- Email: [email protected]
Meningioma of the fourth ventricle presenting with intermittent behaviour disorders: a case report and review of the literature
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Journal of Clinical Neuroscience (2001) 8(Supplement 1), 59–62 © 2001 Harcourt Publishers Ltd doi: 10.1054/jocn.2001.0879, available online at http://www.idealibrary.com on
Meningioma of the fourth ventricle presenting with intermittent behaviour disorders: a case report and review of the literature C. Chaskis MD, T. Buisseret1 MD, A. Michotte2 MD, J. D’Haens MD Departments of Neurosurgery, 1Neuroradiology and 2Neuropathology, Academic Hospital, Vrije Universiteit Brussel, Brussels, Belgium
Summary Intraventricular meningiomas are rare, representing 0.5–5% of all intracranial meningiomas. They arise mostly within the lateral ventricles and more rarely in the third ventricle. Meningiomas of the fourth ventricle are exceptional. They are clearly defined as meningiomas arising from the choroid plexus and lying strictly within the fourth ventricle. We report a 76 year old male patient presenting with a 2-week history of headache and cognitive disorders with agitation and restlessness particularly exacerbated at night or when lying down. CT scan and MR imaging showed a contrast-enhancing lesion located purely within the whole fourth ventricle, with slight ventricular enlargement. At surgery, we totally removed a well-vascularised, greyish encapsulated mass attached to the choroid plexus. Pathological examination revealed a WHO grade I fibroblastic meningioma. We reviewed the literature concerning this unusual meningioma location. © 2001 Harcourt Publishers Ltd Keywords: fourth ventricle, neoplasm, meningioma, choroid plexus, hydrocephalus
CASE STUDY Clinical history We report the case of a 76 year old male patient admitted on emergency after an episode of seizures related to sudden withdrawal of benzodiazepine therapy. Two weeks before admission, he developed intermittent headache and behaviour disturbances with agitation and restlessness. The symptoms were particularly exacerbated at night or when lying down. Neurological examination on admission revealed spatial-temporal disorientation, impairment of memory functions and attention span, ataxic dysarthria and ataxia of gait with a Romberg sign.
Imaging CT scan showed a homogeneous spontaneously hyperdense lesion occupying the whole fourth ventricle, with an enlarged aqueduct and slightly enlarged third ventricle and temporal ventricle horns. There were neither intralesional calcifications nor cyst formations. MR imaging confirmed the exclusively intraventricular location of the tumour that appeared isointense to the surrounding grey matter on both T1- and T2-weighted spin-echo sequences (Fig. 1A), with very intense and homogenous enhancement after intravenous injection of Gadolinium-DTPA (Fig. 1B). MR imaging also confirmed the slight degree of installing or possibly episodic obstructive hydrocephalus. The lesion was fairly regular with smooth margins. It had a caudal extension to the foramen of Magendie but the cisterna magna was free (Fig. 2). There was no extension through the foramina of Luschka.
Surgical treatment Surgery was carried out with the patient prone. Through a midline incision, a suboccipital craniotomy was performed and the dura opened. Splitting the lower vermis, we totally removed a firm, well-vascularised, greyish, encapsulated mass lying more or less free in the fourth ventricle, only attached to the choroid plexus. The patient was completely normal postoperatively. Ten days after surgery, he developed somnolence. CT scan demonstrated a small blood clot in the foramen of Magendie and
Correspondence to: Cristo Chaskis MD, Department of Neurosurgery, A.Z.-V.U.B., Laarbeeklaan 101, 1090 Brussels, Belgium. Tel.:;322 4775514; Fax:;322 4776505; E-mail: [email protected]
diffuse cerebellar oedema causing obstructive hydrocephalus. The patient recovered completely after ventriculoperitoneal shunting. The further postoperative course was uneventful and the patient was discharged home 10 days later.
