Clinical Neuropathology, Vol. 38 – No.
5/2019 (238-244)
Imaging and clinicopathologic features of
myxoid meningiomas
Mahlon D. Johnson1 and Ali Hussain2
1Departments of Pathology and Laboratory Medicine, Division of Neuropathology, and
©2019 Dustri-Verlag Dr. K. Feistle 2Imaging Sciences2, University of Rochester Medical Center, Rochester, NY, USA
ISSN 0722-5091
DOI 10.5414/NP301139
e-pub: July 26, 2019
Key words Abstract. Aims: Due to their rarity, the
myxoid meningioma – natural history and imaging of myxoid menin-
clinicopathologic giomas are not completely characterized. We
analyzed clinical, imaging, and pathologic
features of myxoid meningioma seen neuro-
surgically or in consultation between 1999 and
2018. Materials and methods: Archival mate-
rial was searched for meningiomas designated
“myxoid meningioma” at Vanderbilt Univer-
sity School of Medicine (1997 – 2004) and the
University of Rochester School of Medicine
and Dentistry (1994 – 2018). Results: Our
cases were predominantly in females and pre-
sented with a slow progression of symptoms.
Each tumor was in the hemispheres. Magnetic
resonance imaging (MRI) found most were
hyperintense on T2-weighted images. Each
meningioma had foci of limited meningo-
thelial amongst extensive myxoid histology
with Alcian-blue-staining stroma and EMA-
immunoreactive cells. Conclusion: Myxoid
meningiomas present with atypical imaging
and histologic characteristics but are not truly
metaplastic, i.e., are not differentiated to a dif-
ferent cell type.
Introduction
Myxoid meningiomas are an uncommon
metaplastic variant which, due to their rarity,
Received are incompletely characterized radiographi-
August 18, 2018; cally and biologically. The unusual magnetic
accepted in revised form resonance imaging (MRI) and histological
November 26, 2018
features are atypical for meningiomas and
Correspondence to may obscure the diagnosis intraoperatively.
Mahlon Johnson, MD, Studies on their long-term behavior and
PhD risk of recurrence are also limited. To our
Department of Pathol-
knowledge, only approx. 7 cases have been
ogy and Laboratory
Medicine, Univ. of described in case reports in English limiting
Rochester Medical our understanding of their clinical features,
Center, 601 Elmwood histological spectrum, and behavior [1, 2, 3,
Ave. Box 626, Rochester,
4, 5, 6, 7, 8]. In this report we describe clini-
NY 14623, USA
mahlon_johnson@ cal, radiological, pathological, and follow-up
urmc.rochester.edu findings of 5 cases.
Materials and methods
Pathology archives were searched for
myxoid meningiomas seen at Vanderbilt Uni-
versity School of Medicine (1997 – 2004)
and the University of Rochester School of
Medicine and Dentistry (2004 – 2018). Five
cases were identified. Hematoxylin and eo-
sin, Alcian blue-PAS stains epithelial mem-
brane antigen (EMA), S100 protein, Ki-67,
and, where available, vimentin, progester-
one receptor, AE1/AE3, CAM 5.2, smooth
muscle actin, muscle-specific actin, myo-
genin, CD34, glial fibrillary acidic protein,
and CD68 immunohistochemistry were
re-reviewed by one of us (MDJ) and classi-
fied using world health organization (WHO)
criteria [9]. Electron microscopic photomi-
crographs available in two cases were also
re-reviewed.
Results
Case 1
This 33-year-old female was evaluated
for right thigh pain extending down the me-
dial thigh to the calf. MRIs showed minimal
changes in the lumbosacral spine.
She was thought to have a radiculopa-
thy with compression. Non-contrasted head
computed tomography (CT) scan showed a
small dural-based isodense extra-axial mass
lesion at the left occipital region without
calcification, hemorrhage, or bony changes
(Figure 1a, b). Subsequent MRI head with
contrast re-demonstrated the dural-based
extra-axial space occupying lesion with viv-
id enhancement, measuring 7.4 × 12.4 mm
(Figure 1c). It appeared slightly hypointense
Myxoid meningiomas 239

