Acta Neuropathol (1992) 84:342 - 345
Acta Neumpathologtca (~) Springer-Verlag 1992
Secretory meningioma associated with numerous meningothelial rosettes Tsuyoshi Tada l, Keiko Ishii 2, Syuichi Oshima 1, Hideaki Hara 1, and Shigeaki Kobayashi 1 Department of 1Neurosurgery, and 2Laboratory Medicine, Sbinsbu University School of Medicine, Asahi 341-1, Matsumoto 390, Japan Received December 19, 1992/Revised, accepted March 17, 1992
Summary. A case of secretory m e n i n g i o m a with numerous meningothelial rosettes is reported. A 66-year-old m a n with m o y a m o y a disease gradually developed skull deformity, and underwent surgery for the skull t u m o r overlying the hemisphere. Histological e x a m i n a t i o n disclosed n u m e r o u s meningothelial rosettes quite similar to those induced by subarachnoid injection of epinephrine and p s e u d o p s a m m o m a bodies described by Kepes. This m a y be the first case of m e n i n g i o m a associated with n u m e r o u s rosette formations. Key words: Secretory m e n i n g i o m a - Rosette - Moyam o y a vessel
Although rosette formations are often seen in neuroect o d e r m a l tumors, they are rare in meningiomas. We recently experienced a case of secretory m e n i n g i o m a associated with m o y a m o y a disease. T h e t u m o r had n u m e r o u s rosette formations, which are quite similar to meningothelial rosettes induced by the subarachnoid injection of epinephrine in dogs [7].
residual tumor rapidly grew in the following 3 months to invade the scalp over the superior sagittal sinus. Surgery was repeated to resect the tumor from the superior sagittal sinus. Postoperative radiation was given locally (50 Gy). There has been no signs of recurrence for 1 year after the second surgery.
Materials and methods For light microscopy, the specimens were fixed in 10 % buffered formalin, and paraffin-embedded sections were stained with diastase-treated periodic acid-Schiff (d-PAS) and hematoxylineosin (H&E). Immunohistochemical staining was performed with the avidin-biotin complex technique using monoclonal antibodies for vimentin (Dako Corporation, Santa Barbara, Calif.), epithelial membrane antigen (EMA; Becton Dickinson Immunocytometry Systems, San Jose, Calif.), factor VIII-related antigen (Dako) carcinoembryonic antigen (CEA; Dako), chromogranin A (Dako), cytokeratin (CAM 5.2; Becton Dickinson) and S-100 protein (Dako). Human intestine was used as a positive control. For electron microscopy, the specimens were fixed in 2.5 % glutaraldehyde and 1% OsO4, and were embedded in Epon. Ultrathin sections were stained with uranyl acetate and lead citrate, and were examined with Hitachi HS-9 electron microscoPY. Frozen sections of the specimens were processed for glyoxylic acid fluorescence method [4] to detect catecholamine in the tissue.
Case history A 66-year-old man had gradually developed skull deformity over a period of 6 months, and was admitted to our hospital for examination and operation. Skull X-ray showed an extensive decalcification of the left calvaria. Computed tomography scan showed a large isodensity mass overlying the left hemisphere, which was well enhanced with contrast medium. Magnetic resonance imaging showed a crescent mass over the left hemisphere, extending to the superior sagittal sinus, frontal air sinus and temporatis muscle (Fig. la). Cerebral angiography showed that the tumor was hypervascular and there were moyamoya vessels in the basal ganglia (Fig. l b ) . T h e tumor was almost totally removed except the portion of the tumor invading the superior sagittal sinus. The
Correspondence to: T. Tada (address see above)
Results O n light microscopy, meningothelial cells with a distinct nucleolus often showed rosette formations. Although the nuclei showed a slight degree of p l e o m o r p h i s m , there was no mitosis (Fig. 2) . N u m e r o u s eosinophilic inclusion bodies were distributed within and a m o n g the t u m o r cells, and stained positive for d-PAS staining (Fig. 3). Immunohistochemically, the t u m o r cells and inclusion bodies were positive for vimentin, but negative for E M A , factor V I I I - r e l a t e d antigen, C E A , chromogranin A, cytokeratin and S-100 protein.