Microscopic examination Microscopic sections revealed a well-differentiated meningeal tumour presenting a lobulated pattern, mostly composed of elongated cells with bipolar nuclei (Fig. 3). In some parts of the tumour, typical whorls were seen with rare psammoma bodies. There were no signs of anaplasia. Immunohistochemistry showed the tumour cells to be strongly positive for Vimentin and negative for Glial Fibrillary Acidic Protein and S-100 protein (Fig. 4). The Ki-67 proliferation index was lower than 1%. Pathological examination concluded to a WHO grade I meningioma of the fibroblastic type.
DISCUSSION Preoperative imaging work-up Obviously, metastasis is the most frequent tumour of the fourth ventricle in adulthood but the relatively benign radiological appearance of this regular-shaped lesion and its solitary character made the diagnosis less likely. Medulloblastoma usually shows a lobulated, lightly irregular border and a heterogeneous signal and enhancement pattern on MR imaging. Haemangioblastoma finds its origin in the paraventricular cerebellum and tends to distort rather than invade the fourth ventricle. Ependymoma or subependymoma is an occasional finding in adulthood but usually shows a more diffuse growing pattern with possible extension through the foramina of Luschka to the ponto-cerebellar cisterns, frequent calcifications and usually mild to moderate contrast enhancement. Lastly, intraventricular meningioma is very uncommon in the fourth ventricle. Both CT and MR imaging findings suggested this last diagnosis, showing a well-circumscribed lesion with regular and smooth borders, probably slow-growing, with a very intense and homogeneous contrast enhancement. As in other meningioma locations, MR imaging is the imaging procedure of choice of fourth ventricle meningioma. 59
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A
B
Fig. 1 Axial T1- (A) and T2- (B) weighted MR images confirming the totally intraventricular location of the tumour that appears isointense to the surrounding grey matter on T2-weighted image without tumour extension through the foramina of Luschka.
Fig. 3 Microscopic examination showing a well-differentiated meningioma, with lobulated pattern, whorls and no signs of anaplasia (Haematoxylin and eosin160). Fig. 2 Sagittal T1-weighted MR image showing a fairly regular intraventricular tumour with very intense and homogenous Gadolinium enhancement. Lesion extension to the foramen of Magendie with slight degree of hydrocephalus.
Review of the literature Meningioma is the commonest nonglial primary brain tumour and accounts for 13–26% of the primary intracranial neoplasms.1,2 Intraventricular meningiomas represent 0.5–5% of the tumours in large adult meningioma series.1,3–7 Several authors found a higher incidence in childhood with 13–44% of intraventricular meningiomas.8,9 It has also been suggested that meningiomas may have a much more rapid and aggressive growth rate in children. However, Germano et al. reported in a retrospective study of combined meningioma series an incidence of 9.4% in this age group with histological types and features similar to those observed in adulthood.10 Intraventricular meningiomas arise mostly within the lateral ventricles (80%), more commonly on the left side for unexplained reasons.1,3–5,7,11 The majority of them (50–68%) are found in the trigone region with the ventricular body as second most common Journal of Clinical Neuroscience (2001) 8(Supplement 1), 59–62
Fig. 4 Immunohistochemical examination demonstrating the Vimentinpositive cells (Vimentin,225).
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Fourth ventricle meningioma with behaviour disorders 61 Table1 Review of the varified intraventricular meningiomas of the fourth ventricle Author Sachs, 1938 Vogel and Stevenson, 1950 Haas and Ritter, 1954 Schaerer and Woolsey, 1960 Hoffman, 1970 Hoffman, 1970 Rodriguez-Carbajal and Palacios, 1974 Rodriguez-Carbajal and Palacios, 1974 Gökalp et al., 1981 Giromini et al., 1981 Tsuboi et al., 1983 Nagata et al., 1983 Matsumara et al., 1988 Ceylan et al., 1992 Lima-de-Freitas et al., 1994 Iseda et al., 1997 Cummings et al., 1999 Chaskis et al., 2000
Sex
Age
Pathology
Treatment
F M
38 65
Fibroblastic Meningothelial
Total removal Autopsy finding
M F
41 42
Fibroblastic Fibroblastic
Autopsy finding Total removal
F M F
44 61 32
Transitional Transitional Meningothelial
Total removel Total removel Total removal
F
49
Meningothelial
Partial removal
F F F F M M F
30 14 30 52 62 48 32
Psammomatous Endotheliomatous Fibroblastic Fibroblastic Fibroblastic Meningothelial Meningotheolial
Total removal Total removal Total removal Total removal Total removal Total removal Total removal
F M
47 72
Transitional Fibroblastic
Total removal Total removal
M
72
Fibroblastic
Total removal
be related to the intermittent occlusion of the fourth ventricle as they arose most often in supine position. A similar mechanism is commonly evoked in the colloid cysts of the third ventricle. The histological types and features in the series were similar to other intracranial locations with fibroblastic, meningothelial and transitional subtypes reported respectively with decreasing frequency. Single cases of psammomatous or endotheliomatous subtypes have been also reported.14,19 A total removal was achieved in all but one operated patient. Splitting the lower vermis, the tumour was easily removed after intracapsular debulking as it is only attached to the choroid plexus. In contrast to posterior fossa meningiomas lying partially within the fourth ventricle, the total removal of purely intraventricular meningiomas can be achieved successfully at low operative risks with an excellent outcome.
