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DOI: 10.1055/s-0037-1602169
Meningioma of the Fourth Ventricle: Literature Review[*]
Article in several languages: English | portuguêsAddress for correspondence
Publication History
30 December 2016
16 March 2017
Publication Date:
17 April 2017 (online)
Abstract
Meningiomas are among the most common central nervous system tumors, with an incidence that ranges from 15% to 40% of intracranial tumors. Of these, only 0.5% to 3% are intraventricular, and the rarest of them occurs in the fourth ventricle.
Fourth-ventricle meningiomas originate generally from the choroid plexus and have no dural adhesions. Most often, they manifest in young patients, around 41 years of age, with a possible predominance in females, through intracranial hypertension and cerebellar syndromes. The treatment consists of surgical resection, which commonly presents good results due to the characteristics of the tumor. So, for better preoperative planning, the radiological differentiation of the most frequent tumors in this location is important.
The most common histologic subtypes are fibroblastic and meningothelial, both grade I according to the World Health Organization (WHO), although there are reports of tumors of grades II and III.
We report a case of meningioma of the fourth ventricle operated in our institution, and we have conducted a literature review, through which we found that 57 cases have been reported so far, with the first one reported in 1938.
#
Introduction
Meningiomas are meningothelial neoplasms that originate in the arachnoid fibroblast, a squamous cell that covers the arachnoid villi and the Pacchioni granulations. They correspond to at least 15% of all intracranial neoplasms,[1] but may reach 40%,[2] and are classically adhered to meningeal structures.
Purely intraventricular lesions are rare and have a calculated incidence between 0.5% and 3% of intracranial meningiomas.[2] Out of these, 77.8% occur in the lateral ventricle trigone, with a higher frequency on the left side; 15.6% occur in the third ventricle; and 6.6%, in the fourth ventricle.[1] [3] [4]
It is believed that the meningiomas in the fourth ventricle originate from the choroid plexus, and do not present dural adhesions.[1] [3] [5] The first case was described by Cushing and operated by Sachs in 1938.[3] [6] [7]
We describe the case of a 31-year-old patient, who was operated at our service for resection of a tumor in the fourth ventricle whose anatomopathological diagnosis indicated meningioma; we also performed a review of the literature.
#
Case Report
A 31-year-old male patient, from the city of Caçu, state of Goiás, Midwestern Brazil, was referred to Hospital de Amor, in the city of Barretos, with a vertiginous condition that had been progressing for ∼ 8 months, associated with nausea and malaise. Upon neurological examination, he manifested dysfunction of cranial nerves IX, X, XI and XII and cerebellar syndrome, which presented in the form of dysbasia, ataxia and dysmetria, and was more pronounced on the left side. The subsequent investigation, with magnetic resonance imaging (MRI) ([Figs. 1], [2] and [3]), revealed a solid lesion of lobulated contour and intense enhancement after injection of a paramagnetic contrast medium in the Luschka foramen topography to the left and fourth ventricle. The lesion had thin cerebrospinal fluid fissure between the cerebellar vermis and the tumoral plane, with 4.7 × 4.0 cm in its major axes, compressing the bulb, without contact with adjacent meninx, with no dural tail identification. There was also a slight dilation of the supratentorial cerebral ventricles.
A bilateral suboccipital craniotomy was performed, with the installation of an external ventricular bypass and total macroscopic excision of the lesion ([Figs. 4], [5] and [6]), without intercurrences. During the intraoperative period, absence of tumor adhesions to the meningeal structures of the posterior fossa was confirmed. A microsurgical resection of the lesion was performed, and we opted for intralesional emptying with the aid of ultrasonic aspiration, followed by resection in fragments of the tumor remnant, without the use of electrophysiological monitoring, which was not available at the time, and avoiding traction maneuvers. Total macroscopic resection of the neoplasm was achieved.
In the postoperative period, the condition evolved with worsening of the dysfunctions of cranial nerves IX, X and XI and grade-III hemiparesis to the left, presenting a slow and progressive improvement. The patient underwent early tracheostomy and gastrostomy.
A picture of bronchopneumonia was diagnosed, with probable aspiration etiology, and urinary tract infection, which were treated satisfactorily, in addition to cerebrospinal fluid fistula associated with hydrocephalus, which was readily treated with a ventricular-peritoneal shunt (VPS) during the same hospitalization. The patient evolved with prolonged hospitalization and was enrolled into an intensive physical rehabilitation program.