Figure 1.  Case 1: CT head with contrast (a) shows small dural-based vividly enhancing extra-axial mass
lesion that is isodense in non-contrasted image (b). Similarly, vivid enhancement seen in T1-weighted MRI
after gadolinium administration (c) without appreciable dural tail. The mass is hyperintense in T2-weighted
image (d). Myxoid tumor (e) with spindle cell component (f) showing extensive EMA immunoreactivity (g).
Original magnifications × 200 (e, g) and × 400 (f).

on T1-weighted images and hyperintense on calcification or hemorrhage (Figure  2a,b).

T2-weighted images with increased diffusiv-
ity in the diffusion-weighted images (Fig-
ure 1d). No dural reaction was noted. Retro-
spectively, the lesion measured 3 × 3 mm in
MR study done 3 years earlier (Table 1).
Sections reveal monomorphic spindle
cells arranged in loose bundles in a highly
myxoid background (Figure 1e, f), with foci
of meningothelial cells. There was no mitotic
activity. Immunostaining for EMA is strong
and diffuse (Figure 1g). Ki-67 labeling index
is ~ 2%. She was diagnosed with a menin-
gioma, WHO grade I with extensive highly
myxoid background. Molecular analysis re-
vealed no mutations.
Seven years postoperatively she has had
no recurrence.
Case 2
This 64-year-old female who had cataract
surgery 2 months prior was evaluated by her
optometrist for blurred vision and found to
have bilateral papilledema. Non-contrasted
head CT scan showed hypodense extra-axial
space-occupying lesion along the left sphe-
noid bone with 1.3 cm midline shift but no
She was started on decadron.
There were significant adjacent hyperos-
totic bony changes (Figure 2c). The lesion
appeared hypointense on T1-weighted MRI
(Figure 2d) and hyperintense on T2-weight-
ed image, associated with vivid enhancement
and few internal signal voids and a dural tail
(Figure 2e, f). The mass appeared isointense
on diffusion weighted imaging (DWI) and
high on apparent diffusion coefficient (ADC)
maps (Figure 2g). Very minimal perilesion-
al edema was noted. The perfusion maps
showed elevated relative cerebral blood vol-
ume (rCBV) and relative cerebral blood vol-
ume (rcBF) (Figure 2h) (Table 1).
Her past medical history included deep
venous thrombosis. On exam, she had nor-
mal memory and cognition. The cranial
nerves were normal. Her strength was nor-
mal.
Intraoperatively the tumor was noted to
be soft and myxoid but favored being a me-
ningioma. The tumor was removed en toto,
measured 1.5 × 1 × 0.5 cm and was gross-
ly soft. Sections revealed a predominantly
myxoid tumor (Figure 2i) with meningothe-
lial foci (Figure  2j). There was no mitotic
activity or necrosis. The Alcian blue PAS
 Johnson and Hussain 240

stain showed extensive staining (Figure 2k).

Perfusion Dural tail

Tumor cells showed extensive EMA immu-

Yes
No

No
noreactivity (Figure 2l). The Ki-67 label-
ing was ~ 6%. A diagnosis of meningioma,
WHO grade I, myxoid type was made.

Low
NA

NA

NA
One year postoperatively there is no re-
currence.