CONCLUSIONS Meningiomas of the fourth ventricle are exceptional. Originating from the choroid plexus, the tumour is located purely within the ventricular cavity. A total removal can be achieved successfully even in elderly patients.
ACKNOWLEDGEMENTS
7
1
location. They occasionally arise in the frontal horn. These neoplasms are usually well circumscribed. The origin of intraventricular meningiomas is commonly accepted to be the choroid plexus as small whorls of meningothelial (arachnoidal) cells are common in the normal choroid plexus.1,2 In the present case, the tumour clearly originated from the choroid plexus of the fourth ventricle. There is no specific clinical feature related to the intraventricular location of the tumour. Headache and cognitive disorders are the commonest symptoms of meningiomas of the lateral ventricles.1,3,5,7 The clinical evolution is usually progressive with a mean duration of symptoms of 36 months.11 Motor weakness, homonymous hemianopsia and speech disorders are met less frequently.4 Fifteen percent of intraventricular meningiomas occur within the third ventricle, occupying more commonly the posterior portion.1,5 They commonly present with symptoms of increased intracranial pressure. Unless there are other neoplasms in that location, Parinaud’s syndrome is less common. Hypothalamic disturbances are exceptional.1 Meningiomas of the fourth ventricle are clearly defined as arising from the choroid plexus and lying strictly within the ventricular cavity.12–14 They correspond to the Type 1 of the classification of posterior fossa meningiomas without dural attachment proposed by Abraham and Chandy.15 Through an exhaustive review of the literature, we found only 17 tumours actually fulfilling the above-mentioned definition as shown on Table 1.1,12–14,16–27 Cushing has reported the first successful resection performed by Sachs in 1938.1 Two cases were incidental autopsy findings.16,17 In one case, no microscopic sections were reported.27 The other reports concerned meningiomas of the posterior fossa lying partially within the fourth ventricle or even intracerebellar tumours with marked ventricular shift.15,28–32 In two cases, the description of the pathological findings was suspiciously like ependymoma.33 Reviewing the 18 verified cases, we found a slight female predominance with a sex ratio of 1.6 : 1 women to men. The average age of the patients was 46 years, ranging from 14 to 72. Patients presented with the clinical features of intracranial hypertension related to obstructive hydrocephalus and cerebellar dysfunction, as did our patient. The fluctuating character of his symptoms could © 2001 Harcourt Publishers Ltd
The authors thank the Medical Information Research Services of GlaxoWellcome for assistance in collecting the reports of the literature.
REFERENCES 1.
2.
3. 4.
5. 6. 7. 8.
9. 10. 11.
12. 13. 14.
15. 16. 17.
