Neuro-radiology
Neuroradiology(1992) 34:331-333
9 Springer-Verlag1992
Gliomatosis cerebri: a case report with autopsy correlation S. A. K o s i o w 1, D. Claassen 2, W. L. Hirsch ~, and C. A. Jungreis 1
Departments of 1Radiology, 2Neuropathology,Universityof Pittsburgh, MedicalCenter DeSoto & O'Hara Streets, Pittsburgh,PA 15213, USA Received: 1 March1991
Summary. MRI-autopsy correlation in a case of glioma-
tosis cerebri suggests that poor gray-white matter demarcation on MRI may be sign of neoplastic infiltration. The extent of infiltration is imperfectly assessed by current imaging modalities. K e y words: Glioma - Computed tomography - Magnetic
resonance imaging
Gliomatosis cerebri is a rare form of diffuse glioma that infiltrates the central nervous system extensively without greatly altering the gross neural architecture [1-3]. We present a case of gliomatosis cerebri, correlating autopsy findings with antemortem computed tomography (CT), and magnetic resonance imaging (MRI), and in vitro postmortem MRI. The time interval between in vivo MRI and death was less than 2 weeks.
Case report
A 41-year-old woman developed headaches, vomiting, blurred vision, dizziness, and episodic blackouts over the few weeks prior to admission. Examination revealed bilateral papilledema, left leg hyperreflexia with a flexor plantar response, and left arm and leg ataxia. CT demonstrated diffuse cerebral swelling with small lateral ventricles, bilateral effacement of sulci, and obliteration of the basal cisterns (Fig. 1). MRI at 1.5 T disclosed a 4-cm peritrigonal mass, hypointense on short TR and hyperintense on long TR spin-echo (SE) sequences (Fig. 2). The gray-white matter interface was poorly defined throughout the ipsilateral cerebral hemisphere and the contralateral parietal and occipital lobes; this was best demonstrated on the long TR/short TE SE sequence (Fig. 3). The basal ganglia, left temporal and frontal lobes, and cerebellum appeared normal. CT-guided stereotactic biopsy of the mass revealed a glial neoplasm with characteristics of anaplastic astrocytoma. The patient was discharged for outpatient radiation
therapy but was readmitted moribund with evidence of transtentorial herniation 4 days later. She died 24 h thereafter, 5 weeks after the onset of symptoms, and 13 days after the MRI. Postmortem MRI of the formalin-fixed brain in vitro showed the high signal periatrial mass with a similar appearance to that on the in vivo study (Fig. 4), but obscuration of the gray-white matter interface, a prominent feature of the clinical MRI study, was less apparent. Histopathologic examination showed a diffusely infiltrative anaplastic astrocytoma, involving the gray and white matter of the cerebral hemispheres, cerebellum and brain stem. The regions of highest cellular density were the right basal ganglia and upper brain stem. There was no necrosis, mitoses, or vascular endothelial proliferation. Pleomorphic astrocytes infiltrated the regions of poor gray-white matter differentiation identified on antemortem MRI, as well as areas that appeared normal on both in vivo and in vitro MRI, such as the right corpus striatum, thalamus, cerebellum, and brain stem. Neoplastic astrocytes were seen as far away as the cervical spinal cord.
Discussion
Gliomatosis cerebri is an unusual form of glial neoplasm that is difficult to quantify antemortem [3]. Histologicall3; gliomatosis cerebri comprises astrocytic elements with varying degrees of pleomorphism and small round cells of glial origin [1,4-8]. Central nervous system involvement is widespread and diffuse: both cerebral hemispheres, the brain stem, cerebellum, and even the spinal cord will often be infiltrated by pleomorphic cells. The incidence of gliomatosis cerebri is greatest in the third and fourth decades, although all age groups may be affected [1, 3, 4]. Clinical manifestations commonly include early mental deterioration, personality changes, seizures, and signs of elevated intracranial pressure [1, 3]. Focal neurologic signs appear late in the course of the disease. Cerebrospinal fluid analysis is usually normal [1].
