
228
P. Ponce et al. / European Journal of Radiology 28 (1998) 226–229
and the use of multiplanar views, lesions that appear subtle or are not apparent on CT may be identified by MRI. Sagittal and coronal images were useful particularly for the evaluation of the corpus callosum. Expansion of affected areas and mass effect were well delineated, particularly on coronal images. In these cases, It detected disease in areas that CT did not show, better defined the localization of the lesions and their distribution (in the white matter or in the cortex). MRI was decisive in establishing an early diagnostic suspicion. Findings on CT and MRI in GC are relatively nonspecific and have been misinterpreted in the literature as leukoencephalopathy, multiple sclerosis, progressive multifocal leukoencephalopathy, or isquemic change. Involvement of the deep or superficial gray matter should help exclude a leukoencephalopathy. Although plaques seen with multiple sclerosis can be large, there is usually a discrete or focal mass epicenter with focal mass effect. Other considerations should be given to an
Fig. 2. (a) CT on admission reveals diffuse low density swelling of frontal lobes and central parts of the brain. (arrows). (b) Axial T2 (2050/95) weighted MR images. There is a large bilateral fronto-parietal hyperintensity dominant in white matter and sulci effacement.
In the patients bilateral lesions were observed, involving both the gray and white matter, associated with mass effect. Involvement of basal ganglia was found in all of the cases. Infratentorial lesions were not seen. Except in the final stage of one of the patients, no contrast enhancement was observed. The observation of a focal lesion with contrast enhancement does not generally exclude the diagnosis of GC and has also been described by other authors [8], indicating disruption of the blood-brain barrier. On CT scans, many lesions appeared as poorly defined, subtle hypodense or isodense areas. In one case, the initial CT was read as normal whereas MRI revealed hyperintense signal lesions on T2WI. The authors suppose that the negative results of CT might be due to its infiltrative and minimally expansive character as well as the isodensity of the lesions. The MRI findings in the cases were similar to those of cases previously reported. MRI showed hypo or isointense lesions on T1WI and diffusely hyperintense on proton density and T2WI that were superior to T1WI for evaluating the extent of the lesion. Because of the greater sensitivity of MRI examination in detecting signal changes in the cerebral parenchyma
Fig. 3. (a) Axial CT scan with contrast enhancement shows bilateral diffuse hypodense lesions in frontal, occipital lobes (arrows) with corpus callosum involvement and shift of the midline. (b) Axial T2WI MR (2000/95) scan. Asymmetric bilateral areas of high signal intensity lesion involving gray and white matter in cerebral hemispheres. Shift of midline and sulcal effacement of left hemisphere. Compare, contrast and extent of lesion with that seen in (a).
P. Ponce et al. / European Journal of Radiology 28 (1998) 226–229
infiltrative glioma and inflammatory or infectious processes including viral encephalitis. Clinical history and neurologic findings on examination are important in narrowing this differential. GC requires clinical, radiological and pathological correlation for diagnosis. A review of the literature led to the conclusion that GC is a disease with a principally post-mortem diagnosis. However, the authors suppose that the MRI and biopsy might be decisive in the ante-mortem diagnosis.
References [1] Artigas J, Cervos-Navarro J, Iglesias J, Ebhardt G. Gliomatosis cerebri: clinical and histological findings. Clin Neuropathol 1985;4:135 – 48. [2] Schober R, Mai JK, Volk B, Wechsler W. Gliomatosis cerebri: bioptical approach and neuropathological verification. Acta Neurochir (Vienna) 1991;113:131–7.
.
