- Email: [email protected]
Intraventricular rhabdoid meningioma
672
5.
6.
7. 8.
9.
10. 11.
12.
13. 14.
15.
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675 Implications for diagnosis and genetic counselling. Am J Med Genet 2001;101:46–9. Westphal V, Peterson S, Patterson M, et al. Functional significance of PMM2 mutations in mildly affected patients with congenital disorder of glycosylation Ia. Genet Med 2001;3:393–8. Jensen H, Kjaergaard S, Klie F, et al. Ophthalmic mainfestations of congenital disorder of glycosylation type Ia. Ophthal Genet 2003;24: 81–8. Gene Reviews (Online). 2005 August; Available from: URL: http:// www.genetests.org/ (Accessed 14 March 2006). Miossec-Chauvet E, Mikaeloff Y, Heron D, et al. Neurological presentations in pediatric patients with congenital disorder of glycosylation type Ia. Neuropediatrics 2003;34:1–6. Jensen PR, Hansen FJ, Skovby F. Cerebellar hypoplasia in children with the carbohydrate-deficient glycoprotein syndrome. Neuroradiology 1995;37:328–30. Pearl P, Krasnewich D. Neurologic course of congenital disorders of glycosylation. J Child Neurol 2001;16:409–13. Barone R, Pavone L, Fiumara A, et al. Developmental patterns and neuropsychological assesment in patients with carbohydrate-deficient glycoconjugate syndrome type Ia (phosphomannomutase deficiency). Brain Dev 1999;21:260–3. Van Ommenn C, Peters M, Barth P, et al. Carbohydrate-deficient glycoprotein syndrome type Ia: A variant phenotype with borderline cognitive dysfunction, cerebellar hypoplasia, and coagulation disturbances. J Pediatr 2000;136:400–3. Jaeken J, Carchon H. What’s new in congenital disorders of glycosylation? Eur J Pediatr Neurol 2000;4:163–7. deLonay P, Seta N, Barrot S, et al. A broad spectrum of clinical presentations in congenital disorders of glycosylation I: a serries of 26 cases. J Med Genet 2001;38:14–9. Stibler H, Blennow G, Kristiansson B, et al. Carbohydrate-deficient glycoprotein syndrome: clinical expression in adults with a new metabolic disease. J Neurol Neurosurg Psychiatry 1994;57:552–6.
16. Westphal V, Enns GM, McCracken MF, et al. Functional analysis of novel mutations in a congenital disorder of glycosylation ia patient with mixed Asian ancestry. Mol Genet Metab 2001;73:71–6. 17. Briones P, Vilaseca MA, Schollen E, et al. Biochemical and molecular studies in 26 Spanish patients with congenital disorder of glycosylation type Ia. J Inherit Metab Dis 2002;25:635–46. 18. De Zegher F, Jaeken J. Endocrinology of the carbohydrate-deficient glycoprotein syndrome type i from birth through adolescence. Pediatr Res 1995;37:395–401. 19. Kjaergaard S, Muller J, Skovby F. Prepubertal growth in congenital disorder of glycosylation type Ia (CDG-Ia). Arch Dis Child 2002;87:324–7. 20. Matthijs G, Schollen E, Bjursell C. Mutations in PMM2 that cause congenital disorders if glycosylation, type Ia (CDG-Ia). Hum Mutat 2000;16:386–94. 21. Grunewald S, Matthijs G, Jaeken J. Congenital disorders of glycosylation: A review. Pediatr Res 2002;52:618–24. 22. Jaeken J. Congenital disorders of glycosylation (CDG): It’s all in it! J Inherit Metab Dis 2003;26:99–118. 23. Enn G, Steiner R, Bruist N, et al. Clinical and molecular features of congenital disorder of glycosylation in patients with type 1 sialotransferrin pattern and diverse ethnic origins. J Pediatr 2002;141:695–700. 24. Giurgea I, Michel A, Le Merrer M, et al. Under diagnosis of mild congenital disorders of glycosylation type Ia. Pediatr Neurol 2005;32:121–3. 25. Coman D, Klingberg S, Morris D, et al. Congenital disorder of glycosylation type Ia in a 6-year-old girl with a mild intellectual phenotype: Two novel PMM2 mutations. J Inherit Metab Dis 2005;28:1189–90. 26. Westphal V, Kjaergaard S, Schollen E, et al. A frequent mild mutation in ALG6 may exacerbate the clinical severity of patients with congenital disorder of glycosylation Ia (CDG-Ia) caused by phosphomannomutase deficiency. Hum Molec Genet 2002;11: 599–604.