332
Fig. 1. Contrast-enhanced CT shows effacement of the cerebral sulci and a vague area of increased density in the corpus callosum also seen before injection (arrow). There is no pathological enhancement Fig.2. Coronal SE (3000/25) MRI shows a high-signal mass in the posterior right cerebral hemisphere (arrow). The cerebellum appears normal Fig.3. Coronal SE (3000/25) MRI of frontal and temporal lobes demonstrates loss of the gray-white matter interface throughout the right hemisphere (arrows). Gray-white matter demarcation is relatively preserved in the left temporal lobe
Imaging studies frequently underestimate the extent of disease. Angiography m a y be normal or demonstrate an avascular mass [1]. CT m a y be normal but usually shows diffuse swelling. T h e r e m a y be a localized mass in areas of very dense neoplastic infiltration. W h e n a focal mass is present, it is difficult to differentiate gfiomatosis cerebri from m o r e typical glial neoplasms. E n h a n c e m e n t is usually minimal or absent, suggesting that the blood-brain barrier is relatively preserved [2, 9]. However, e n h a n c e m e n t can occur in late stages of the disease [2] and m a y even have an unusual leptomeningeal appearance on M R I [10]. The M R I characteristics of gliomatosis cerebri in vivo have rarely been correlated with p o s t m o r t e m M R I or gross pathology [9-11]. In the single prior report of autopsy correlation, the patient's clinical course was protracted [10]. The short interval between M R I and death in the present case permits m o r e confident correlation of imaging features with gross pathology. Areas of dense neoplastic proliferation a p p e a r e d as a focal high-signal mass lesion indistin-
Fig.4. In vitro MRI (2500/25) of the formalin-fixed specimen. The high signal mass involes the corpus callosum and cingulate gyrus (arrows). Gray-white matter demarcation is relatively preserved compared with the in vivo images Fig. 5. Coronal section of the gross specimen reveals compression of the right lateral ventricle by the mass. Areas of poor gray-white matter demarcation (arrows) are less evident on gross examination than on in vivo MRI (cf. Fig. 3)
guishable from other primary brain neoplasms. Less cellular areas of neoplastic infiltration were seen as loss of the gray-white m a t t e r interface on MRI. Loss of gray-white matter demarcation has not previously b e e n described as a feature of gliomatosis cerebri on MRI, but is a characteristic feature at gross examination of the cut brain surface [1, 7, 10]. In our case, subtle loss of the gray-white matter distinction was the only indication of the very extensive nature of the neoplasm on a n t e m o r t e m MRI. Long TR/short T E SE sequences best displayed this subtle sign. M R I underestimates the extent of neoplasm in gliomatosis cerebri; areas of less dense neoplastic infiltration m a y a p p e a r completely normal by in vivo and in vitro MRI. M R I 2 weeks before death failed to demonstrate the neoplastic involvement of the brain stem and cerebellum found at autopsy. The loss of gray-white matter demarcation on the antem o r t e m M R I was poorly demonstrated by in vitro postm o r t e m M R I examination, perhaps because of artifactual
333 s h o r t e n i n g of T1 in t h e f o r m a l i n - f i x e d g r a y m a t t e r . T h e dis c r e p a n c y b e t w e e n t h e in vivo a n d in vitro i m a g e s raises d o u b t s a b o u t t h e v a l i d i t y of p o s t m o r t e m i m a g i n g in d e l i n e a t i n g t h e e x t e n t of glial n e o p l a s m s .
Acknowledgements. We thank Dara Tomasi for typing this manuscript and Meg Sachse for editing.
References 1. Couch JR, Weiss SA (1974) Gliomatosis cerebri, report of four cases and review of the literature. Neurology 24:504-511 2. Hayek J, Valavanis A (1982) Computed tomography of gliomatosis cerebri. Comput Radiol 6:93-98 3. Sarhaddi S, Bravo E, Cyrus A E (1973) Gliomatosis cerebri: a case report and review of the literature. South Med J 66:883-888 4. Scheinker IM, Evans JP (1943) Diffuse cerebral glioblastosis. J Neuropathol Exp Neurol 2:178-189 5. Cervos-Navarro J, Artigas J, Aruffo C, Iglesias J (1987) The fine structure of gliomatosis cerebri. Virchows Arch [A] 411:93-98
6. Dunn J, Kernohan JW (1957) Gliomatosis cerebri. A M A Arch Patho164:82-91 7. Malmud N, Wise BL, Jones OW (1952) Gliomatosis cerebri. J Neurosurg 9:409417 8. Nevin S (1938) Gliomatosis cerebri. Brain 61:170-191 9. Spagnoli MV, Grossman RI, Packer RJ (1987) Magnetic resonance imaging determination of gliomatosis cerebri. Neuroradiology 29:15-18 10. Rippe DJ, Boyko OB, Fuller GN, Friedman HS, Oakes WJ, Schold SC (1990) Gadopentetate-dimeglumine-enhanced MR imaging of gliomatosis cerebri: appearance mimicking leptomeningeal tumor dissemination. A JR 11:800-801 11. Troost D, Kuper H, Valk J, Fleury P (1987) Gliomatois cerebri: report of a clinicallydiagnosed and histologicallyconfirmed case. Clin Neurol Neurosurg 89:43-47
Dr. S. A. Koslow Department of Radiology University of Pittsburgh Medical Center De Soto & O'Hara Streets Pittsburgh, PA 15 213, USA
Neuroradiology(1992) 34:331-333
9 Springer-Verlag1992
Gliomatosis cerebri: a case report with autopsy correlation S. A. K o s i o w 1, D. Claassen 2, W. L. Hirsch ~, and C. A. Jungreis 1
Departments of 1Radiology, 2Neuropathology,Universityof Pittsburgh, MedicalCenter DeSoto & O'Hara Streets, Pittsburgh,PA 15213, USA Received: 1 March1991
Summary. MRI-autopsy correlation in a case of glioma-
tosis cerebri suggests that poor gray-white matter demarcation on MRI may be sign of neoplastic infiltration. The extent of infiltration is imperfectly assessed by current imaging modalities. K e y words: Glioma - Computed tomography - Magnetic
resonance imaging
Gliomatosis cerebri is a rare form of diffuse glioma that infiltrates the central nervous system extensively without greatly altering the gross neural architecture [1-3]. We present a case of gliomatosis cerebri, correlating autopsy findings with antemortem computed tomography (CT), and magnetic resonance imaging (MRI), and in vitro postmortem MRI. The time interval between in vivo MRI and death was less than 2 weeks.