229
[3] Felsberg GJ, Silver SA, Brown MT, Tien RD. Radiologic– pathologic correlation gliomatosis cerebri. Am J Neororadiol 1994;15:1745 – 53. [4] Couch J, Weiss S. Gliomatosis cerebri. Neurology 1974;24:504– 11. [5] Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumors. Brain Pathol 1993;3:255 – 68. [6] Geremia GK, Wollman R, Foust R. Computed tomography of gliomatosis cerebri. J Comput Tomogr 1988;12:698 – 701. [7] Del Carpio O, Donovan R, Korah I, Salazar A, Melancon D. Gliomatosis cerebri. Radiology 1996;198(3):831– 5. [8] Koslow SA, Claassen D, Hirsch WL, Jungreis CA. Gliomatosis cerebri: a case report with autopsy correlation. Neuroradiology 1992;34:331 – 3. [9] Shin YM, Chang KH, Han MH, Myung NH, Chi JG, Cha SH, Han MC. Gliomatosis cerebri: comparison of MR and CT features. Am J Roentgenol 1993;161:859 – 62. [10] Pyhtinen J, Paakko E. A difficult diagnosis of gliomatosis cerebri. Neuroradiology 1996;38(5):444– 8. [11] Rivas E, Guzman J, Mora La Cruz E, Cardozo Duran J. Gliomatosis cerebri. Ante-mortem diagnosis. Invest Clin 1995;36(3):149– 57.
Gliomatosis cerebri: findings with computed tomography and magnetic resonance imaging Pedro Ponce *, M. Victoria Alvarez-Santullano, Emilio Otermin, Miguel Angel Santana, MaVicenta Garcı´a Luden˜a Department of Diagnostic Radiology, Hospital Nuestra Sen˜ora del Pino, c/ Angel Guimera no. 93, Las Palmas de GC. E 35005, Canary Islands, Spain Received 11 August 1997; received in revised form 14 November 1997; accepted 17 November 1997
Abstract Gliomatosis cerebri (GC), is a rare neoplastic disease (less than 150 cases reported in the literature) with a diffuse, widespread proliferation of neoplastic glial cells in the brain, generally affecting both hemispheres and involving the gray and white matter [1–3]. Less commonly, the cerebellum, the brain stem and the medulla can be affected. Histologic evaluation reveals neoplastic astrocytes with varying levels of differentiation. Perineuronal and perivascular spread of tumor infiltration is observed. Demyelination can be found in the affected areas. A well-preserved underlying neuroanatomic architecture is considered characteristic [2]. Clinical signs vary and are non-specific, including changes in the mental state and headaches, followed by focal motor deficits and convulsive episodes [4]. The prognosis is poor, ranging from weeks to some years after the manifestation of the symptoms. Steroids may be useful in the short term, but chemotherapy is of little value and radiotherapy of questionable benefit. The literature was reviewed and the radiological pattern of three new cases of GC is reported. In two cases the diagnosis was achieved ante-mortem. © 1998 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Gliomatosis cerebri; Magnetic resonance; Computerized tomography
1. Patients
1.1. Case 1 A 43-year-old woman was admitted to our hospital for the evaluation of convulsive seizures. Computed tomography (CT) was normal. In magnetic resonance imaging (MRI) showed diffuse and poorly delineated infiltrating hypo- to isointense lesions on T1 weighted (T1WI) and hyperintense to gray matter on T2 weighted (T2WI), located in both thalami and temporal lobes (Fig. 1a), affecting the cortical as well as subcortical white matter with ventricular compression. After administration of contrast media the lesions did not * Corresponding author. Tel.: + 34 28 441087; fax: + 34 28 441062; e-mail: [email protected]
enhance. The patient continued to deteriorate, developing progressive memory disturbances and 14 months later CT and MRI showed a progression of the lesions with increasing mass effect (Fig. 1b). Given these findings, the diagnosis of gliomatosis cerebri (GC) was suspected and confirmed by stereotactic biopsy (thalamus and right uncus). Repeat CT and MRI studies some months later showed an extensively enhancing lesion in the left parietal lobe (Fig. 1c).
