CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2020; 39(01): 005-011
DOI: 10.1055/s-0037-1602169
Review Article | Artigo de Revisão
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Meningioma of the Fourth Ventricle: Literature Review[*]

Article in several languages: English | português
Allan Dias Polverini
1  Department of Neurosurgery, Hospital de Amor, Barretos, SP, Brazil
,
1  Department of Neurosurgery, Hospital de Amor, Barretos, SP, Brazil
,
Guilherme Fonseca Bortoluzzi
1  Department of Neurosurgery, Hospital de Amor, Barretos, SP, Brazil
,
Ismael Augusto Silva Lombardi
1  Department of Neurosurgery, Hospital de Amor, Barretos, SP, Brazil
,
Giusepe Picone Junior
1  Department of Neurosurgery, Hospital de Amor, Barretos, SP, Brazil
,
Carlos Roberto de Almeida Junior
1  Department of Neurosurgery, Hospital de Amor, Barretos, SP, Brazil
,
Carlos Afonso Clara
1  Department of Neurosurgery, Hospital de Amor, Barretos, SP, Brazil
› Author Affiliations
Further Information

Address for correspondence

Rodrigo de Almeida Simon Sola, MSc
Rua Coronel Bento
394, Vilas Boas, Campo Grande, MS, 79051-110
Brazil   

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.


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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.


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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.

Zoom Image
Fig. 1 Encephalon T1-weighted MRI scan with contrast showing tumor mass in the region of the fourth ventricle, with homogeneous enhancement and significant mass effect on the brainstem in the axial section.
Zoom Image
Fig. 2 Encephalon T1-weighted MRI scan with contrast showing tumor mass in the region of the fourth ventricle, with homogeneous enhancement and significant mass effect on the brainstem in the coronal section.
Zoom Image
Fig. 3 Encephalon T1-weighted MRI scan with contrast demonstrating tumor mass in the region of the fourth ventricle, with homogeneous enhancement and an important mass effect on the brainstem in the sagittal section.

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.

Zoom Image
Fig. 4 Postoperative encephalon T1-weighted MRI scan with contrast in the axial section, showing complete resection of the tumor lesion, with centralization of the brainstem and free fourth ventricle.
Zoom Image
Fig. 5 Postoperative encephalon T1-weighted MRI scan with contrast in the coronal section, showing complete resection of the tumor lesion, with centralization of the brainstem and free fourth ventricle.
Zoom Image
Fig. 6 Postoperative encephalon T1-weighted MRI scan with contrast, in the sagittal section, showing complete resection of the tumor lesion, with centralization of the brainstem and free fourth ventricle.

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.


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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.

Table 1

Case

Publication

Author

Year

Age

Gender

Resection

Histology

1

1

Sachs et al[1] [3] [8] [11] [18] [19] [20]

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


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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.


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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.



Address for correspondence

Rodrigo de Almeida Simon Sola, MSc
Rua Coronel Bento
394, Vilas Boas, Campo Grande, MS, 79051-110
Brazil   


  
Zoom Image
Fig. 1 Encephalon T1-weighted MRI scan with contrast showing tumor mass in the region of the fourth ventricle, with homogeneous enhancement and significant mass effect on the brainstem in the axial section.
Zoom Image
Fig. 2 Encephalon T1-weighted MRI scan with contrast showing tumor mass in the region of the fourth ventricle, with homogeneous enhancement and significant mass effect on the brainstem in the coronal section.
Zoom Image
Fig. 3 Encephalon T1-weighted MRI scan with contrast demonstrating tumor mass in the region of the fourth ventricle, with homogeneous enhancement and an important mass effect on the brainstem in the sagittal section.
Zoom Image
Fig. 4 Postoperative encephalon T1-weighted MRI scan with contrast in the axial section, showing complete resection of the tumor lesion, with centralization of the brainstem and free fourth ventricle.
Zoom Image
Fig. 5 Postoperative encephalon T1-weighted MRI scan with contrast in the coronal section, showing complete resection of the tumor lesion, with centralization of the brainstem and free fourth ventricle.
Zoom Image
Fig. 6 Postoperative encephalon T1-weighted MRI scan with contrast, in the sagittal section, showing complete resection of the tumor lesion, with centralization of the brainstem and free fourth ventricle.
Zoom Image
Fig. 1 RM de encéfalo ponderada em T1 com contraste demonstrando massa tumoral em região do quarto ventrículo, com realce homogêneo e importante efeito de massa sobre o tronco encefálico, no corte axial.
Zoom Image
Fig. 2 RM de encéfalo ponderada em T1 com contraste demonstrando massa tumoral em região do quarto ventrículo, com realce homogêneo e importante efeito de massa sobre o tronco encefálico, no corte coronal.
Zoom Image
Fig. 3 RM de encéfalo ponderada em T1 com contraste demonstrando massa tumoral em região do quarto ventrículo, com realce homogêneo e importante efeito de massa sobre o tronco encefálico, no corte sagital.
Zoom Image
Fig. 4 RM de encéfalo pós-operatória ponderada em T1 com contraste, no corte axial, evidenciando ressecção completa da lesão tumoral, com centralização do tronco encefálico e quarto ventrículo livre.
Zoom Image
Fig. 5 RM de encéfalo pós-operatória ponderada em T1 com contraste, no corte coronal, evidenciando ressecção completa da lesão tumoral, com centralização do tronco encefálico e quarto ventrículo livre.
Zoom Image
Fig. 6 RM de encéfalo pós-operatória ponderada em T1 com contraste, no corte sagital, evidenciando ressecção completa da lesão tumoral, com centralização do tronco encefálico e quarto ventrículo livre.