Case report
A 41-year-old woman developed headaches, vomiting, blurred vision, dizziness, and episodic blackouts over the few weeks prior to admission. Examination revealed bilateral papilledema, left leg hyperreflexia with a flexor plantar response, and left arm and leg ataxia. CT demonstrated diffuse cerebral swelling with small lateral ventricles, bilateral effacement of sulci, and obliteration of the basal cisterns (Fig. 1). MRI at 1.5 T disclosed a 4-cm peritrigonal mass, hypointense on short TR and hyperintense on long TR spin-echo (SE) sequences (Fig. 2). The gray-white matter interface was poorly defined throughout the ipsilateral cerebral hemisphere and the contralateral parietal and occipital lobes; this was best demonstrated on the long TR/short TE SE sequence (Fig. 3). The basal ganglia, left temporal and frontal lobes, and cerebellum appeared normal. CT-guided stereotactic biopsy of the mass revealed a glial neoplasm with characteristics of anaplastic astrocytoma. The patient was discharged for outpatient radiation
therapy but was readmitted moribund with evidence of transtentorial herniation 4 days later. She died 24 h thereafter, 5 weeks after the onset of symptoms, and 13 days after the MRI. Postmortem MRI of the formalin-fixed brain in vitro showed the high signal periatrial mass with a similar appearance to that on the in vivo study (Fig. 4), but obscuration of the gray-white matter interface, a prominent feature of the clinical MRI study, was less apparent. Histopathologic examination showed a diffusely infiltrative anaplastic astrocytoma, involving the gray and white matter of the cerebral hemispheres, cerebellum and brain stem. The regions of highest cellular density were the right basal ganglia and upper brain stem. There was no necrosis, mitoses, or vascular endothelial proliferation. Pleomorphic astrocytes infiltrated the regions of poor gray-white matter differentiation identified on antemortem MRI, as well as areas that appeared normal on both in vivo and in vitro MRI, such as the right corpus striatum, thalamus, cerebellum, and brain stem. Neoplastic astrocytes were seen as far away as the cervical spinal cord.
Discussion
Gliomatosis cerebri is an unusual form of glial neoplasm that is difficult to quantify antemortem [3]. Histologicall3; gliomatosis cerebri comprises astrocytic elements with varying degrees of pleomorphism and small round cells of glial origin [1,4-8]. Central nervous system involvement is widespread and diffuse: both cerebral hemispheres, the brain stem, cerebellum, and even the spinal cord will often be infiltrated by pleomorphic cells. The incidence of gliomatosis cerebri is greatest in the third and fourth decades, although all age groups may be affected [1, 3, 4]. Clinical manifestations commonly include early mental deterioration, personality changes, seizures, and signs of elevated intracranial pressure [1, 3]. Focal neurologic signs appear late in the course of the disease. Cerebrospinal fluid analysis is usually normal [1].