Cushing H, Eisenhardt L. Meningiomas: Their Classification, Regional Behavior, Life History, and Surgical End Results. Springfield, Ill: Charles C. Thomas, 1938. Lantos PL, VandenBerg SR, Kleihues P. Tumours of the Nervous System. In: Graham DI, Lantos PL (eds) Greenfield’s Neuropathology, 6th edn. London: Arnold, 1996; 583–879. Rohringer M, Sutherland GR, Louw DF, Sima AAF. Incidence and clinicopathological features of meningiomas. J Neurosurg 1989; 71: 665–672. Guidetti B, Delfini J. Lateral and fourth ventricle meningiomas: surgical experience with 61 cases and long-term results. In: Al-Mefty O (ed.) Meningiomas. New York: Raven Press, 1991; 569–581. McDermott MW, Wilson CB. Meningiomas. In: Youmans JR (ed.) Neurological Surgery. Philadelphia: WB Saunders, 1996; 2782–2825. Kobayashi S, Okazaki H, MacCarty C. Intraventricular meningiomas. Mayo Clin Proc 1971; 46: 735–741. Cerullo L, Ghaly R. Meningioma of the Lateral Ventricles. Techniques in Neurosurgery 1998; 4: 21–31. Herz DA, Shapiro K, Shulman K. Intracranial meningiomas in infancy, childhood and adolescence. Review of the literature and addition of 9 case reports. Childs Brain 1980; 7: 43–56. Merten DF, Gooding CA, Newton TH, Malamud N. Meningiomas of childhood and adolescence. J Pediatr 1974; 84: 696–770. Germano IM, Edwards MSB, Davis RL, Schiffer D. Intracranial meningiomas of the first two decades of life. J Neurosurg 1994; 80: 447–453. Fornari M, Savoiardo M, Morello G, Solero CL. Meningioma of the lateral ventricles. Neuroradiological and surgical considerations in 18 cases. J Neurosurg 1981; 54: 64–74. Schaerer JP, Woolsey RD. Intraventricular meningiomas of the fourth ventricle. J Neurosurg 1960; 17: 337–341. Hoffman JC, Bufkin WJ, Richardson HD. Primary intraventricular meningiomas of the fourth ventricle. Am J Radiol 1972; 115: 100–104. Gökalp HZ, Ozkal E, Erdogan A, Selcuki M. A Giant Meningioma of the Fourth Ventricle Associated with Sturge-Weber Disease. Acta Neurochir (Wien) 1981; 57: 115–120. Abraham J, Chandy J. Meningiomas of the posterior fossa without dural attachment: a case report. J Neurosurg 1963; 20: 177–179. Vogel FS, Stevenson LD. Meningothelial meningioma of the fourth ventricle. J Neuropathol Exp Neurol 1950; 9: 443–448. Haas A, Ritter SA. A post-thyroidectomy fatality due to a silent pedunculated meningioma of the fourth ventricle of the brain. Am J Surg 1954; 88: 346–350.
Journal of Clinical Neuroscience (2001) 8(Supplement 1), 59–62
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18.
Rodriguez-Carbajal J, Palacios E. Intraventricular meningiomas of the fourth ventricle. Am J Radiol 1974; 120: 27–31. Giromini D, Pfeiffer J, Tzonos T. Über zwei Fälle von Ventrikelmeningiomeen im Kindesalter. Neurochirurgia 1981; 24: 144–146. Tsuboi K, Nose T, Maki Y. Meningioma of the fourth ventricle: case report. Neurosurgery 1983; 13: 163–166. Nagata K, Basugi N, Sasaki T, Hashimoto K, Manaka S, Takakura K. Intraventricular meningioma of the fourth ventricle. Case report. Neurol Med Chir (Tokyo) 1988; 28: 86–90. Matsumara M, Takahashi, Kurashi H, Tamura M. Primary intraventricular meningioma of the fourth ventricle: case report. Neurol Med Chir (Tokyo) 1988; 28: 996–1000. Ceylan S, Ilbay K, Kuzeyli K, Kalelioglu M, Akturk F, Ozoran Y. Intraventricular meningioma of the fourth ventricle. Clin Neurol Neurosurg 1992; 94: 181–184. Lima de Freitas M, Dourado M, Escartin A, Roig C. Meningioma del IV ventriculo. Migrana sin aura como sintoma inicial. Neurologia 1994; 9: 121–122. Iseda T, Goya T, Nakano S, Wakisaka S. Magnetic resonance imaging and angiographic appearance of meningioma of the fourth ventricle: two case reports. Neurol Med Chir (Tokyo) 1997; 37: 36–40.