doi:10.1016/j.jocn.2006.04.008
Intraventricular rhabdoid meningioma Jacqueline McMaster *, Thomas Ng, Mark Dexter Westmead Hospital, Department of Neurosurgery, PO Box 533, Wentworthville, New South Wales, 2145, Australia Received 19 December 2005; accepted 22 February 2006
Abstract Rhabdoid meningioma is a rare variant of meningioma, often found in tumour recurrences. We report a 55-year-old woman with a history of intraventricular fibroblastic meningioma, who developed headache and tinnitus 5 years after complete resection of the initial tumour. Imaging confirmed a recurrent tumour in the intraventricular location. Histological analysis revealed rhabdoid meningioma. We reviewed the literature and were unable to find any previously reported cases of intraventricular rhabdoid meningioma. 2006 Elsevier Ltd. All rights reserved. Keywords: Meningioma; Rhabdoid; Intraventricular; Recurrence
*
Corresponding author. E-mail address: [email protected] (J. McMaster).
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675
1. Introduction Meningiomas arise from arachnoid cap cells and account for approximately 15% of all primary brain tumours. They most commonly arise over the convexity, associated with the falx but may also be found elsewhere, including the ventricles. Intraventricular meningiomas are very rare, encompassing between 0.7%1 and 5%2 of intracranial meningiomas. The majority of meningiomas are benign tumours. There are a wide variety of histological subtypes, which correspond to different WHO grades from I to III. Those with a more aggressive nature and poorer prognosis
673
are given a higher grading. This includes the rhabdoid variant, which is classed as a grade III tumour in the most recent WHO classification.3 The clinical behaviour of rhabdoid meningioma was first suggested by Kepes4 and further characterised by Perry.5 We present a unique case of intraventricular rhabdoid meningioma arising in a patient with a history of fibroblastic meningioma treated 5 years earlier with complete surgical resection and no adjuvant therapy. 2. Case report A 55-year-old woman presented with a three day history of sudden onset of headache, nausea, vomiting and neck stiffness. Clinical examination was unremarkable. Head CT scan on admission showed an intraventricular haemorrhage most likely secondary to tumour. MRI showed a 3-cm mass adjacent to the trigone of the left lateral ventricle (Fig. 1a). The tumour enhanced after gadolinium administration. At operation, the tumour was soft and plum-coloured in appearance and located in the trigone of the left lateral ventricle. The tumour was removed in its entirety (Fig. 1b). Frozen section suggested meningioma. The patient represented 5 years after the original surgery with one month’s history of headache and right-sided tinnitus. MRI confirmed the recurrence of tumour with associated intraventricular haemorrhage (Fig. 2). She returned to theatre for further excision. Post-operatively, she received whole brain irradiation and at 6 months follow-up, there is no evidence of recurrence of the tumour. 2.1. Pathology The initial resection showed a fibroblastic meningioma composed of spindle cells. There were no mitoses or areas
Fig. 1. (a) Pre-operative axial T1 weighted MRI showing the tumour in the left trigone (left). (b) Post-operative axial T1 weighted MRI showing complete excision of tumour (right).
Fig. 2. Axial T1 weighted MRI showing recurrent tumour.