T2 shine through

DWI and high on

Isointense on
Diffusion

ADC
NA

NA
Case 3

This 31-year-old, previously healthy

of the right lateral ventricle.

compression anterior horn
Minimal despite huge size

Mild edema and mild

woman experienced a new-onset generalized
Peritumoral edema

seizure during the 37th week of her pregnan-

None

cy. She also had a history of slowly increasing

weakness in her right leg over the past sever-
al months. Prenatal examinations identified
no neurological abnormalities. On exam, she
was somnolent but easily arousable and had
Heterogeneous

a hemiparesis with 3 to 4-/5 strength in the

Enhancement

enhancement
Vivid with few
Vivid homog-

signal voids

right lower extremity and 4/5 strength in the

Marked
enous

Yes

right upper extremity. A mild right-sided fa-

cial droop was also present. A Babinski sign
was present bilaterally. The ophthalmologi-
cal exam revealed bilateral papilledema. CT
weighted
Hyper

Hyper

Hyper
T2-

NA

NA

scans demonstrated a large left parasagittal

mass with midline shift. MRI of the brain re-
vealed an enhancing 7.8 × 4.6 cm left frontal
weighted

NA = not available; DWI = diffusion weighted imaging; ADC = apparent diffusion coefficient.
Slightly

Hypo

Hypo

lobe mass lesion adjacent to the falx cere-

hypo
T1-

NA

NA

bri effacing the left lateral ventricle with a

1.5 cm of midline shift (Figure 3a) (Table 1).
of high density likely calcifications.
High density with peripheral areas
Isodense. No calcification or bony

hemorrhage. There is significant

This case was reported previously [8].

Hypodense. No calcification or
CT/calcification/hemorrhage/

hyperstotic bony changes

Contrast enhancement

The patient had an emergency cesarean

section. 24 hours postoperatively she de-
bony changes

changes

veloped recurrent generalized seizures and

ultimately became comatose. This decline
in her neurologic exam was accompanied
by persistent hypertension and bradycardia.
Table 1.  Radiological features of myxoid meningiomas.

Repeat imaging revealed no evidence of

hemorrhage. With mannitol and high-dose
glucocorticoids she had a dramatic clini-
7.4 × 12.4 × 11 cm left

7.5 × 4.6 cm; midline

abutting the right falx

shift; compression of
1.3 cm midline shift,

1.9 × 2.0 × 1.5 cm

left lateral ventricle
3.7 × 3.5 × 3.7 cm

cal response, regaining wakefulness and the

size is 55 × 65 ×

ability to follow commands.

occipital

40 mm
Size

Six days post cesarian section, she had a

craniotomy. Intraoperatively, the tumor had
the gross appearance and consistency of a me-
ningioma. It was found to arise from the falx
L. parafalcine

R. sphenoid

cerebri. A gross-total resection was achieved.

L. sphenoid
L. occipital

R. frontal

The patient’s postoperative course was com-

plicated by hemiparesis and a transient severe
Site

depression. By 6 weeks postoperatively, the

Case

patient was doing extremely well and had

2
1

made a complete neurologic recovery.

Myxoid meningiomas 241

Figure 2.  Case 2: Non-contrasted head CT image at the level of the sphe-
noid wing (a) and more superior cut (b) showing very large, slightly hypodense
extra-axial mass lesion with significant hyperostotic changes, best depicted in
bone window algorithm (c). MRI evaluation of the mass reveals uniform hy-
pointensity in T1-weighted axial image (d), marked hyperintensity in T2-
weighted axial image (e); marked tumoral enhancement with few signal voids
are noted in sagittal post contrast image (f) better identifying the relation of
the tumor to the sphenoid ridge. High corresponding apparent diffusion coef-
ficient (ADC) values (g) and low relative cerebral blood volume in MR perfu-
sion imaging (h). Extensively myxoid (i) with focal meningothelial component
(j) showing extensive Alcian blue PAS staining (k) and EMA immunoreactivity
(brown) (l). Original magnifications × 100 (i, k) and × 200 (j, l).