The patient followed with progressive improvement of the deficits after hospital discharge, and, around 9 months after the procedure, presented complete recovery of the cranial nerve impairment, significant reversal of motor deficit, and significant improvement in the ataxic frame, maintaining a slight incoordination on the left side.
The histopathologic study revealed that it was a fibroblastic meningioma, grade I according to the World Health Organization (WHO). After 2 years of follow-up, he was referred to the hospital service in his hometown due to dysfunction of the DVS and history of viral meningitis treated in that city months before. The patient was then diagnosed again with meningitis and presented rapid worsening in the clinical condition due to sepsis, which caused his death despite the treatment.
#
Discussion
Meningiomas of the fourth ventricle are defined as those that have their origin in the local choroid plexus and occupy this ventricular cavity without meningeal implantation.[1] [3] [5] In 1963, Abraham and Chandy suggested a classification for posterior fossa meningiomas, without dural implant, consisting of three types: 1) choroid plexus meningiomas, which develop only in the fourth ventricle; 2) choroid screen meningiomas, which develop partially in the interior of the ventricle and partially in the cerebellar hemisphere and vermis; and 3) cisterna magna meningiomas, without dural implantation and with intraventricular extension, which are originate from the most lateral portion of the choroid plexus, outside the Luschka formen.[1] [8] [9] Those classified as types 1 or 2 are deemed true. The case herein reported is an example of ventricle meningiomas classified as Abraham type I, as there is evidence of cerebrospinal fluid fissure between the vertex and the medial portion of the right cerebellar hemisphere with the tumor interface, as well as a more lateralized disposition of the neoplasia on the left.
Some published series have reported a prevalence of female patients, with a 2:1 ratio.[1] [4] [10] In this review, we noted an equivalent distribution between genders, with a slight prevalence of females, with 28 cases (48.28%). There were 25 male cases (43.10%), and there was no identification of gender in 5 cases (8.62%).
The overall mean age of the reported cases is 41.64 years old. When analyzed separately by gender, the mean age is discretely different: the female gender is slightly younger (mean age: 41.25 years) than the male gender (mean age: 42.08 years). Some publications suggest a higher incidence in younger female patients.[1]
Patients with fourth-ventricle meningiomas usually manifest signs of insidious intracranial hypertension (IH), such as morning headache, nausea, vomiting and vertigo, as well as focal signs most commonly characterized by ataxic syndrome (cerebellar), long tracts,[2] [3] [10] and cranial nerve involvement, notably those whose nuclei are located in the point-bulbar segment of the brainstem, such as the presently reported case.
Among the differential diagnosis of fourth-ventricle tumors are metastasis, choroid plexus papilloma, hemangioblastoma, medulloblastoma and ependymoma.[3] [5] [11] The radiological differentiation of such lesions is important for the surgical planning, because it implies different levels of difficulty for total excision and, therefore, different initial forms of intraoperative management of the lesions. The characteristics of meningiomas, both in the MRI and in computeed tomograhy (CT), indicate well-circumscribed lesions with regular and mild edges, probably of slow growth, with homogeneous and very intense enhancement by the contrast agent.[3] [6]
The treatment of choice is the microsurgical excision of the lesion, using intralesional emptying, with aid, if possible, given the characteristics of the neoplasia, ultrasonic aspiration or piacemeal resection when necessary, initially avoiding traction maneuvers, and paying special attention to the dissection along the floor of the fourth ventricle, usually with a clear cleavage plane, which, together with the fact that it does not present meningeal fixation, leads to the good results published in the literature, with complete excision of the lesion.[12] Considering the cases with resection volumes reported so far, only four did not indicate complete resection.
Data from the international literature suggest the prevalence of the fibroblastic and meningothelial subtypes, corresponding respectively to 40% and 24% of the cases.[1] In our review, the subtypes classified as WHO grade I correspond to 79.31% of the cases, with a prevalence of fibroblastic (29.31%) and meningothelial (15.52%) meningiomas.
Meningiomas classified as WHO grade II correspond to 17.24% of the cases, with a prevalence of clear cell (6.89%) and atypical (6.89%) meningiomas. Chordoid meningiomas corresponded to 3.44% (3) of the reported cases.[13] [14] Only one anaplastic meningioma (WHO grade III) was reported, whose diagnosis was established in the recurrence of the disease after a long period of remission.[15] The histopathologic classification of the lesion was not available in 5 (8.62%) reported cases.
Through research in the database of indexed journals, as shown in[Table 1], we detected the existence of 57 cases of meningiomas of the fourth ventricle, and 40 publications or references to them are in articles in English or Spanish.