674
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675
Fig. 3. Initial histopathology showed fibroblastic meningioma H&E (·100).
of necrosis. There was no evidence of atypia or malignancy within the specimen (Fig. 3). The second resection showed tumour cells of different morphology arranged in sheets. The cells appeared rhabdoid with eccentric nuclei and abundant eosinophilic cytoplasm. The nuclei were irregular with prominent nucleoli and occasional pseudonuclear inclusions (Fig. 4). Mitoses were easily identified. There was no visible necrosis or microvascular proliferation and no evidence of brain invasion. Tumour cells stained positive for vimentin and had a membranous staining pattern for epithelial membrane antigen (EMA) (Fig. 5). Ki-67 proliferation index varied from 5% to 15% depending on the area. Staining for cytokeratins was negative.
Fig. 5. Immunohistochemistry of the rhabdoid meningioma showing staining with epithelial membrane antigen (EMA) (top) and vimentin (bottom) (·400).
3. Discussion
Fig. 4. Histopathological examination of the recurrent tumour showed rhabdoid meningioma H&E (·400).
Intracranial rhabdoid tumours are rare but resemble rhabdoid tumours found elsewhere in the body. The cells are medium-sized, with an eccentrically placed nucleus and prominent nucleoli. The abundant eosinophilic cytoplasm has a fine granular appearance, which may resemble an inclusion. The cell borders are distinct and there are often many mitoses. Rhabdoid cells characteristically stain for vimentin (diffuse staining) and EMA (membranous pattern) and occasionally smooth muscle antigen (SMA). The rhabdoid morphology appears to confer aggressiveness in clinical behaviour. Rhabdoid meningioma is a relatively new entity, being classified separately for the first time in the 2000 WHO classification of central nervous system tumours.6 Its acceptance as a variant of meningioma comes following its initial reporting by Kepes4 and then by Perry in 1998.5 Their observations of its malignant histological features and reported aggressive behaviour led to its classification as a grade III neoplasm.
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675
Rhabdoid morphology may occur in a primary meningioma5,7–9 or more commonly in recurrent, previously benign meningioma.5 There is much speculation as to whether rhabdoid meningiomas arise de novo or are transformed from a more benign tumour type.4,10 There have also been reports of rhabdoid meningioma arising concurrently with papillary meningioma,5,11,12 within pre-existing ganglioglioma10 or in a site of previous trauma.13 Why this variant occurs in recurrent tumours is unknown. Radiotherapy has been implicated in this malignant transformation but this is not always the case. In Perry’s series5 half the patients with rhabdoid meningioma as recurrence had radiotherapy. Koenig6 suggests that release of growth factors which occurs during healing following an initial insult, (whether injury or surgery) may lead to neoplastic transformation. Further study is needed to determine the pathogenesis. While it is most common that the rhabdoid meningioma with its atypical histology behaves aggressively, there are studies that show a more benign course.5,8 In these cases, the rhabdoid cells lack the histological features of malignancy such as mitoses, anaplasia and high Ki-67 labelling index.9 It remains to be seen how this tumour will behave, although evidence suggests that it will behave aggressively based on its histology. Others suggest that the rhabdoid phenotype is a morphological marker for malignant transformation of meningiomas.5 Intraventricular meningiomas are rare, accounting for only 0.7–5.0% of all primary intracranial meningiomas.1,2 The majority of these occur in the left lateral ventricle.14 Most commonly, these are fibroblastic or meningotheliomatous subtypes1 although angiomatous, transitional, psammomatous,1 chordoid15 and malignant16 tumours have also been reported. Review of the literature reveals no previous report of the rhabdoid subtype in the intraventricular location. The differential diagnoses include rhabdoid