343
Fig. 1. a Coronal magnetic resonance imaging showing a huge meningioma on the left parietal lobe. The tumor is located mainly in the epidural space, invading the superior sagittal sinus and temporalis muscle, b Angiogram showing stenosis of bilateral carotid arteries and moyamoya vessels Fig. 2. Photomicrograph showing numerous rosette formations of meningothelial cells. H&E, x 265 Fig. 3. Photomicrograph demonstrating PAS-positive inclusion bodies distributed intra- and intercellularly. Diastase-treated PAS, • 712
Electron microscopy
Discussion
The tumor cells showed ultrastructural features compatible with meningioma such as desmosomes, interdigitations and intermediate filaments (Fig. 4a). Glycogen granules and mitochondria were abundant with in the cytoplasm. The cytoplasm also had an inclusion coated with microvilli, the size of which was consistent with an eosinophilic inclusion body on light microscopy (Fig. 4b).
It is widely accepted that meningiomas derive from arachnoidal cap cells [8]. They show various mesenchyreal and epithelial features such as well-developed desmosomes [3]. A variety of histological features of meninges can be explained by the earlier report that the meninges consist of the cells of mesodermal, neural crest and neural tube origin [5]. Secretory meningiomas have a strong tendency for epithelial differentiation, and accumulate secretory materials in the form of hyaline inclusions. They are called pseudopsammoma bodies and are easily confirmed by PAS staining. ImmunohistochemicaUy, the tumor cells and inclusion bodies are usually positive for C E A , E M A and occasionally positive for cytokeratin [1]. Ultrastructurally, lumina coy-
Catecholamine histofluorescence No catecholamine histofluorescence was detected within the specimen studied.
345 Fig. 4. a Electron micrograph showing desmosomes, interdigitations and intermediate filaments. Glycogen granules and mitochondria were abundant in the cytoplasm, b Intracellular lumina coated with microvilli, a • 11,400, b x 22,800
ered with microvilli are observed within and among the tumor cells [1]. In the present case, although the tumor cells and inclusion bodies were negative for C E A , E M A and cytokeratin, positive staining of the inclusion bodies for PAS and the ultrastructural features are characteristics of secretory meningiomas. Meningiomas rarely show rosette formations [2, 3]. The present report describes the first case of a secretory meningioma with numerous rosettes. Meningothelial rosettes were experimentally induced in the canine meninges by subarachnoid injection of epinephrine [7, 9]. The experimentally induced rosettes consisted of tightly packed cells arranged like spokes as in the case of H o m e r Wright rosettes in the neuroectodermal tumors, which showed both intracytoplasmic and extracellular inclusions by light microscopy, with the inclusions displaying lumina with projecting microvilli by electron microscopy. As these features were quite similar to those of the present case,we measured various catecholamines including epinephrine, norepinephrine, and dopamine in the cerebrospinal fluid, serum and urine of this patient. However, all of them were normal, and the specimen showed no catecholamine histofluorescence by the glyoxylic acid method. The present case was also characterized by moyamoya vessels in the basal ganglia. The pathological
features of m o y a m o y a vessels are characterized by the fibrocellular thickening of the intima with enhanced angiogenesis [6]. Although we could not find discernible relation between the secretory meningioma and moyamoya vessels, the coincidence might be interesting when considering the etiology of both diseases.
References 1. Alguacil-Garcia A, Pettigrew NM, Sima AAF (1986) Secretory meningioma. A distinct subtype of meningioma. Am J Surg Pathol 10:102-111 2. Kepes JJ (1982) Light-microscopic features of meningioma. In: Meningiomas. Biology, pathology, and differential diagnosis. Masson, New York, pp 64-149 3. Kepes JJ (1986) Presidental address: the histopathology of meningiomas. A reflection of origins and expected behavior? J Neuropathol Exp Neurol 45:95-107 4. LindvallO, Bjorklund A (1974) The glyoxylic acid fluorescence histochemical method: a detailed account of the methodology for the visualization of central catecholamine neurons. Histochemistry 39:97-127 5. O'Rahilly R, Muller F (1986) The meninges in human development. J Neuropathol Exp Neurol 45:588-608 6. Takebayashi S, Matsuo K, Kaneko M (1984) Ultrastructural studies of cerebral arteries and collateral vessels in moyamoya disease. Stroke 15:728-732 7. Yamashima T (1985) Meningothelial rosettes in the canine subarachnoid space. Acta Neuropathol (Berl) 66:223-232 8. Yamashima T (1988) Ultrastructural comparison of arachnoid villi and meningiomas in man. Mod Pathol 1:224-234 9. Yamashima T, Yamamoto S (1983) Cerebral arterial pathology in experimental subarachnoid hemorrhage. J Neurosurg 58: 843-850