Case |
Publication |
Author |
Year |
Age |
Gender |
Resection |
Histology |
---|---|---|---|---|---|---|---|
1 |
1 |
1938 |
38 |
Female |
Total |
Fibroblastic |
|
2 |
2 |
Voguel and Stevenson[7] |
1950 |
65 |
Male |
Necropsy |
Meningothelial |
3 |
3 |
Haas and Ritter[16] |
1954 |
41 |
Male |
Necropsy |
Not available |
4 |
4 |
Bustamente Zuleta and Londono[17] |
1955 |
12 |
Male |
Subtotal |
Laminar trend |
5 |
4 |
Bustamente Zuleta and Londono[17] |
8 |
Male |
Biopsy |
Diffuse |
|
6 |
5 |
Schaerer and Woosley[18] |
1960 |
42 |
Female |
Total |
Not available |
7 |
6 |
Chafee and Donaghy[19] |
1963 |
38 |
Female |
Total |
Meningothelial |
8 |
7 |
Hoffman et al[20] |
1972 |
61 |
Male |
Total |
Transitional |
9 |
7 |
Hoffman et al[20] |
44 |
Female |
Total |
Transitional |
|
10 |
8 |
Rodrigues- Carbajal and Palacios[21] |
1974 |
49 |
Female |
Partial |
Meningothelial |
11 |
8 |
Rodrigues-Carbajal and Palacios[21] |
32 |
Female |
Total |
Meningothelial |
|
12 |
9 |
Gökalp et al[22] |
1981 |
30 |
Female |
Total |
Psammomatous |
13 |
10 |
Tsuboi et al[23] |
1983 |
30 |
Female |
Total |
Fibroblastic |
14 |
11 |
Nagata et al[24] |
1988 |
52 |
Female |
Total |
Fibroblastic |
15 |
12 |
Matsumara et al[25] |
1988 |
62 |
Male |
Total |
Fibroblastic |
16 |
13 |
Nakano et al[26] |
1989 |
58 |
Female |
Total |
Transitional |
17 |
14 |
Jhonson et al[27] |
1989 |
53 |
Male |
Total |
Osteoblastic |
18 |
15 |
Diaz et al[28] |
1990 |
5 |
Female |
Total |
Meningothelial |
19 |
16 |
Ceylan et al[9] |
1992 |
48 |
Male |
Total |
Angiomatous |
20 |
17 |
Delfini and al[29] |
1992 |
22 |
Male |
Total |
Fibroblastic |
21 |
18 |
Lima de Freitas et al[30] |
1994 |
32 |
Female |
Total |
Meningothelial |
22 |
19 |
Iseda et al[31] |
1997 |
67 |
Female |
Total |
Atypical |
23 |
19 |
Iseda et al[31] |
47 |
Female |
Total |
Transitional |
|
24 |
20 |
Cummings et al[32] |
1999 |
72 |
Male |
Total |
Fibroblastic |
25 |
21 |
Chaskis et al[6] |
2001 |
76 |
Male |
Total |
Fibroblastic |
26 |
22 |
Akimoto et al[12] |
2001 |
72 |
Female |
Total |
Transitional |
27 |
23 |
Ooigawa et al[11] |
2004 |
51 |
Female |
Total |
Transitional |
28 |
24 |
Carlotti et al[33] |
2003 |
23 |
Female |
Total |
Clear cells |
29 |
24 |
Carlotti et al[33] |
28 |
Female |
Total |
Clear cells |
|
30 |
25 |
Bathoe et al[34] |
2006 |
Not available |
Not available |
Not available |
Fibroblastic |
31 |
25 |
Bathoe et al[34] |
Not available |
Not available |
Not available |
Meningothelial |
|
32 |
26 |
Liu et al[4] |
2006 |
Not available |
Not available |
Not available |
Mixed |
33 |
27 |
Epari et al[14] |
2006 |
20 |
Female |
Total |
Chordoid |
34 |
28 |
Bertalanffy et al[35] |
2006 |
Not available |
Not available |
Total |
Not available |
35 |
29 |
da Costa et al[36] |
2007 |
45 |
Male |
Total |
Not available |
36 |
30 |
Shintaku et al[15] |
2007 |
61 |
Female |
Total |
Anaplastic |
37 |
31 |
Wind and al[13] |
2010 |
23 |
Male |
Total |
Chordoid |
38 |
32 |
Burgan et al[37] |
2010 |
14 |
Male |
Total |
Clear cells |
39 |
33 |
Alver et al[1] |
2011 |
61 |
Male |
Total |
Fibroblastic |
40 |
34 |
Pichierri et al[8] |
2011 |
30 |
Female |
Not available |
Meningothelial |
41 |
34 |
Pichierri et al[8] |
22 |
Male |
Not available |
Fibroblastic |
|
42 |
34 |
Pichierri et al[8] |
22 |
Male |
Not available |
Fibroblastic |
|
43 |
35 |
Zhang et al[38] |
2012 |
23 |
Male |
Total |
Angiomatous |
44 |
36 |
Qin et al[5] |
2012 |
25 |
Male |
Not available |
Fibrous |
45 |
37 |
Takeuchi et al[3] |
2012 |
60 |
Male |
Subtotal |
Meningothelial |
46 |
38 |
Zhang et al[10] |
2012 |
40 |
Female |
Not available |