Hematoxylin and eosin-stained sections 1 : 40. There was no AE1/AE3 (repeated),

revealed a myxoid, spindle cell tumor with
hypo- and hypercellular areas (Figure 3b, c).
There was no mitotic activity, necrosis, in-
flammation, or granuloma formation. One
focus with a vague meningothelial pattern
and a nuclear pseudoinclusion was found.
The tumor stained diffusely with Alcian
blue, pH 2.5 (Figure 3d) and PAS. No keloid-
like collagen or slit-like vascular pattern was
found, and CD34 immunohistochemistry
was negative. A reticulin stain demonstrated
scattered reticulin within the myxoid extra-
cellular matrix material. Tumor cells exhib-
ited no significant EMA immunostaining
at a dilution of 1 : 400 but did demonstrate
immunoreactivity at dilutions of 1 : 100 and
CAM 5.2, smooth muscle actin, muscle-spe-
cific actin, myogenin, CD34, glial fibrillary
acidic protein, or S-100 protein immunore-
activity. CD68 immunohistochemistry dem-
onstrated only rare monocytes. Electron mi-
croscopic analysis revealed external lamina
around some cells and showed evidence of
primitive cell junctions. She was diagnosed
with a myxoid meningioma, WHO grade I.
Long-term follow-up could not be obtained.
Case 4
This 74-year-old female was evaluated
for mental status changes with confusion and
Johnson and Hussain 242

Figure 3.  Case 3: Coronal T1-weighted MRI with gadolinium shows markedly enhancing left parafalcine
rounded extra-axial space occupying lesion, measuring 7.8 × 4.6 cm, with few non-enhancing internal foci
(a). Meningioma with myxoid features in majority of tumor (b) and spindle cells (c). Myxoid material stains
with Alcian blue PAS. (d) (Original magnifications × 100 (b) and × 400 (c, d). Extensively myxoid (a) with
focal spindle cell component (b) showing extensive EMA immunoreactivity (c). Original magnifications
× 200 (b) and × 400 (c and d).

slurred speech. Additional clinical findings Case 5

are not available.
MRI pre and post contrast revealed a
3.7 × 3.5 × 1.7 cm right frontal mass abut-
ting the falx. The mass is hypointense on
T1-weighted images (Figure 4a) and mark-
edly hyperintense on T2-weighted images
(Figure 4b). It shows vasogenic edema (Fig-
ure 4d) and vivid, though heterogeneous,
enhancement in the underlying brain com-
pressing the right lateral ventricle (Figure
4d) (Table 1). The sections reveal an exten-
sively myxoid tumor with no notable atypia
or mitotic activity (Figure 4e). There was no
apparent brain invasion. Myxoid spindle cell
areas (Figure 4f) and psammoma bodies were
found. Tumor cells exhibit extensive EMA
(Figure 4g), vimentin, and progesterone re-
ceptor immunoreactivity, rare AE1/AE3 but
no smooth muscle actin immunostaining. A
diagnosis of meningioma with myxoid fea-
tures, WHO grade I was made. Long-term
follow-up was not available.
This 36-year-old male was evaluated
for frontal headaches. MRI demonstrated a
skull-based tumor involving the inferior tur-
binate and anterior right sphenoid (Table 1).
This was resected. Two years later, when
frontal headaches reappeared, MRI revealed
an extra-axial recurrence with enhancement
post gadolinium (Table 1).
Pathological analysis revealed a myxoid
spindle cell mass (Figure 5a, b) that demon-
strated extensive EMA (Figure 5c) and focal
S-100 immunoreactivity. CD34 was patchy
and weak and primarily in blood vessels. The
case was sent to B. Scheithauer at Mayo Clin-
ic, and he also sent it to memorial Sloan Ket-
tering Cancer Center (Dr. Woodruff, Earland-
son, and Rosenbloom). Their final diagnoses
was “meningioma with myxoid and to a lesser
extent chondroid features”. Electron micros-
copy showed “some basement membrane
around some cells”. This has been described
in myxoid and chondroid meningiomas.
Long-term follow-up could not be obtained.
Myxoid meningiomas 243

Figure 4.  Case 4: MRI axial images illustrate a dural-based right parafalcine frontal extra-axial space-
occupying lesion that has homogenously hypointense signal in T1-weighted sequence (a), markedly in-
creased signal in T2-weighted image (b), with mild perilesional edema in T2 FLAIR (c). Vivid, though
inhomogeneous enhancement is noted after administration of gadolinium contrast agent (d). Myxoid me-
ningioma with focal spindle cell component (e), but predominantly myxoid (f). Tumor cells show extensive
EMA immunoreactivity (g) (Original magnifications × 100 (e) and × 200 (f, g).

