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Meningeal neurosarcoidosis mimicking convexity en-plaque meningioma
Surg Neurol 1986;26:387-90
387
Meningeal Neurosarcoidosis Mimicking Convexity En-Plaque Meningioma CPT Richard K. Osenbach, MC, USA; MAJ Bennett Blumenkopf, MC, USA; MAJ Hector Ramirez, Jr,, MC, USA; and COLJorge Gutierrez, MC, USA Neurosurgery Service, Department of Surgery; Department of Radiology; and Department of Pathology; Brooke Army Medical Center, Ft. Sam, Houston, Texas
Osenbach RK, Blumenkopf B, Ramirez H Jr, Gutierrez J. Meningeal neurosarcoidosis mimicking convexity en-plaque meningioma. Surg Neurol 1986;26:387-90.
A 34-year old man presented with headaches. Computed tomography scanning revealed an enhancing subdural mass extending from the skull base to the convexity, thought to represent an en-plaque meningioma. Pathologic study revealed extraaxial subdural granulomatous inflammation consistent with neurosarcoidosis. KEYWORDS: Meningioma en plaque; Neurosarcoidosis; Subdural mass; Computed tomography; Headache
a repeat CT scan 1 year later revealed an isodense, homogeneously enhancing leptomeningeal mass that involved the right sphenoid ridge and contiguous convexity (Figure 1). The patient's past medical history was remarkable for an episode of anterior chamber uveitis 7 years earlier, diagosed as Reiter's syndrome. Results of physical examination including a detailed neurologic evaluation were entirely normal. Pertinent laboratory studies included an erythrocyte sedimentation rate of 33 mm/hr, a normal serum calcium level, and negative results from serologic testing for histoplasmosis, blastomycosis and coccidiomycosis. Angiotensin-converting enzyme level was normal. Radiographs of the chest and extremities were also normal.
Central nervous system involvement by sarcoidosis is uncommon. Neurologic manifestations are present in 5 % - 1 6 % of patients [9,13]. The most frequently reported clinical findings are cranial neuropathies, hypothalamic dysfunction, and meningitis [13]. Rarely is the presentation an intracranial mass lesion [2,4-6, 8,11,12,14]. We wish to report the clinical and radiographic features of a patient with neurosarcoidosis who presented with an extraaxial mass initially considered an en-plaque meningioma. Case Report
This 34-year-old, right-handed man presented with a 3-year history of right temporal and retro-orbital headaches. Managed as cluster headaches, they abated on treatment with nonsteroidal, antiinflammatory medications. Cranial computed tomography (CT) scan at that time was reported as normal. After a generalized seizure, A The opinions or assertions contained herein are the private views of the authors and are not to be construed as reflecting the view of the Department of the Army or the DePartment of Defense. Address reprint requests to: Bennett Blumenkopf, M.D., Department of Neurological Surgery, Vanderbilt University Medical Center, T-4224 Medical Center North, Nashville, Tennessee 37232.
Figure 1. (A-C). Enhanced axial computed tomography ¢CT) image~ demonstrate homogeneously enhancing mass extending from the right .*phenoid ridge to the adjacent frontotemporal convexio, along the meninge< (D) Direct coronal CT scan after intravenous enhancement shows the superolateral extension of the leptomeningeal granulomatous mass from the skull base. on the right. Unenhanced brain CT scan was unrevealing. 0090- 3019/86/$0.00
388
Surg Neurol 1986;26:387-90
B
Osenbach et al
D
was a granulomatous pachymeningitis with extensive fibrosis. Intraoperative cultures and special stains were negative for mycobacteria and fungi. T h e final results of histopathologic study were consistent with neurosarcoidosis (Figure 3), and the patient was treated with corticosteroids. At follow-up at 18 months, there was resolution of the mass effect as seen on CT scanning.
Figure 2. Encasement with irregularity of the middle meningeal artery (between arrows) is evident on a subtraction imagefrom the external carotid angiogram.
....