19. 20. 21.
22.
23.
24.
25.
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26. 27. 28. 29. 30. 31.
32. 33.
Cummings TJ, Bentley RC, Gray L, Check WE, Lanier TE, McLendon RE. Meningioma of the fourth ventricle. Clin Neuropathol 1999; 18: 265–269. Petit-Dutaillis D, Daum S. Les méningiomes de la fosse postérieure. Rev Neurol 1950; 83: 241–255. Chaffee B, Donaghy P. Case reports and technical notes. Meningioma of the fourth ventricle. J Neurosurg 1963; 20: 520–522. Johnson M, Tulipan N, Whetsell WO: Osteoblastic meningioma of the fourth ventricle. Neurosurgery 1989; 24: 587–590. Nakano S, Uehara H, Wakisaka S, Kinoshita K. Meningioma of the fourth ventricle: case report. Neurol Med Chir (Tokyo) 1989; 29: 52–54. Diaz P, Maillo A, Morales F, Gomez-Moreta JA, Hernandez J. Multiple meningioma of the fourth ventricle in infancy: case report. Neurosurgery 1990; 26: 1057–1060. Delfini R, Capone R, Ciapetta P, Domenicucci M. Meningioma of the fourth ventricle: a case report. Neurosurg Rev 1992; 15: 147–149. Zuleta EB and Londone LR. Meningiomas del IV ventriculo. Acta Neurochir (Wien) 228–232? 1955. Chaffee B and Donaghy P: Case reports and technical notes. Meningioma of the fourth ventricle. J Neurosurg 1963; 20: 520–522.
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Journal of Clinical Neuroscience (2001) 8(Supplement 1), 59–62 © 2001 Harcourt Publishers Ltd doi: 10.1054/jocn.2001.0879, available online at http://www.idealibrary.com on
Meningioma of the fourth ventricle presenting with intermittent behaviour disorders: a case report and review of the literature C. Chaskis MD, T. Buisseret1 MD, A. Michotte2 MD, J. D’Haens MD Departments of Neurosurgery, 1Neuroradiology and 2Neuropathology, Academic Hospital, Vrije Universiteit Brussel, Brussels, Belgium
Summary Intraventricular meningiomas are rare, representing 0.5–5% of all intracranial meningiomas. They arise mostly within the lateral ventricles and more rarely in the third ventricle. Meningiomas of the fourth ventricle are exceptional. They are clearly defined as meningiomas arising from the choroid plexus and lying strictly within the fourth ventricle. We report a 76 year old male patient presenting with a 2-week history of headache and cognitive disorders with agitation and restlessness particularly exacerbated at night or when lying down. CT scan and MR imaging showed a contrast-enhancing lesion located purely within the whole fourth ventricle, with slight ventricular enlargement. At surgery, we totally removed a well-vascularised, greyish encapsulated mass attached to the choroid plexus. Pathological examination revealed a WHO grade I fibroblastic meningioma. We reviewed the literature concerning this unusual meningioma location. © 2001 Harcourt Publishers Ltd Keywords: fourth ventricle, neoplasm, meningioma, choroid plexus, hydrocephalus
CASE STUDY Clinical history We report the case of a 76 year old male patient admitted on emergency after an episode of seizures related to sudden withdrawal of benzodiazepine therapy. Two weeks before admission, he developed intermittent headache and behaviour disturbances with agitation and restlessness. The symptoms were particularly exacerbated at night or when lying down. Neurological examination on admission revealed spatial-temporal disorientation, impairment of memory functions and attention span, ataxic dysarthria and ataxia of gait with a Romberg sign.
Imaging CT scan showed a homogeneous spontaneously hyperdense lesion occupying the whole fourth ventricle, with an enlarged aqueduct and slightly enlarged third ventricle and temporal ventricle horns. There were neither intralesional calcifications nor cyst formations. MR imaging confirmed the exclusively intraventricular location of the tumour that appeared isointense to the surrounding grey matter on both T1- and T2-weighted spin-echo sequences (Fig. 1A), with very intense and homogenous enhancement after intravenous injection of Gadolinium-DTPA (Fig. 1B). MR imaging also confirmed the slight degree of installing or possibly episodic obstructive hydrocephalus. The lesion was fairly regular with smooth margins. It had a caudal extension to the foramen of Magendie but the cisterna magna was free (Fig. 2). There was no extension through the foramina of Luschka.