variant of choroid plexus carcinoma, atypical teratoid/rhabdoid tumour (AT/RT) or metastatic rhabdoid tumour. In this patient, none of these were considered likely given the clinical situation and histology. However, it is important to exclude the differential diagnoses. AT/RT are tumours of childhood which rarely occur in adults. They are composed of rhabdoid cells as well as an additional component of primitive neuroectodermal, epithelial and mesenchymal cells.17 The proportions of each of these components varies between individual tumours. These tumours while staining for EMA and vimentin, also stain for glial fibrillary acidic protein and neurofilament which are negative in meningiomas. Differentiating rhabdoid meningioma from other tumours including gemistocytic astrocytoma or ependymoma may rely on the characteristic ultrastructural features, namely intracytoplasmic intermediate filaments and the absence of intercellular junctions and interdigitating doi:10.1016/j.jocn.2006.02.019
675
cell processes.5 The immunohistochemistry profile helps to exclude the possibilities of tumours of epithelial origin such as carcinoma, which should stain for cytokeratins. In conclusion, rhabdoid meningioma is a rare, clinically aggressive variant of meningioma, often found in recurrent tumours as illustrated by this patient. The intraventricular location is previously unreported in this meningioma subtype. References 1. Nakamura M, Roser F, Bundschuh O, et al. Intraventricular meningiomas: A review of 16 cases with reference to the literature. Surg Neurol 2003;59:491–504. 2. Rohringer M, Sutherland GR, Louw DF, et al. Incidence and clinicopathological features of meningioma. J Neurosurg 1989;71:665–72. 3. Louis DN, Scheithauer BW, Budka H, et al. Meningiomas. In: Kleihues P, Cavenee WK, editors. World Health Organization Classification of Tumours. Pathology and Genetics: Tumours of the Nervous System. Lyon: IARC Press; 2000. p. 176–84. 4. Kepes JJ, Moral LA, Wilkinson SB, et al. Rhabdoid transformation of tumour cells in meningiomas: A histologic indication of increased proliferative activity: Report of four cases. Am J Surg Pathol 1998;22:231–8. 5. Perry A, Scheithauer BW, Stafford SL, et al. ‘‘Rhabdoid’’ meningioma: An aggressive variant. Am J Surg Pathol 1998;22:1482–90. 6. Kleihues P, Louis DN, Scheithauer BW, et al. The WHO classification of tumours of the nervous system. J Neuropathol Exp Neurol 2002;61:215–25. 7. Kesavan S. Meningioma with rhabdoid transformation. Singapore Med J 2000;41:464–7. 8. Mawrin C, Hahne R, Scherlach C, et al. June 2004: a male in his late 60s with recurrent extracerebral tumor. Brain Pathol 2004;14:457–9. 9. Cooper WA, Shingde M, Lee VK, et al. ‘‘Rhabdoid meningioma’’ lacking malignant features. Clin Neuropathol 2004;23:16–20. 10. Bannykh SI, Perry A, Powell HC, et al. Malignant rhabdoid meningioma arising in the setting of preexisting ganglioglioma: a diagnosis supported by fluorescence in situ hybridization. Case report. J Neurosurg 2002;97:1450–5. 11. Hojo H, Abe M. Rhabdoid papillary meningioma. Am J Surg Pathol 2001;25:964–9. 12. Al-Habib A, Lach B, Al Khani A. Intracerebral rhabdoid and papillary meningioma with leptomeningeal spread and rapid clinical progression. Clin Neuropathol 2005;24:1–7. 13. Koenig MA, Geocadin RG, Kulesza P, et al. Rhabdoid meningioma occurring in an unrelated resection cavity with leptomeningeal carcinomatosis. J Neurosurg 2005;102:371–5. 14. Lantos PL, Louis DN, Rosenblum MK, et al. Tumours of the nervous system. 7th edition. In: Graham DI, Lantos PL, editors. Greenfield’s Neuropathology, Vol. 2. London: Arnold; 2002. p. 767–1052. 15. Arima T, Natsume A, Hatano H, et al. Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. J Neurosurg 2005;102:733–7. 16. Erman T, Gocer AI, Tuna M, et al. Malignant meningioma of the lateral ventricle. Case report. Neurosurg Focus 2003;15:CP2. 17. Rorke LB, Biegel JA. Atypical teratoid/rhabdoid tumour. In: Kleihues P, Cavenee WK, editors. World Health Organization Classification of Tumours. Pathology and Genetics: Tumours of the Nervous System. Lyon: IARC Press; 2000. p. 145–8.