C Figure 1. (continued)
Cerebral angiography demonstrated encasement of the middle meningeal artery (Figure 2). A clinical diagnosis o f a sphenoid wing and convexity en-plaque meningioma was made. At the time of craniotomy, a subdural fibrous mass attached to the underlying pia-arachnoid was encountered. The specimen obtained consisted of dura mater with a nodular tumoral thickening of up to 0.7 cm on its inner surface. Microscopically, the pathologic process
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390
Surg Neurol 1986;26:387-90
Discussion Central nervous system involvement by sarcoidosis is uncommon, but well recognized. Neurologic symptoms occur in about 5% of patients, and in these the majority exhibit systemic involvement [12,13]. In about half of these patients, the neurological symptoms may be the presenting manifestation. Neurosarcoidosis affects younger patients and usually heralds a more aggressive course of the disease with a rather poor response to therapy [10]. Besides cranial neuropathies (peripheral facial palsy being most common), symptoms of aseptic meningitis, hydrocephalus, and hypothalamic dysfunction also occur [8,13]. A literature review by Powers and Miller [12] yielded report of 30 lesions in 22 cases that presented as intracranial masses [2,14], mostly intraparenchymal in location. Only 3 cases involved the convexity meninges and, therefore, would be expected to resemble the present case. The typical CT image of neurosarcoidosis is filling of the basal cisterns by slightly hyperdense granulomatous tissue that enhances homogeneously without significant mass effect or parenchymal edema. Hydrocephalus is the most common CT finding, occurring in the majority of patients secondary to the adhesive arachnoiditis that results from meningeal involvement [ 1,2,7]. Intracranial sarcoid masses were previously considered the least common presentation. However, a review of the CT literature reveals this type of presentation in over half of the cases reported [2]. A mass lesion of the posterior fossa has also been described [14]. Cortical enhancement involving the temporal regions has been reported without mass effect [1,3]. As in our patient, other reports of CT scanning at the time of initial presentation for suspected neurosarcoidosis have been unremarkable, requiring follow-up contrast-enhanced examinations for detection [2]. Although the majority of intracranial sarcoid masses are intraparenchymal, over 20% have presented as extraaxial mass lesions [2,5,6,12]. A subdurally located mass resembled a subdural hematoma [4]. In their series of 12 patients, Brooks et al demonstrated by CT scanning a prechiasmal mass that mimicked a cribriform plate meningioma [2]. Several cases of optic nerve involvement have demonstrated CT features that were mistaken fi)r those of optic nerve sheath meningioma [5]. Our case illustrates that masslike meningeal involvement may
Osenbach et al
extend from the base of the skull to involve the lateral side wall and convexity of the calvarium and the meningeal vasculature, resembling an extensive en-plaque meningioma. Due to the variability of CT findings in neurosarcoidosis, it cannot be reliably distinguished from neoplasm [12]. The differential diagnosis of an intracranial mass lesion in a patient with known or suspected sarcoidosis should include neurosarcoidosis, even when the lesion extends extraaxially from the basal cisterns to involve the convexity.
Special thanks to Betty Lyell for typing the manuscript.
References 1. Bahr AL, Krumholz A, Kristt D, Hodges FJ. Neuroradiological manifestations of intracranial sarcoidosis. Radiology 1978;127: 713-7. 2. Brooks BS, Gammal TE, Hungerford GD, Acker J, Trevor RP, Russell W. Radiologic evaluation of neurosarcoidosis: role of computed tomography. AJNR 1982;3:513-21. 3. Brooks J, Strickland MC, Williams JP, Vulpe M, Fowler HL. Computed tomography changes in neurosarcoidosis clearing with steroid treatment. J Comput Assist Tomogr 1979;3(3):398-9. 4. de Tribolet N, Zander E. Intracranial sarcoidosis presenting angiographically as a subdural hematoma. Surg Neurol 1978;9:169-71. 5. Gudeman SK, SelhorstJB, SasacJO, Waybright EA. Sarcoid optic neuropathy. Neurology 1982;32:597-603. 6. Healton EB, Zito G, Chauhan P, BrustJCM. Intracranial subdural sarcoid granuloma. J Neurosurg 1982;56:728-31. 7. Kendall BE, Tatler GLV. Radiological findings in neurosarcoidosis. Br J Radiol 1978;51:81-92. 8. Kumpe DA, Rao CVGK, GarciaJH, Heck AF. Intracranial neurosarcoidosis. J Comput Assist Tomogr 1979;3(3):324-30. 9. Lee SH. Infectious diseases. In: Lee SH, Rao CVGK. Cranial computed tomography. New York: McGraw-Hill, 1983:529-30. 10. ManzHJ. Pathobiology ofneurosarcoidosis and clinicopathologic correlation. Can J Neurol Sci 1983;10:50-5. 11. Post MJD, Quencer RM, Tabei SZ. CT demonstration of sarcoidosis of the optic nerve, frontal lobes, and falx cerebri: case report and literature review. AJNR 1982;3:523-6. 12. PowersWJ, Miller EM. Sarcoidosismimickingglioma: casereport and review of intracranial sarcoid mass lesions. Neurology 1981;31:907-10. 13. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol 1985;42:909-17. 14. Whelan MA, Stern J. Sarcoidosis presenting as a posterior fossa mass. Surg Neurol 1981;15:455-7.