Acta Neumpathologtca (~) Springer-Verlag 1992
Secretory meningioma associated with numerous meningothelial rosettes Tsuyoshi Tada l, Keiko Ishii 2, Syuichi Oshima 1, Hideaki Hara 1, and Shigeaki Kobayashi 1 Department of 1Neurosurgery, and 2Laboratory Medicine, Sbinsbu University School of Medicine, Asahi 341-1, Matsumoto 390, Japan Received December 19, 1992/Revised, accepted March 17, 1992
Summary. A case of secretory m e n i n g i o m a with numerous meningothelial rosettes is reported. A 66-year-old m a n with m o y a m o y a disease gradually developed skull deformity, and underwent surgery for the skull t u m o r overlying the hemisphere. Histological e x a m i n a t i o n disclosed n u m e r o u s meningothelial rosettes quite similar to those induced by subarachnoid injection of epinephrine and p s e u d o p s a m m o m a bodies described by Kepes. This m a y be the first case of m e n i n g i o m a associated with n u m e r o u s rosette formations. Key words: Secretory m e n i n g i o m a - Rosette - Moyam o y a vessel
Although rosette formations are often seen in neuroect o d e r m a l tumors, they are rare in meningiomas. We recently experienced a case of secretory m e n i n g i o m a associated with m o y a m o y a disease. T h e t u m o r had n u m e r o u s rosette formations, which are quite similar to meningothelial rosettes induced by the subarachnoid injection of epinephrine in dogs [7].
residual tumor rapidly grew in the following 3 months to invade the scalp over the superior sagittal sinus. Surgery was repeated to resect the tumor from the superior sagittal sinus. Postoperative radiation was given locally (50 Gy). There has been no signs of recurrence for 1 year after the second surgery.
Materials and methods For light microscopy, the specimens were fixed in 10 % buffered formalin, and paraffin-embedded sections were stained with diastase-treated periodic acid-Schiff (d-PAS) and hematoxylineosin (H&E). Immunohistochemical staining was performed with the avidin-biotin complex technique using monoclonal antibodies for vimentin (Dako Corporation, Santa Barbara, Calif.), epithelial membrane antigen (EMA; Becton Dickinson Immunocytometry Systems, San Jose, Calif.), factor VIII-related antigen (Dako) carcinoembryonic antigen (CEA; Dako), chromogranin A (Dako), cytokeratin (CAM 5.2; Becton Dickinson) and S-100 protein (Dako). Human intestine was used as a positive control. For electron microscopy, the specimens were fixed in 2.5 % glutaraldehyde and 1% OsO4, and were embedded in Epon. Ultrathin sections were stained with uranyl acetate and lead citrate, and were examined with Hitachi HS-9 electron microscoPY. Frozen sections of the specimens were processed for glyoxylic acid fluorescence method [4] to detect catecholamine in the tissue.
Case history A 66-year-old man had gradually developed skull deformity over a period of 6 months, and was admitted to our hospital for examination and operation. Skull X-ray showed an extensive decalcification of the left calvaria. Computed tomography scan showed a large isodensity mass overlying the left hemisphere, which was well enhanced with contrast medium. Magnetic resonance imaging showed a crescent mass over the left hemisphere, extending to the superior sagittal sinus, frontal air sinus and temporatis muscle (Fig. la). Cerebral angiography showed that the tumor was hypervascular and there were moyamoya vessels in the basal ganglia (Fig. l b ) . T h e tumor was almost totally removed except the portion of the tumor invading the superior sagittal sinus. The
Correspondence to: T. Tada (address see above)
Results O n light microscopy, meningothelial cells with a distinct nucleolus often showed rosette formations. Although the nuclei showed a slight degree of p l e o m o r p h i s m , there was no mitosis (Fig. 2) . N u m e r o u s eosinophilic inclusion bodies were distributed within and a m o n g the t u m o r cells, and stained positive for d-PAS staining (Fig. 3). Immunohistochemically, the t u m o r cells and inclusion bodies were positive for vimentin, but negative for E M A , factor V I I I - r e l a t e d antigen, C E A , chromogranin A, cytokeratin and S-100 protein.