Surgical treatment Surgery was carried out with the patient prone. Through a midline incision, a suboccipital craniotomy was performed and the dura opened. Splitting the lower vermis, we totally removed a firm, well-vascularised, greyish, encapsulated mass lying more or less free in the fourth ventricle, only attached to the choroid plexus. The patient was completely normal postoperatively. Ten days after surgery, he developed somnolence. CT scan demonstrated a small blood clot in the foramen of Magendie and
Correspondence to: Cristo Chaskis MD, Department of Neurosurgery, A.Z.-V.U.B., Laarbeeklaan 101, 1090 Brussels, Belgium. Tel.:;322 4775514; Fax:;322 4776505; E-mail: [email protected]
diffuse cerebellar oedema causing obstructive hydrocephalus. The patient recovered completely after ventriculoperitoneal shunting. The further postoperative course was uneventful and the patient was discharged home 10 days later.
Microscopic examination Microscopic sections revealed a well-differentiated meningeal tumour presenting a lobulated pattern, mostly composed of elongated cells with bipolar nuclei (Fig. 3). In some parts of the tumour, typical whorls were seen with rare psammoma bodies. There were no signs of anaplasia. Immunohistochemistry showed the tumour cells to be strongly positive for Vimentin and negative for Glial Fibrillary Acidic Protein and S-100 protein (Fig. 4). The Ki-67 proliferation index was lower than 1%. Pathological examination concluded to a WHO grade I meningioma of the fibroblastic type.
DISCUSSION Preoperative imaging work-up Obviously, metastasis is the most frequent tumour of the fourth ventricle in adulthood but the relatively benign radiological appearance of this regular-shaped lesion and its solitary character made the diagnosis less likely. Medulloblastoma usually shows a lobulated, lightly irregular border and a heterogeneous signal and enhancement pattern on MR imaging. Haemangioblastoma finds its origin in the paraventricular cerebellum and tends to distort rather than invade the fourth ventricle. Ependymoma or subependymoma is an occasional finding in adulthood but usually shows a more diffuse growing pattern with possible extension through the foramina of Luschka to the ponto-cerebellar cisterns, frequent calcifications and usually mild to moderate contrast enhancement. Lastly, intraventricular meningioma is very uncommon in the fourth ventricle. Both CT and MR imaging findings suggested this last diagnosis, showing a well-circumscribed lesion with regular and smooth borders, probably slow-growing, with a very intense and homogeneous contrast enhancement. As in other meningioma locations, MR imaging is the imaging procedure of choice of fourth ventricle meningioma. 59
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A
B
Fig. 1 Axial T1- (A) and T2- (B) weighted MR images confirming the totally intraventricular location of the tumour that appears isointense to the surrounding grey matter on T2-weighted image without tumour extension through the foramina of Luschka.
Fig. 3 Microscopic examination showing a well-differentiated meningioma, with lobulated pattern, whorls and no signs of anaplasia (Haematoxylin and eosin160). Fig. 2 Sagittal T1-weighted MR image showing a fairly regular intraventricular tumour with very intense and homogenous Gadolinium enhancement. Lesion extension to the foramen of Magendie with slight degree of hydrocephalus.
Review of the literature Meningioma is the commonest nonglial primary brain tumour and accounts for 13–26% of the primary intracranial neoplasms.1,2 Intraventricular meningiomas represent 0.5–5% of the tumours in large adult meningioma series.1,3–7 Several authors found a higher incidence in childhood with 13–44% of intraventricular meningiomas.8,9 It has also been suggested that meningiomas may have a much more rapid and aggressive growth rate in children. However, Germano et al. reported in a retrospective study of combined meningioma series an incidence of 9.4% in this age group with histological types and features similar to those observed in adulthood.10 Intraventricular meningiomas arise mostly within the lateral ventricles (80%), more commonly on the left side for unexplained reasons.1,3–5,7,11 The majority of them (50–68%) are found in the trigone region with the ventricular body as second most common Journal of Clinical Neuroscience (2001) 8(Supplement 1), 59–62
Fig. 4 Immunohistochemical examination demonstrating the Vimentinpositive cells (Vimentin,225).