1.2. Case 2 A 64-year-old male presented with a 6 months history of progressive neurological deterioration with loss of short-term memory and behavior disorders. This was accompanied by convulsive episodes. An initial CT showed a generalized hypodensity of the white matter,
0720-048X/98/$ - see front matter © 1998 Elsevier Science Ireland Ltd. All rights reserved. PII S0720-048X(97)00172-1
P. Ponce et al. / European Journal of Radiology 28 (1998) 226–229
predominantly on the left side and basal ganglia with effacement of the basal cisterns and sulci (Fig. 2a). On post-contrast studies no enhancement was observed. T1WI MR images were normal. Proton density and T2WI images revealed hyperintense lesions, more marked in the white matter of the left hemisphere (Fig. 2b), associated with small lesions in gray matter—left frontal, temporal lobes and lenticular nucleus—No enhancement was observed. The patient presented clinical increased deterioration and he died 28 days after admission. The autopsy yielded a diagnosis of GC.
227
white matter tracts is felt to represent tumor spread alone or in conjunction with secondary destruction of myelin fibers. [7,9–11].
1.3. Case 3 A 58-year-old woman, was admitted to the hospital with epileptic seizures, loss of short-term memory and headaches. Initial CT revealed a hypodensity of left semioval center and corpus callosum, involvement of the frontal and occipital parasagittal cortex and mass effect with shift of the midline, without postcontrast enhancement (Fig. 3a). T1WI MR images revealed hypointense signal intensity in the paramedial cortex of both frontal lobes and the hippocampus. On T2WI MR images there was increased signal in both medial temporal and left parietal regions, the parasagittal cortex, the left lenticular nucleus, corpus callosum and in the white matter of the left hemisphere. Midline shift and sulcal effacement were observed without postcontrast enhancement (Fig. 3b). The suspicion diagnosis of GC was confirmed by stereotactic biopsy.
2. Discussion GC is an uncommon entity. Controversy surrounding its etiology led to a number of appellations being applied to it in the literature. The World Health Organization has considered this form of glioma as a clinic-pathologic entity, in which the criterion for diagnosis is the infiltration of at least two cerebral lobes, without any histologic presence of central cellular necrosis [5]. The radiological appearance has been described by several authors. CT studies show areas of structural enlargement, focal or diffuse, with hypodensity reflecting the widespread nature of the infiltrative process. Enhancement is usually minimal or absent, suggesting that the blood-brain barrier is relatively preserved [6– 9]. However enhancement can occur in late stages of the disease [8]. The MRI examinations show extensive parenchymal involvement, especially of the white matter, as manifested by ill-defined regions isointense or slightly hypointense on T1WI and of uniformly high signal on T2WI. Extension to corpus callosum, basal ganglia and thalamus is common. Hyperintensity in
Fig. 1. (a) Axial T2-weighted SE (2190/105) MR scan at cerebral peduncle level, shows asymmetrical hyperintense to gray matter lesions in both medial temporal lobes (arrows). (b) Axial T2-weighted SE (2000/80) MR scan 14 months later. The progression of the disease can be seen with extension to both thalami, frontal and occipital lobes (arrows). Observe the round lesion in right thalamus due to biopsy (open arrow). (c) Axial postcontrast CT scan shows edema that surrounds a thick, shaggy, irregular rim-enhancing mass (arrow).
228
P. Ponce et al. / European Journal of Radiology 28 (1998) 226–229
and the use of multiplanar views, lesions that appear subtle or are not apparent on CT may be identified by MRI. Sagittal and coronal images were useful particularly for the evaluation of the corpus callosum. Expansion of affected areas and mass effect were well delineated, particularly on coronal images. In these cases, It detected disease in areas that CT did not show, better defined the localization of the lesions and their distribution (in the white matter or in the cortex). MRI was decisive in establishing an early diagnostic suspicion. Findings on CT and MRI in GC are relatively nonspecific and have been misinterpreted in the literature as leukoencephalopathy, multiple sclerosis, progressive multifocal leukoencephalopathy, or isquemic change. Involvement of the deep or superficial gray matter should help exclude a leukoencephalopathy. Although plaques seen with multiple sclerosis can be large, there is usually a discrete or focal mass epicenter with focal mass effect. Other considerations should be given to an
Fig. 2. (a) CT on admission reveals diffuse low density swelling of frontal lobes and central parts of the brain. (arrows). (b) Axial T2 (2050/95) weighted MR images. There is a large bilateral fronto-parietal hyperintensity dominant in white matter and sulci effacement.