Psammomatous |
47 |
38 |
Zhang et al[10] |
43 |
Female |
Not available |
Clear cells |
|
48 |
38 |
Zhang et al[10] |
65 |
Female |
Not available |
Fibroblastic |
|
49 |
38 |
Zhang et al[10] |
60 |
Female |
Not available |
Fibroblastic |
|
50 |
38 |
Zhang et al[10] |
20 |
Female |
Not available |
Transitional |
|
51 |
38 |
Zhang et al[10] |
39 |
Male |
Not available |
Fibroblastic |
|
52 |
38 |
Zhang et al[10] |
50 |
Male |
Not available |
Atypical |
|
53 |
38 |
Zhang et al[10] |
9 |
Female |
Not available |
Fibroblastic |
|
54 |
38 |
Zhang et al[10] |
69 |
Female |
Not available |
Fibroblastic |
|
55 |
38 |
Zhang et al[10] |
57 |
Male |
Not available |
Atypical |
|
56 |
39 |
Ødegaard et al[2] |
2013 |
Not available |
Not available |
Not available |
Not available |
57 |
40 |
Liu and Kasper[39] |
2014 |
60 |
Male |
Total |
Atypical |
#
Conclusion
Fourth-ventricle meningiomas have little incidence, possibly with a slight prevalence in young women, commonly presenting with progressive symptoms of IH and cerebellar syndrome. The surgical treatment is usually effective and has good results, considering, for that, adequate preoperative planning obtained through the radiological characteristics of the tumors. The low-grade lesions are the majority, although there is a broad spectrum of diagnosed subtypes.
#
#
Conflict of Interests
The authors have no conflict of interests to declare.
* Work developed at the Orthopedics and Traumatology Service, Centro Médico Hospitalar de Vila Velha (CMHVV), Vila Velha, ES, Brazil.
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References
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- 2 Ødegaard KM, Helseth E, Meling TR. Intraventricular meningiomas: a consecutive series of 22 patients and literature review. Neurosurg Rev 2013; 36 (01) 57-64 , discussion 64
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- 14 Epari S, Sharma MÇ, Sarkar C, Garg A, Gupta A, Mehta VS. Chordoid meningioma, an uncommon variant of meningioma: a clinicopathologic study of 12 cases. J Neurooncol 2006; 78 (03) 263-269
- 15 Shintaku M, Hashimoto K, Okamoto S. Intraventricular meningioma with anaplastic transformation and metastasis via the cerebrospinal fluid. Neuropathology 2007; 27 (05) 448-452
- 16 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 (02) 346-350
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- 18 Schaerer JP, Woolsey RD. Intraventricular meningiomas of the fourth ventricle. J Neurosurg 1960; 17: 337-341
- 19 Chaffee B, Donaghy RM. MENINGIOMA OF THE FOURTH VENTRICLE. J Neurosurg 1963; 20: 520-522
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- 21 Rodriguez-Carbajal J, Palacios E. Intraventricular meningiomas of the fourth ventricle. Am J Roentgenol Radium Ther Nucl Med 1974; 120 (01) 27-31
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- 23 Tsuboi K, Nose T, Maki Y. Meningioma of the fourth ventricle: a case report. Neurosurgery 1983; 13 (02) 163-166
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- 29 Delfini R, Capone R, Ciappetta P, Domenicucci M. Meningioma of the fourth ventricle: a case report. Neurosurg Rev 1992; 15 (02) 147-149
- 30 Lima de Freitas M, Dourado M, Escartín A, Roig C. [Meningioma of the fourth ventricle. Migraine without aura as the first sign]. Neurologia 1994; 9 (03) 121-122
- 31 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 (01) 36-40
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- 33 Carlotti Jr CG, Neder L, Colli BO. , et al. Clear cell meningioma of the fourth ventricle. Am J Surg Pathol 2003; 27 (01) 131-135