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Fourth ventricle meningioma with behaviour disorders 61 Table1 Review of the varified intraventricular meningiomas of the fourth ventricle Author Sachs, 1938 Vogel and Stevenson, 1950 Haas and Ritter, 1954 Schaerer and Woolsey, 1960 Hoffman, 1970 Hoffman, 1970 Rodriguez-Carbajal and Palacios, 1974 Rodriguez-Carbajal and Palacios, 1974 Gökalp et al., 1981 Giromini et al., 1981 Tsuboi et al., 1983 Nagata et al., 1983 Matsumara et al., 1988 Ceylan et al., 1992 Lima-de-Freitas et al., 1994 Iseda et al., 1997 Cummings et al., 1999 Chaskis et al., 2000
Sex
Age
Pathology
Treatment
F M
38 65
Fibroblastic Meningothelial
Total removal Autopsy finding
M F
41 42
Fibroblastic Fibroblastic
Autopsy finding Total removal
F M F
44 61 32
Transitional Transitional Meningothelial
Total removel Total removel Total removal
F
49
Meningothelial
Partial removal
F F F F M M F
30 14 30 52 62 48 32
Psammomatous Endotheliomatous Fibroblastic Fibroblastic Fibroblastic Meningothelial Meningotheolial
Total removal Total removal Total removal Total removal Total removal Total removal Total removal
F M
47 72
Transitional Fibroblastic
Total removal Total removal
M
72
Fibroblastic
Total removal
be related to the intermittent occlusion of the fourth ventricle as they arose most often in supine position. A similar mechanism is commonly evoked in the colloid cysts of the third ventricle. The histological types and features in the series were similar to other intracranial locations with fibroblastic, meningothelial and transitional subtypes reported respectively with decreasing frequency. Single cases of psammomatous or endotheliomatous subtypes have been also reported.14,19 A total removal was achieved in all but one operated patient. Splitting the lower vermis, the tumour was easily removed after intracapsular debulking as it is only attached to the choroid plexus. In contrast to posterior fossa meningiomas lying partially within the fourth ventricle, the total removal of purely intraventricular meningiomas can be achieved successfully at low operative risks with an excellent outcome.
CONCLUSIONS Meningiomas of the fourth ventricle are exceptional. Originating from the choroid plexus, the tumour is located purely within the ventricular cavity. A total removal can be achieved successfully even in elderly patients.
ACKNOWLEDGEMENTS
7
1
location. They occasionally arise in the frontal horn. These neoplasms are usually well circumscribed. The origin of intraventricular meningiomas is commonly accepted to be the choroid plexus as small whorls of meningothelial (arachnoidal) cells are common in the normal choroid plexus.1,2 In the present case, the tumour clearly originated from the choroid plexus of the fourth ventricle. There is no specific clinical feature related to the intraventricular location of the tumour. Headache and cognitive disorders are the commonest symptoms of meningiomas of the lateral ventricles.1,3,5,7 The clinical evolution is usually progressive with a mean duration of symptoms of 36 months.11 Motor weakness, homonymous hemianopsia and speech disorders are met less frequently.4 Fifteen percent of intraventricular meningiomas occur within the third ventricle, occupying more commonly the posterior portion.1,5 They commonly present with symptoms of increased intracranial pressure. Unless there are other neoplasms in that location, Parinaud’s syndrome is less common. Hypothalamic disturbances are exceptional.1 Meningiomas of the fourth ventricle are clearly defined as arising from the choroid plexus and lying strictly within the ventricular cavity.12–14 They correspond to the Type 1 of the classification of posterior fossa meningiomas without dural attachment proposed by Abraham and Chandy.15 Through an exhaustive review of the literature, we found only 17 tumours actually fulfilling the above-mentioned definition as shown on Table 1.1,12–14,16–27 Cushing has reported the first successful resection performed by Sachs in 1938.1 Two cases were incidental autopsy findings.16,17 In one case, no microscopic sections were reported.27 The other reports concerned meningiomas of the posterior fossa lying partially within the fourth ventricle or even intracerebellar tumours with marked ventricular shift.15,28–32 In two cases, the description of the pathological findings was suspiciously like ependymoma.33 Reviewing the 18 verified cases, we found a slight female predominance with a sex ratio of 1.6 : 1 women to men. The average age of the patients was 46 years, ranging from 14 to 72. Patients presented with the clinical features of intracranial hypertension related to obstructive hydrocephalus and cerebellar dysfunction, as did our patient. The fluctuating character of his symptoms could © 2001 Harcourt Publishers Ltd
The authors thank the Medical Information Research Services of GlaxoWellcome for assistance in collecting the reports of the literature.