5.
6.
7. 8.
9.
10. 11.
12.
13. 14.
15.
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675 Implications for diagnosis and genetic counselling. Am J Med Genet 2001;101:46–9. Westphal V, Peterson S, Patterson M, et al. Functional significance of PMM2 mutations in mildly affected patients with congenital disorder of glycosylation Ia. Genet Med 2001;3:393–8. Jensen H, Kjaergaard S, Klie F, et al. Ophthalmic mainfestations of congenital disorder of glycosylation type Ia. Ophthal Genet 2003;24: 81–8. Gene Reviews (Online). 2005 August; Available from: URL: http:// www.genetests.org/ (Accessed 14 March 2006). Miossec-Chauvet E, Mikaeloff Y, Heron D, et al. Neurological presentations in pediatric patients with congenital disorder of glycosylation type Ia. Neuropediatrics 2003;34:1–6. Jensen PR, Hansen FJ, Skovby F. Cerebellar hypoplasia in children with the carbohydrate-deficient glycoprotein syndrome. Neuroradiology 1995;37:328–30. Pearl P, Krasnewich D. Neurologic course of congenital disorders of glycosylation. J Child Neurol 2001;16:409–13. Barone R, Pavone L, Fiumara A, et al. Developmental patterns and neuropsychological assesment in patients with carbohydrate-deficient glycoconjugate syndrome type Ia (phosphomannomutase deficiency). Brain Dev 1999;21:260–3. Van Ommenn C, Peters M, Barth P, et al. Carbohydrate-deficient glycoprotein syndrome type Ia: A variant phenotype with borderline cognitive dysfunction, cerebellar hypoplasia, and coagulation disturbances. J Pediatr 2000;136:400–3. Jaeken J, Carchon H. What’s new in congenital disorders of glycosylation? Eur J Pediatr Neurol 2000;4:163–7. deLonay P, Seta N, Barrot S, et al. A broad spectrum of clinical presentations in congenital disorders of glycosylation I: a serries of 26 cases. J Med Genet 2001;38:14–9. Stibler H, Blennow G, Kristiansson B, et al. Carbohydrate-deficient glycoprotein syndrome: clinical expression in adults with a new metabolic disease. J Neurol Neurosurg Psychiatry 1994;57:552–6.
16. Westphal V, Enns GM, McCracken MF, et al. Functional analysis of novel mutations in a congenital disorder of glycosylation ia patient with mixed Asian ancestry. Mol Genet Metab 2001;73:71–6. 17. Briones P, Vilaseca MA, Schollen E, et al. Biochemical and molecular studies in 26 Spanish patients with congenital disorder of glycosylation type Ia. J Inherit Metab Dis 2002;25:635–46. 18. De Zegher F, Jaeken J. Endocrinology of the carbohydrate-deficient glycoprotein syndrome type i from birth through adolescence. Pediatr Res 1995;37:395–401. 19. Kjaergaard S, Muller J, Skovby F. Prepubertal growth in congenital disorder of glycosylation type Ia (CDG-Ia). Arch Dis Child 2002;87:324–7. 20. Matthijs G, Schollen E, Bjursell C. Mutations in PMM2 that cause congenital disorders if glycosylation, type Ia (CDG-Ia). Hum Mutat 2000;16:386–94. 21. Grunewald S, Matthijs G, Jaeken J. Congenital disorders of glycosylation: A review. Pediatr Res 2002;52:618–24. 22. Jaeken J. Congenital disorders of glycosylation (CDG): It’s all in it! J Inherit Metab Dis 2003;26:99–118. 23. Enn G, Steiner R, Bruist N, et al. Clinical and molecular features of congenital disorder of glycosylation in patients with type 1 sialotransferrin pattern and diverse ethnic origins. J Pediatr 2002;141:695–700. 24. Giurgea I, Michel A, Le Merrer M, et al. Under diagnosis of mild congenital disorders of glycosylation type Ia. Pediatr Neurol 2005;32:121–3. 25. Coman D, Klingberg S, Morris D, et al. Congenital disorder of glycosylation type Ia in a 6-year-old girl with a mild intellectual phenotype: Two novel PMM2 mutations. J Inherit Metab Dis 2005;28:1189–90. 26. Westphal V, Kjaergaard S, Schollen E, et al. A frequent mild mutation in ALG6 may exacerbate the clinical severity of patients with congenital disorder of glycosylation Ia (CDG-Ia) caused by phosphomannomutase deficiency. Hum Molec Genet 2002;11: 599–604.