387
Meningeal Neurosarcoidosis Mimicking Convexity En-Plaque Meningioma CPT Richard K. Osenbach, MC, USA; MAJ Bennett Blumenkopf, MC, USA; MAJ Hector Ramirez, Jr,, MC, USA; and COLJorge Gutierrez, MC, USA Neurosurgery Service, Department of Surgery; Department of Radiology; and Department of Pathology; Brooke Army Medical Center, Ft. Sam, Houston, Texas
Osenbach RK, Blumenkopf B, Ramirez H Jr, Gutierrez J. Meningeal neurosarcoidosis mimicking convexity en-plaque meningioma. Surg Neurol 1986;26:387-90.
A 34-year old man presented with headaches. Computed tomography scanning revealed an enhancing subdural mass extending from the skull base to the convexity, thought to represent an en-plaque meningioma. Pathologic study revealed extraaxial subdural granulomatous inflammation consistent with neurosarcoidosis. KEYWORDS: Meningioma en plaque; Neurosarcoidosis; Subdural mass; Computed tomography; Headache
a repeat CT scan 1 year later revealed an isodense, homogeneously enhancing leptomeningeal mass that involved the right sphenoid ridge and contiguous convexity (Figure 1). The patient's past medical history was remarkable for an episode of anterior chamber uveitis 7 years earlier, diagosed as Reiter's syndrome. Results of physical examination including a detailed neurologic evaluation were entirely normal. Pertinent laboratory studies included an erythrocyte sedimentation rate of 33 mm/hr, a normal serum calcium level, and negative results from serologic testing for histoplasmosis, blastomycosis and coccidiomycosis. Angiotensin-converting enzyme level was normal. Radiographs of the chest and extremities were also normal.
Central nervous system involvement by sarcoidosis is uncommon. Neurologic manifestations are present in 5 % - 1 6 % of patients [9,13]. The most frequently reported clinical findings are cranial neuropathies, hypothalamic dysfunction, and meningitis [13]. Rarely is the presentation an intracranial mass lesion [2,4-6, 8,11,12,14]. We wish to report the clinical and radiographic features of a patient with neurosarcoidosis who presented with an extraaxial mass initially considered an en-plaque meningioma. Case Report
This 34-year-old, right-handed man presented with a 3-year history of right temporal and retro-orbital headaches. Managed as cluster headaches, they abated on treatment with nonsteroidal, antiinflammatory medications. Cranial computed tomography (CT) scan at that time was reported as normal. After a generalized seizure, A The opinions or assertions contained herein are the private views of the authors and are not to be construed as reflecting the view of the Department of the Army or the DePartment of Defense. Address reprint requests to: Bennett Blumenkopf, M.D., Department of Neurological Surgery, Vanderbilt University Medical Center, T-4224 Medical Center North, Nashville, Tennessee 37232.
Figure 1. (A-C). Enhanced axial computed tomography ¢CT) image~ demonstrate homogeneously enhancing mass extending from the right .*phenoid ridge to the adjacent frontotemporal convexio, along the meninge< (D) Direct coronal CT scan after intravenous enhancement shows the superolateral extension of the leptomeningeal granulomatous mass from the skull base. on the right. Unenhanced brain CT scan was unrevealing. 0090- 3019/86/$0.00
388
Surg Neurol 1986;26:387-90
B
Osenbach et al
D
was a granulomatous pachymeningitis with extensive fibrosis. Intraoperative cultures and special stains were negative for mycobacteria and fungi. T h e final results of histopathologic study were consistent with neurosarcoidosis (Figure 3), and the patient was treated with corticosteroids. At follow-up at 18 months, there was resolution of the mass effect as seen on CT scanning.
Figure 2. Encasement with irregularity of the middle meningeal artery (between arrows) is evident on a subtraction imagefrom the external carotid angiogram.
....