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Address for correspondence
-
References
- 1 Alver I, Abuzayed B, Kafadar AM, Muhammedrezai S, Sanus GZ, Akar Z. Primary fourth ventricular meningioma: case report and review of the literature. Turk Neurosurg 2011; 21 (02) 249-253
- 2 Ødegaard KM, Helseth E, Meling TR. Intraventricular meningiomas: a consecutive series of 22 patients and literature review. Neurosurg Rev 2013; 36 (01) 57-64 , discussion 64
- 3 Takeuchi S, Sugawara T, Masaoka H, Takasato Y. Fourth ventricular meningioma: a case report and literature review. Acta Neurol Belg 2012; 112 (01) 97-100
- 4 Liu M, Wei Y, Liu Y, Zhu S, Li X. Intraventricular meninigiomas: a report of 25 cases. Neurosurg Rev 2006; 29 (01) 36-40
- 5 Qin Y, Kanasaki Y, Takasugi M. , et al. Primary fourth ventricular meningioma: a case report of an adult male. Clin Imaging 2012; 36 (04) 379-382
- 6 Chaskis C, Buisseret T, Michotte A, D'Haens J. Meningioma of the fourth ventricle presenting with intermittent behaviour disorders: a case report and review of the literature. J Clin Neurosci 2001; 8 (Suppl. 01) 59-62
- 7 Vogel FS, Stevenson LD. Meningothelial meningioma of the fourth ventricle. J Neuropathol Exp Neurol 1950; 9 (04) 443-448
- 8 Pichierri A, Ruggeri A, Morselli C, Delfini R. Fourth ventricle meningiomas: a rare entity. Br J Neurosurg 2011; 25 (04) 454-458
- 9 Ceylan S, Ilbay K, Kuzeylï K, Kalelïoğlu M, Aktürk F, Ozoran Y. Intraventricular meningioma of the fourth ventricle. Clin Neurol Neurosurg 1992; 94 (02) 181-184
- 10 Zhang BY, Yin B, Li YX. , et al. Neuroradiological findings and clinical features of fourth-ventricular meningioma: a study of 10 cases. Clin Radiol 2012; 67 (05) 455-460
- 11 Ooigawa H, Miyazawa T, Otani N, Fukui S, Nawashiro H, Shima K. Usefulness of thallium-201 chloride single photon emission computed tomography for the preoperative diagnosis of fourth ventricle meningioma--case report. Neurol Med Chir (Tokyo) 2004; 44 (12) 660-664
- 12 Akimoto J, Sato Y, Tsutsumi M, Haraoka J. Fourth ventricular meningioma in an adult--case report. Neurol Med Chir (Tokyo) 2001; 41 (08) 402-405
- 13 Wind JJ, Jones RV, Roberti F. Fourth ventricular chordoid meningioma. J Clin Neurosci 2010; 17 (10) 1301-1303
- 14 Epari S, Sharma MÇ, Sarkar C, Garg A, Gupta A, Mehta VS. Chordoid meningioma, an uncommon variant of meningioma: a clinicopathologic study of 12 cases. J Neurooncol 2006; 78 (03) 263-269
- 15 Shintaku M, Hashimoto K, Okamoto S. Intraventricular meningioma with anaplastic transformation and metastasis via the cerebrospinal fluid. Neuropathology 2007; 27 (05) 448-452
- 16 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 (02) 346-350
- 17 Bustamante Zuleta E, Londono R. [Meningiomas of the fourth ventricle]. Acta Neurochir (Wien) 1955; 4 (03) 228-232
- 18 Schaerer JP, Woolsey RD. Intraventricular meningiomas of the fourth ventricle. J Neurosurg 1960; 17: 337-341
- 19 Chaffee B, Donaghy RM. MENINGIOMA OF THE FOURTH VENTRICLE. J Neurosurg 1963; 20: 520-522
- 20 Hoffman Jr JC, Bufkin WJ, Richardson HD. Primary intraventricular meningiomas of the fourth ventricle. Am J Roentgenol Radium Ther Nucl Med 1972; 115 (01) 100-104
- 21 Rodriguez-Carbajal J, Palacios E. Intraventricular meningiomas of the fourth ventricle. Am J Roentgenol Radium Ther Nucl Med 1974; 120 (01) 27-31
- 22 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 (1-2): 115-120
- 23 Tsuboi K, Nose T, Maki Y. Meningioma of the fourth ventricle: a case report. Neurosurgery 1983; 13 (02) 163-166
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