343
Fig. 1. a Coronal magnetic resonance imaging showing a huge meningioma on the left parietal lobe. The tumor is located mainly in the epidural space, invading the superior sagittal sinus and temporalis muscle, b Angiogram showing stenosis of bilateral carotid arteries and moyamoya vessels Fig. 2. Photomicrograph showing numerous rosette formations of meningothelial cells. H&E, x 265 Fig. 3. Photomicrograph demonstrating PAS-positive inclusion bodies distributed intra- and intercellularly. Diastase-treated PAS, • 712
Electron microscopy
Discussion
The tumor cells showed ultrastructural features compatible with meningioma such as desmosomes, interdigitations and intermediate filaments (Fig. 4a). Glycogen granules and mitochondria were abundant with in the cytoplasm. The cytoplasm also had an inclusion coated with microvilli, the size of which was consistent with an eosinophilic inclusion body on light microscopy (Fig. 4b).
It is widely accepted that meningiomas derive from arachnoidal cap cells [8]. They show various mesenchyreal and epithelial features such as well-developed desmosomes [3]. A variety of histological features of meninges can be explained by the earlier report that the meninges consist of the cells of mesodermal, neural crest and neural tube origin [5]. Secretory meningiomas have a strong tendency for epithelial differentiation, and accumulate secretory materials in the form of hyaline inclusions. They are called pseudopsammoma bodies and are easily confirmed by PAS staining. ImmunohistochemicaUy, the tumor cells and inclusion bodies are usually positive for C E A , E M A and occasionally positive for cytokeratin [1]. Ultrastructurally, lumina coy-
Catecholamine histofluorescence No catecholamine histofluorescence was detected within the specimen studied.
345 Fig. 4. a Electron micrograph showing desmosomes, interdigitations and intermediate filaments. Glycogen granules and mitochondria were abundant in the cytoplasm, b Intracellular lumina coated with microvilli, a • 11,400, b x 22,800
ered with microvilli are observed within and among the tumor cells [1]. In the present case, although the tumor cells and inclusion bodies were negative for C E A , E M A and cytokeratin, positive staining of the inclusion bodies for PAS and the ultrastructural features are characteristics of secretory meningiomas. Meningiomas rarely show rosette formations [2, 3]. The present report describes the first case of a secretory meningioma with numerous rosettes. Meningothelial rosettes were experimentally induced in the canine meninges by subarachnoid injection of epinephrine [7, 9]. The experimentally induced rosettes consisted of tightly packed cells arranged like spokes as in the case of H o m e r Wright rosettes in the neuroectodermal tumors, which showed both intracytoplasmic and extracellular inclusions by light microscopy, with the inclusions displaying lumina with projecting microvilli by electron microscopy. As these features were quite similar to those of the present case,we measured various catecholamines including epinephrine, norepinephrine, and dopamine in the cerebrospinal fluid, serum and urine of this patient. However, all of them were normal, and the specimen showed no catecholamine histofluorescence by the glyoxylic acid method. The present case was also characterized by moyamoya vessels in the basal ganglia. The pathological
features of m o y a m o y a vessels are characterized by the fibrocellular thickening of the intima with enhanced angiogenesis [6]. Although we could not find discernible relation between the secretory meningioma and moyamoya vessels, the coincidence might be interesting when considering the etiology of both diseases.
References 1. Alguacil-Garcia A, Pettigrew NM, Sima AAF (1986) Secretory meningioma. A distinct subtype of meningioma. Am J Surg Pathol 10:102-111 2. Kepes JJ (1982) Light-microscopic features of meningioma. In: Meningiomas. Biology, pathology, and differential diagnosis. Masson, New York, pp 64-149 3. Kepes JJ (1986) Presidental address: the histopathology of meningiomas. A reflection of origins and expected behavior? J Neuropathol Exp Neurol 45:95-107 4. LindvallO, Bjorklund A (1974) The glyoxylic acid fluorescence histochemical method: a detailed account of the methodology for the visualization of central catecholamine neurons. Histochemistry 39:97-127 5. O'Rahilly R, Muller F (1986) The meninges in human development. J Neuropathol Exp Neurol 45:588-608 6. Takebayashi S, Matsuo K, Kaneko M (1984) Ultrastructural studies of cerebral arteries and collateral vessels in moyamoya disease. Stroke 15:728-732 7. Yamashima T (1985) Meningothelial rosettes in the canine subarachnoid space. Acta Neuropathol (Berl) 66:223-232 8. Yamashima T (1988) Ultrastructural comparison of arachnoid villi and meningiomas in man. Mod Pathol 1:224-234 9. Yamashima T, Yamamoto S (1983) Cerebral arterial pathology in experimental subarachnoid hemorrhage. J Neurosurg 58: 843-850