doi:10.1016/j.jocn.2006.04.008
Intraventricular rhabdoid meningioma Jacqueline McMaster *, Thomas Ng, Mark Dexter Westmead Hospital, Department of Neurosurgery, PO Box 533, Wentworthville, New South Wales, 2145, Australia Received 19 December 2005; accepted 22 February 2006
Abstract Rhabdoid meningioma is a rare variant of meningioma, often found in tumour recurrences. We report a 55-year-old woman with a history of intraventricular fibroblastic meningioma, who developed headache and tinnitus 5 years after complete resection of the initial tumour. Imaging confirmed a recurrent tumour in the intraventricular location. Histological analysis revealed rhabdoid meningioma. We reviewed the literature and were unable to find any previously reported cases of intraventricular rhabdoid meningioma. 2006 Elsevier Ltd. All rights reserved. Keywords: Meningioma; Rhabdoid; Intraventricular; Recurrence
*
Corresponding author. E-mail address: [email protected] (J. McMaster).
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675
1. Introduction Meningiomas arise from arachnoid cap cells and account for approximately 15% of all primary brain tumours. They most commonly arise over the convexity, associated with the falx but may also be found elsewhere, including the ventricles. Intraventricular meningiomas are very rare, encompassing between 0.7%1 and 5%2 of intracranial meningiomas. The majority of meningiomas are benign tumours. There are a wide variety of histological subtypes, which correspond to different WHO grades from I to III. Those with a more aggressive nature and poorer prognosis
673
are given a higher grading. This includes the rhabdoid variant, which is classed as a grade III tumour in the most recent WHO classification.3 The clinical behaviour of rhabdoid meningioma was first suggested by Kepes4 and further characterised by Perry.5 We present a unique case of intraventricular rhabdoid meningioma arising in a patient with a history of fibroblastic meningioma treated 5 years earlier with complete surgical resection and no adjuvant therapy. 2. Case report A 55-year-old woman presented with a three day history of sudden onset of headache, nausea, vomiting and neck stiffness. Clinical examination was unremarkable. Head CT scan on admission showed an intraventricular haemorrhage most likely secondary to tumour. MRI showed a 3-cm mass adjacent to the trigone of the left lateral ventricle (Fig. 1a). The tumour enhanced after gadolinium administration. At operation, the tumour was soft and plum-coloured in appearance and located in the trigone of the left lateral ventricle. The tumour was removed in its entirety (Fig. 1b). Frozen section suggested meningioma. The patient represented 5 years after the original surgery with one month’s history of headache and right-sided tinnitus. MRI confirmed the recurrence of tumour with associated intraventricular haemorrhage (Fig. 2). She returned to theatre for further excision. Post-operatively, she received whole brain irradiation and at 6 months follow-up, there is no evidence of recurrence of the tumour. 2.1. Pathology The initial resection showed a fibroblastic meningioma composed of spindle cells. There were no mitoses or areas
Fig. 1. (a) Pre-operative axial T1 weighted MRI showing the tumour in the left trigone (left). (b) Post-operative axial T1 weighted MRI showing complete excision of tumour (right).
Fig. 2. Axial T1 weighted MRI showing recurrent tumour.