332
Fig. 1. Contrast-enhanced CT shows effacement of the cerebral sulci and a vague area of increased density in the corpus callosum also seen before injection (arrow). There is no pathological enhancement Fig.2. Coronal SE (3000/25) MRI shows a high-signal mass in the posterior right cerebral hemisphere (arrow). The cerebellum appears normal Fig.3. Coronal SE (3000/25) MRI of frontal and temporal lobes demonstrates loss of the gray-white matter interface throughout the right hemisphere (arrows). Gray-white matter demarcation is relatively preserved in the left temporal lobe
Imaging studies frequently underestimate the extent of disease. Angiography m a y be normal or demonstrate an avascular mass [1]. CT m a y be normal but usually shows diffuse swelling. T h e r e m a y be a localized mass in areas of very dense neoplastic infiltration. W h e n a focal mass is present, it is difficult to differentiate gfiomatosis cerebri from m o r e typical glial neoplasms. E n h a n c e m e n t is usually minimal or absent, suggesting that the blood-brain barrier is relatively preserved [2, 9]. However, e n h a n c e m e n t can occur in late stages of the disease [2] and m a y even have an unusual leptomeningeal appearance on M R I [10]. The M R I characteristics of gliomatosis cerebri in vivo have rarely been correlated with p o s t m o r t e m M R I or gross pathology [9-11]. In the single prior report of autopsy correlation, the patient's clinical course was protracted [10]. The short interval between M R I and death in the present case permits m o r e confident correlation of imaging features with gross pathology. Areas of dense neoplastic proliferation a p p e a r e d as a focal high-signal mass lesion indistin-
Fig.4. In vitro MRI (2500/25) of the formalin-fixed specimen. The high signal mass involes the corpus callosum and cingulate gyrus (arrows). Gray-white matter demarcation is relatively preserved compared with the in vivo images Fig. 5. Coronal section of the gross specimen reveals compression of the right lateral ventricle by the mass. Areas of poor gray-white matter demarcation (arrows) are less evident on gross examination than on in vivo MRI (cf. Fig. 3)
guishable from other primary brain neoplasms. Less cellular areas of neoplastic infiltration were seen as loss of the gray-white m a t t e r interface on MRI. Loss of gray-white matter demarcation has not previously b e e n described as a feature of gliomatosis cerebri on MRI, but is a characteristic feature at gross examination of the cut brain surface [1, 7, 10]. In our case, subtle loss of the gray-white matter distinction was the only indication of the very extensive nature of the neoplasm on a n t e m o r t e m MRI. Long TR/short T E SE sequences best displayed this subtle sign. M R I underestimates the extent of neoplasm in gliomatosis cerebri; areas of less dense neoplastic infiltration m a y a p p e a r completely normal by in vivo and in vitro MRI. M R I 2 weeks before death failed to demonstrate the neoplastic involvement of the brain stem and cerebellum found at autopsy. The loss of gray-white matter demarcation on the antem o r t e m M R I was poorly demonstrated by in vitro postm o r t e m M R I examination, perhaps because of artifactual
333 s h o r t e n i n g of T1 in t h e f o r m a l i n - f i x e d g r a y m a t t e r . T h e dis c r e p a n c y b e t w e e n t h e in vivo a n d in vitro i m a g e s raises d o u b t s a b o u t t h e v a l i d i t y of p o s t m o r t e m i m a g i n g in d e l i n e a t i n g t h e e x t e n t of glial n e o p l a s m s .
Acknowledgements. We thank Dara Tomasi for typing this manuscript and Meg Sachse for editing.
References 1. Couch JR, Weiss SA (1974) Gliomatosis cerebri, report of four cases and review of the literature. Neurology 24:504-511 2. Hayek J, Valavanis A (1982) Computed tomography of gliomatosis cerebri. Comput Radiol 6:93-98 3. Sarhaddi S, Bravo E, Cyrus A E (1973) Gliomatosis cerebri: a case report and review of the literature. South Med J 66:883-888 4. Scheinker IM, Evans JP (1943) Diffuse cerebral glioblastosis. J Neuropathol Exp Neurol 2:178-189 5. Cervos-Navarro J, Artigas J, Aruffo C, Iglesias J (1987) The fine structure of gliomatosis cerebri. Virchows Arch [A] 411:93-98
6. Dunn J, Kernohan JW (1957) Gliomatosis cerebri. A M A Arch Patho164:82-91 7. Malmud N, Wise BL, Jones OW (1952) Gliomatosis cerebri. J Neurosurg 9:409417 8. Nevin S (1938) Gliomatosis cerebri. Brain 61:170-191 9. Spagnoli MV, Grossman RI, Packer RJ (1987) Magnetic resonance imaging determination of gliomatosis cerebri. Neuroradiology 29:15-18 10. Rippe DJ, Boyko OB, Fuller GN, Friedman HS, Oakes WJ, Schold SC (1990) Gadopentetate-dimeglumine-enhanced MR imaging of gliomatosis cerebri: appearance mimicking leptomeningeal tumor dissemination. A JR 11:800-801 11. Troost D, Kuper H, Valk J, Fleury P (1987) Gliomatois cerebri: report of a clinicallydiagnosed and histologicallyconfirmed case. Clin Neurol Neurosurg 89:43-47
Dr. S. A. Koslow Department of Radiology University of Pittsburgh Medical Center De Soto & O'Hara Streets Pittsburgh, PA 15 213, USA