In the patients bilateral lesions were observed, involving both the gray and white matter, associated with mass effect. Involvement of basal ganglia was found in all of the cases. Infratentorial lesions were not seen. Except in the final stage of one of the patients, no contrast enhancement was observed. The observation of a focal lesion with contrast enhancement does not generally exclude the diagnosis of GC and has also been described by other authors [8], indicating disruption of the blood-brain barrier. On CT scans, many lesions appeared as poorly defined, subtle hypodense or isodense areas. In one case, the initial CT was read as normal whereas MRI revealed hyperintense signal lesions on T2WI. The authors suppose that the negative results of CT might be due to its infiltrative and minimally expansive character as well as the isodensity of the lesions. The MRI findings in the cases were similar to those of cases previously reported. MRI showed hypo or isointense lesions on T1WI and diffusely hyperintense on proton density and T2WI that were superior to T1WI for evaluating the extent of the lesion. Because of the greater sensitivity of MRI examination in detecting signal changes in the cerebral parenchyma
Fig. 3. (a) Axial CT scan with contrast enhancement shows bilateral diffuse hypodense lesions in frontal, occipital lobes (arrows) with corpus callosum involvement and shift of the midline. (b) Axial T2WI MR (2000/95) scan. Asymmetric bilateral areas of high signal intensity lesion involving gray and white matter in cerebral hemispheres. Shift of midline and sulcal effacement of left hemisphere. Compare, contrast and extent of lesion with that seen in (a).
P. Ponce et al. / European Journal of Radiology 28 (1998) 226–229
infiltrative glioma and inflammatory or infectious processes including viral encephalitis. Clinical history and neurologic findings on examination are important in narrowing this differential. GC requires clinical, radiological and pathological correlation for diagnosis. A review of the literature led to the conclusion that GC is a disease with a principally post-mortem diagnosis. However, the authors suppose that the MRI and biopsy might be decisive in the ante-mortem diagnosis.
References [1] Artigas J, Cervos-Navarro J, Iglesias J, Ebhardt G. Gliomatosis cerebri: clinical and histological findings. Clin Neuropathol 1985;4:135 – 48. [2] Schober R, Mai JK, Volk B, Wechsler W. Gliomatosis cerebri: bioptical approach and neuropathological verification. Acta Neurochir (Vienna) 1991;113:131–7.
.
229
[3] Felsberg GJ, Silver SA, Brown MT, Tien RD. Radiologic– pathologic correlation gliomatosis cerebri. Am J Neororadiol 1994;15:1745 – 53. [4] Couch J, Weiss S. Gliomatosis cerebri. Neurology 1974;24:504– 11. [5] Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumors. Brain Pathol 1993;3:255 – 68. [6] Geremia GK, Wollman R, Foust R. Computed tomography of gliomatosis cerebri. J Comput Tomogr 1988;12:698 – 701. [7] Del Carpio O, Donovan R, Korah I, Salazar A, Melancon D. Gliomatosis cerebri. Radiology 1996;198(3):831– 5. [8] Koslow SA, Claassen D, Hirsch WL, Jungreis CA. Gliomatosis cerebri: a case report with autopsy correlation. Neuroradiology 1992;34:331 – 3. [9] Shin YM, Chang KH, Han MH, Myung NH, Chi JG, Cha SH, Han MC. Gliomatosis cerebri: comparison of MR and CT features. Am J Roentgenol 1993;161:859 – 62. [10] Pyhtinen J, Paakko E. A difficult diagnosis of gliomatosis cerebri. Neuroradiology 1996;38(5):444– 8. [11] Rivas E, Guzman J, Mora La Cruz E, Cardozo Duran J. Gliomatosis cerebri. Ante-mortem diagnosis. Invest Clin 1995;36(3):149– 57.