674
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675
Fig. 3. Initial histopathology showed fibroblastic meningioma H&E (·100).
of necrosis. There was no evidence of atypia or malignancy within the specimen (Fig. 3). The second resection showed tumour cells of different morphology arranged in sheets. The cells appeared rhabdoid with eccentric nuclei and abundant eosinophilic cytoplasm. The nuclei were irregular with prominent nucleoli and occasional pseudonuclear inclusions (Fig. 4). Mitoses were easily identified. There was no visible necrosis or microvascular proliferation and no evidence of brain invasion. Tumour cells stained positive for vimentin and had a membranous staining pattern for epithelial membrane antigen (EMA) (Fig. 5). Ki-67 proliferation index varied from 5% to 15% depending on the area. Staining for cytokeratins was negative.
Fig. 5. Immunohistochemistry of the rhabdoid meningioma showing staining with epithelial membrane antigen (EMA) (top) and vimentin (bottom) (·400).
3. Discussion
Fig. 4. Histopathological examination of the recurrent tumour showed rhabdoid meningioma H&E (·400).
Intracranial rhabdoid tumours are rare but resemble rhabdoid tumours found elsewhere in the body. The cells are medium-sized, with an eccentrically placed nucleus and prominent nucleoli. The abundant eosinophilic cytoplasm has a fine granular appearance, which may resemble an inclusion. The cell borders are distinct and there are often many mitoses. Rhabdoid cells characteristically stain for vimentin (diffuse staining) and EMA (membranous pattern) and occasionally smooth muscle antigen (SMA). The rhabdoid morphology appears to confer aggressiveness in clinical behaviour. Rhabdoid meningioma is a relatively new entity, being classified separately for the first time in the 2000 WHO classification of central nervous system tumours.6 Its acceptance as a variant of meningioma comes following its initial reporting by Kepes4 and then by Perry in 1998.5 Their observations of its malignant histological features and reported aggressive behaviour led to its classification as a grade III neoplasm.
Case reports / Journal of Clinical Neuroscience 14 (2007) 672–675
Rhabdoid morphology may occur in a primary meningioma5,7–9 or more commonly in recurrent, previously benign meningioma.5 There is much speculation as to whether rhabdoid meningiomas arise de novo or are transformed from a more benign tumour type.4,10 There have also been reports of rhabdoid meningioma arising concurrently with papillary meningioma,5,11,12 within pre-existing ganglioglioma10 or in a site of previous trauma.13 Why this variant occurs in recurrent tumours is unknown. Radiotherapy has been implicated in this malignant transformation but this is not always the case. In Perry’s series5 half the patients with rhabdoid meningioma as recurrence had radiotherapy. Koenig6 suggests that release of growth factors which occurs during healing following an initial insult, (whether injury or surgery) may lead to neoplastic transformation. Further study is needed to determine the pathogenesis. While it is most common that the rhabdoid meningioma with its atypical histology behaves aggressively, there are studies that show a more benign course.5,8 In these cases, the rhabdoid cells lack the histological features of malignancy such as mitoses, anaplasia and high Ki-67 labelling index.9 It remains to be seen how this tumour will behave, although evidence suggests that it will behave aggressively based on its histology. Others suggest that the rhabdoid phenotype is a morphological marker for malignant transformation of meningiomas.5 Intraventricular meningiomas are rare, accounting for only 0.7–5.0% of all primary intracranial meningiomas.1,2 The majority of these occur in the left lateral ventricle.14 Most commonly, these are fibroblastic or meningotheliomatous subtypes1 although angiomatous, transitional, psammomatous,1 chordoid15 and malignant16 tumours have also been reported. Review of the literature reveals no previous report of the rhabdoid subtype in the intraventricular location. The differential diagnoses include