C Figure 1. (continued)
Cerebral angiography demonstrated encasement of the middle meningeal artery (Figure 2). A clinical diagnosis o f a sphenoid wing and convexity en-plaque meningioma was made. At the time of craniotomy, a subdural fibrous mass attached to the underlying pia-arachnoid was encountered. The specimen obtained consisted of dura mater with a nodular tumoral thickening of up to 0.7 cm on its inner surface. Microscopically, the pathologic process
~
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~
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.... !~i~: ~,ii!~i~i! ! ~
'~
i~ iiii
@
i~¸~¸,I
I
@
390
Surg Neurol 1986;26:387-90
Discussion Central nervous system involvement by sarcoidosis is uncommon, but well recognized. Neurologic symptoms occur in about 5% of patients, and in these the majority exhibit systemic involvement [12,13]. In about half of these patients, the neurological symptoms may be the presenting manifestation. Neurosarcoidosis affects younger patients and usually heralds a more aggressive course of the disease with a rather poor response to therapy [10]. Besides cranial neuropathies (peripheral facial palsy being most common), symptoms of aseptic meningitis, hydrocephalus, and hypothalamic dysfunction also occur [8,13]. A literature review by Powers and Miller [12] yielded report of 30 lesions in 22 cases that presented as intracranial masses [2,14], mostly intraparenchymal in location. Only 3 cases involved the convexity meninges and, therefore, would be expected to resemble the present case. The typical CT image of neurosarcoidosis is filling of the basal cisterns by slightly hyperdense granulomatous tissue that enhances homogeneously without significant mass effect or parenchymal edema. Hydrocephalus is the most common CT finding, occurring in the majority of patients secondary to the adhesive arachnoiditis that results from meningeal involvement [ 1,2,7]. Intracranial sarcoid masses were previously considered the least common presentation. However, a review of the CT literature reveals this type of presentation in over half of the cases reported [2]. A mass lesion of the posterior fossa has also been described [14]. Cortical enhancement involving the temporal regions has been reported without mass effect [1,3]. As in our patient, other reports of CT scanning at the time of initial presentation for suspected neurosarcoidosis have been unremarkable, requiring follow-up contrast-enhanced examinations for detection [2]. Although the majority of intracranial sarcoid masses are intraparenchymal, over 20% have presented as extraaxial mass lesions [2,5,6,12]. A subdurally located mass resembled a subdural hematoma [4]. In their series of 12 patients, Brooks et al demonstrated by CT scanning a prechiasmal mass that mimicked a cribriform plate meningioma [2]. Several cases of optic nerve involvement have demonstrated CT features that were mistaken fi)r those of optic nerve sheath meningioma [5]. Our case illustrates that masslike meningeal involvement may
Osenbach et al
extend from the base of the skull to involve the lateral side wall and convexity of the calvarium and the meningeal vasculature, resembling an extensive en-plaque meningioma. Due to the variability of CT findings in neurosarcoidosis, it cannot be reliably distinguished from neoplasm [12]. The differential diagnosis of an intracranial mass lesion in a patient with known or suspected sarcoidosis should include neurosarcoidosis, even when the lesion extends extraaxially from the basal cisterns to involve the convexity.
Special thanks to Betty Lyell for typing the manuscript.
References 1. Bahr AL, Krumholz A, Kristt D, Hodges FJ. Neuroradiological manifestations of intracranial sarcoidosis. Radiology 1978;127: 713-7. 2. Brooks BS, Gammal TE, Hungerford GD, Acker J, Trevor RP, Russell W. Radiologic evaluation of neurosarcoidosis: role of computed tomography. AJNR 1982;3:513-21. 3. Brooks J, Strickland MC, Williams JP, Vulpe M, Fowler HL. Computed tomography changes in neurosarcoidosis clearing with steroid treatment. J Comput Assist Tomogr 1979;3(3):398-9. 4. de Tribolet N, Zander E. Intracranial sarcoidosis presenting angiographically as a subdural hematoma. Surg Neurol 1978;9:169-71. 5. Gudeman SK, SelhorstJB, SasacJO, Waybright EA. Sarcoid optic neuropathy. Neurology 1982;32:597-603. 6. Healton EB, Zito G, Chauhan P, BrustJCM. Intracranial subdural sarcoid granuloma. J Neurosurg 1982;56:728-31. 7. Kendall BE, Tatler GLV. Radiological findings in neurosarcoidosis. Br J Radiol 1978;51:81-92. 8. Kumpe DA, Rao CVGK, GarciaJH, Heck AF. Intracranial neurosarcoidosis. J Comput Assist Tomogr 1979;3(3):324-30. 9. Lee SH. Infectious diseases. In: Lee SH, Rao CVGK. Cranial computed tomography. New York: McGraw-Hill, 1983:529-30. 10. ManzHJ. Pathobiology ofneurosarcoidosis and clinicopathologic correlation. Can J Neurol Sci 1983;10:50-5. 11. Post MJD, Quencer RM, Tabei SZ. CT demonstration of sarcoidosis of the optic nerve, frontal lobes, and falx cerebri: case report and literature review. AJNR 1982;3:523-6. 12. PowersWJ, Miller EM. Sarcoidosismimickingglioma: casereport and review of intracranial sarcoid mass lesions. Neurology 1981;31:907-10. 13. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol 1985;42:909-17. 14. Whelan MA, Stern J. Sarcoidosis presenting as a posterior fossa mass. Surg Neurol 1981;15:455-7.