Keywords
meningioma - fourth ventricle - infratentorial neoplasms
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.
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.
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.
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.
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.
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.
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.
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
|
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.