REFERENCES 1.
2.
3. 4.
5. 6. 7. 8.
9. 10. 11.
12. 13. 14.
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Cushing H, Eisenhardt L. Meningiomas: Their Classification, Regional Behavior, Life History, and Surgical End Results. Springfield, Ill: Charles C. Thomas, 1938. Lantos PL, VandenBerg SR, Kleihues P. Tumours of the Nervous System. In: Graham DI, Lantos PL (eds) Greenfield’s Neuropathology, 6th edn. London: Arnold, 1996; 583–879. Rohringer M, Sutherland GR, Louw DF, Sima AAF. Incidence and clinicopathological features of meningiomas. J Neurosurg 1989; 71: 665–672. Guidetti B, Delfini J. Lateral and fourth ventricle meningiomas: surgical experience with 61 cases and long-term results. In: Al-Mefty O (ed.) Meningiomas. New York: Raven Press, 1991; 569–581. McDermott MW, Wilson CB. Meningiomas. In: Youmans JR (ed.) Neurological Surgery. Philadelphia: WB Saunders, 1996; 2782–2825. Kobayashi S, Okazaki H, MacCarty C. Intraventricular meningiomas. Mayo Clin Proc 1971; 46: 735–741. Cerullo L, Ghaly R. Meningioma of the Lateral Ventricles. Techniques in Neurosurgery 1998; 4: 21–31. Herz DA, Shapiro K, Shulman K. Intracranial meningiomas in infancy, childhood and adolescence. Review of the literature and addition of 9 case reports. Childs Brain 1980; 7: 43–56. Merten DF, Gooding CA, Newton TH, Malamud N. Meningiomas of childhood and adolescence. J Pediatr 1974; 84: 696–770. Germano IM, Edwards MSB, Davis RL, Schiffer D. Intracranial meningiomas of the first two decades of life. J Neurosurg 1994; 80: 447–453. Fornari M, Savoiardo M, Morello G, Solero CL. Meningioma of the lateral ventricles. Neuroradiological and surgical considerations in 18 cases. J Neurosurg 1981; 54: 64–74. Schaerer JP, Woolsey RD. Intraventricular meningiomas of the fourth ventricle. J Neurosurg 1960; 17: 337–341. Hoffman JC, Bufkin WJ, Richardson HD. Primary intraventricular meningiomas of the fourth ventricle. Am J Radiol 1972; 115: 100–104. Gökalp HZ, Ozkal E, Erdogan A, Selcuki M. A Giant Meningioma of the Fourth Ventricle Associated with Sturge-Weber Disease. Acta Neurochir (Wien) 1981; 57: 115–120. Abraham J, Chandy J. Meningiomas of the posterior fossa without dural attachment: a case report. J Neurosurg 1963; 20: 177–179. Vogel FS, Stevenson LD. Meningothelial meningioma of the fourth ventricle. J Neuropathol Exp Neurol 1950; 9: 443–448. Haas A, Ritter SA. A post-thyroidectomy fatality due to a silent pedunculated meningioma of the fourth ventricle of the brain. Am J Surg 1954; 88: 346–350.
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