Figure 5.  Case 5: Myxoid meningioma with spindle cells (a, b). Tumor cells show extensive EMA immu-
noreactivity (c). Original magnifications × 100 (a), × 200 (b), and × 400 (c).

Discussion toms. Nonetheless, the pathological feature

Overall, our cases (mean age 47) tended
to be younger and more likely female than
typical meningiomas that have a mean and
median age of 64 and 65, respectively and
a 2.2 : 1 female-to-male ratio as supraten-
torial tumors [9]. Our limited data suggest
they showed many features typical of WHO
grade I meningiomas, such as occurrence
in the hemispheres, presentation as a dural-
based mass, and slow progression of symp-
with widespread myxoid histology is a rare
finding.
MRI findings, in contrast with classic tu-
mors, were also different. Our myxoid me-
ningiomas were hyperintense on T2-weight-
ed images. This has also been found in the
case of Krishit et al. [4]. In contrast, other
metaplastic variants such as lipomatous me-
ningiomas show bright hyperintensity on T1,
mixed intensity onT2-weighted images, and
inhomogeneous contrast enhancement [10].
Johnson and Hussain 244
Moreover chordoid meningiomas show sig-
nificant elevations in ADC compared to oth-
er WHO grade I meningiomas [11].
Since 1993, the WHO has recognized a
subgroup of “metaplastic” meningiomas in-
cluding the myxoid variant [9]. Nonetheless,
our cases showed limited meningioma cytol-
ogy with extensive Alcian blue staining stro-
ma in EMA immunoreactive meningiomas.
This suggests that myxoid meningiomas are
not truly metaplastic, i.e., are not differenti-
ated to a different cell type. Thus, they do not
qualify as having undergone true metaplastic
change.
The diagnosis of myxoid meningioma
is contingent on: 1) documenting derivation
from the leptomeninges, 2) identification of
focal meningioma histology, 3) extensive
myxoid stroma with Alcian blue staining,
and 4) exclusion of other myxoid tumors [1,
2, 3, 4]. Identification of tight junctions and
basal lamina is also helpful. A critical finding
is the paucity of EMA immunoreactivity at
recommended dilutions for EMA [2, 3]. In
addition, immunostaining for other cytoker-
atins, smooth muscle actin, muscle-specific
actin, and S-100 protein are all generally
negative.
The myxoid variant of meningioma must
be differentiated from other dural-based
lesions that radiographically mimic me-
ningiomas and other myxoid spindle-cell
neoplasms. These include schwannomas,
myxomas, fibromyxomas, or fibroxanthomas
of the dura. Spindle-cell and myxoid histo-
logic patterns, lack of EMA immunoreactiv-
ity, variable S-100 immunoreactivity, and,
when available, the finding of basal lamina
by electron microscopy, are more consistent
with a diagnosis of schwannoma [12]. A di-
agnosis of dural myxomas must be consid-
ered but some tumors, previously character-
ized as such, may be myxoid meningiomas
[12]. Solitary fibrous tumors (SFT) of the
falx or cerebellopontine angle, which more
commonly occur in women in the same age
group as meningiomas, usually have CD34
immunoreactivity. Similarly, myxoid lipo-
sarcomas may rarely metastasize to the dura
but display notably different histologic fea-
tures [13].
Our experience suggests that these tu-
mors grow slowly with a low recurrence rate.
Funding
None.
Conflict of interest
None.
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