rhabdoid variant of choroid plexus carcinoma, atypical teratoid/rhabdoid tumour (AT/RT) or metastatic rhabdoid tumour. In this patient, none of these were considered likely given the clinical situation and histology. However, it is important to exclude the differential diagnoses. AT/RT are tumours of childhood which rarely occur in adults. They are composed of rhabdoid cells as well as an additional component of primitive neuroectodermal, epithelial and mesenchymal cells.17 The proportions of each of these components varies between individual tumours. These tumours while staining for EMA and vimentin, also stain for glial fibrillary acidic protein and neurofilament which are negative in meningiomas. Differentiating rhabdoid meningioma from other tumours including gemistocytic astrocytoma or ependymoma may rely on the characteristic ultrastructural features, namely intracytoplasmic intermediate filaments and the absence of intercellular junctions and interdigitating doi:10.1016/j.jocn.2006.02.019
675
cell processes.5 The immunohistochemistry profile helps to exclude the possibilities of tumours of epithelial origin such as carcinoma, which should stain for cytokeratins. In conclusion, rhabdoid meningioma is a rare, clinically aggressive variant of meningioma, often found in recurrent tumours as illustrated by this patient. The intraventricular location is previously unreported in this meningioma subtype. References 1. Nakamura M, Roser F, Bundschuh O, et al. Intraventricular meningiomas: A review of 16 cases with reference to the literature. Surg Neurol 2003;59:491–504. 2. Rohringer M, Sutherland GR, Louw DF, et al. Incidence and clinicopathological features of meningioma. J Neurosurg 1989;71:665–72. 3. Louis DN, Scheithauer BW, Budka H, et al. Meningiomas. In: Kleihues P, Cavenee WK, editors. World Health Organization Classification of Tumours. Pathology and Genetics: Tumours of the Nervous System. Lyon: IARC Press; 2000. p. 176–84. 4. Kepes JJ, Moral LA, Wilkinson SB, et al. Rhabdoid transformation of tumour cells in meningiomas: A histologic indication of increased proliferative activity: Report of four cases. Am J Surg Pathol 1998;22:231–8. 5. Perry A, Scheithauer BW, Stafford SL, et al. ‘‘Rhabdoid’’ meningioma: An aggressive variant. Am J Surg Pathol 1998;22:1482–90. 6. Kleihues P, Louis DN, Scheithauer BW, et al. The WHO classification of tumours of the nervous system. J Neuropathol Exp Neurol 2002;61:215–25. 7. Kesavan S. Meningioma with rhabdoid transformation. Singapore Med J 2000;41:464–7. 8. Mawrin C, Hahne R, Scherlach C, et al. June 2004: a male in his late 60s with recurrent extracerebral tumor. Brain Pathol 2004;14:457–9. 9. Cooper WA, Shingde M, Lee VK, et al. ‘‘Rhabdoid meningioma’’ lacking malignant features. Clin Neuropathol 2004;23:16–20. 10. Bannykh SI, Perry A, Powell HC, et al. Malignant rhabdoid meningioma arising in the setting of preexisting ganglioglioma: a diagnosis supported by fluorescence in situ hybridization. Case report. J Neurosurg 2002;97:1450–5. 11. Hojo H, Abe M. Rhabdoid papillary meningioma. Am J Surg Pathol 2001;25:964–9. 12. Al-Habib A, Lach B, Al Khani A. Intracerebral rhabdoid and papillary meningioma with leptomeningeal spread and rapid clinical progression. Clin Neuropathol 2005;24:1–7. 13. Koenig MA, Geocadin RG, Kulesza P, et al. Rhabdoid meningioma occurring in an unrelated resection cavity with leptomeningeal carcinomatosis. J Neurosurg 2005;102:371–5. 14. Lantos PL, Louis DN, Rosenblum MK, et al. Tumours of the nervous system. 7th edition. In: Graham DI, Lantos PL, editors. Greenfield’s Neuropathology, Vol. 2. London: Arnold; 2002. p. 767–1052. 15. Arima T, Natsume A, Hatano H, et al. Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report. J Neurosurg 2005;102:733–7. 16. Erman T, Gocer AI, Tuna M, et al. Malignant meningioma of the lateral ventricle. Case report. Neurosurg Focus 2003;15:CP2. 17. Rorke LB, Biegel JA. Atypical teratoid/rhabdoid tumour. In: Kleihues P, Cavenee WK, editors. World Health Organization Classification of Tumours. Pathology and Genetics: Tumours of the Nervous System. Lyon: IARC Press; 2000. p. 145–8.