J Neurosurg Pediatrics 14:155159, 2014

AANS, 2014

Meningeal fibroma: a rare meningioma mimic

Report of 2 cases

Aanchal Kakkar, M.D.,1 Mehar C. Sharma, M.D.,1 Nishant Goyal, M.B.B.S., 2

Chitra Sarkar, M.D.,1 Vaishali Suri, M.D.,1 Ajay Garg, M.D., 3
Shashank S. Kale, M.Ch., 2 and Ashish Suri, M.Ch. 2
Departments of 1Pathology, 2Neurosurgery, and 3Neuroradiology, All India Institute of Medical Sciences, New
Delhi, India

Meningeal fibromas are rare intracranial tumors that mimic meningiomas radiologically as well as histologi-
cally. The authors report 2 cases of meningeal fibroma with detailed clinical, radiological, histopathological, and
immunohistochemical features, and discuss the differential diagnosis of this entity. Knowledge of this rare tumor
is essential for pathologists to be able distinguish it from more common meningeal tumors, especially in younger
patients. This knowledge is also essential for neurosurgeons, as incomplete resection may lead to tumor recurrence,
and such patients require close follow-up.
(http://thejns.org/doi/abs/10.3171/2014.5.PEDS13556)

Key Words fibroma meningeal tumor meningioma oncology

solitary fibrous tumor intracranial mesenchymal

F
ibrous proliferative lesions arising from meningeal
mesenchymal tissue are extremely rare and include
fibromatosis, solitary fibrous tumor (SFT), inflam-
matory myofibroblastic tumor, fibroma, and cranial fasci-
itis.1 The histological appearance of these lesions is simi-
lar to more common tumors such as meningiomas, and
therefore they pose a challenge to the diagnostic skills of
the pathologist. We report 2 cases of meningeal fibroma,
one in a 15-year-old boy and another in an 8-year-old boy,
both of which mimicked meningioma on radiological as
well as on histological examination, causing a diagnostic
dilemma.
Case Reports
Case 1. A 15-year-old boy complained of intermit-
Abbreviations used in this paper: EMA = epithelial membrane
antigen; GFAP = glial fibrillary acidic protein; SFT = solitary
fibrous tumor; SMA = smooth muscle actin.
tent headache and vomiting for 3 months. His parents had
noted changes in his personality and behavior over this
period. On examination, there was no evidence of any
sensorimotor deficits or any stigmata of neurofibromato-
sis. Magnetic resonance imaging revealed a large, hetero-
geneously enhancing mass lesion in the frontotemporo-
parietal region, suggestive of a meningioma (Fig. 1). The
patient underwent a right frontoparietal craniotomy and
gross-total excision of the tumor. Intraoperatively, a gray-
ish-white, hard, extraaxial mass with minimal vascularity
was noted, attached to the falx. The tumor was excised
completely, along with its falcine attachment (Simpson
Grade I excision) and was submitted for histopathological
evaluation. The patient remains recurrence-free 3 years
after the surgery.
Case 2. An 8-year-old boy presented with a history
of intermittent headache and abnormal behavior, includ-
This article contains some figures that are displayed in color
online but in black-and-white in the print edition.

J Neurosurg: Pediatrics / Volume 14 / August 2014 155


Fig. 1.Case 1. Axial contrast-enhanced (left) and bone window
(right) CT scans show a well-defined, homogeneously hyperdense
extraaxial lesion in the left temporoparietal region with perilesional ede-
ma, overlying periosteal reaction, and new bone formation (arrow, right)
from the inner table of the skull. In the anterior portion of the lesion,
hypodensity (asterisk, left) secondary to CSF loculation/cyst is noted.
ing hyperactivity and nonsensical speech, for 1 year. He
had undergone operations twice at another hospital, after
which he had remained symptomatic postoperatively. On
both the previous occasions, a histopathological diagnosis
of WHO Grade I fibroblastic meningioma had been made.
Preoperative MRI of his head showed a well-defined fal-
cine region mass that was hypointense on T2-weighted
images and showed peripheral dense enhancement on
T1-weighted images with contrast administration. There
was no evidence of calcification. Repeat imaging revealed
a residual tumor (Fig. 2). The tumor was approached
through a bifrontal craniotomy. Intraoperatively, a well-
demarcated, firm, lobulated, poorly vascularized tumor
was noted attached to the middle third of the falx cere-
bri, which was reaching up to the base of the falx, more
on the right side. Simpson Grade II tumor excision was
achieved, and the operative specimen was submitted for
histopathological examination. Sections from the prima-
ry tumor stained with H & E were also reviewed. The pa-
tient was followed up for a period of 1 year, during which
time no further recurrence or metastasis occurred.
Pathological Examination. Tumor tissue was fixed
in 10% neutral-buffered formalin, routinely processed,
and paraffin embedded. Five-micron-thick sections were
cut for routine H & E staining and immunohistochemi-
cal analysis. A labeled streptavidin biotin kit (Universal,
Dako) was used as a detection system. Antigen retrieval
was performed in a microwave oven using citrate buf-
fer at pH 6.0 for all antibodies. Monoclonal antibodies
against vimentin (Diagnostic BioSystems, 1:100), epithe-
lial membrane antigen (EMA; Dako, 1:100), pan cytoker-
atin (Neomarkers, 1:200), claudin-1 (Neomarkers, 1:50),
smooth muscle actin (SMA; Dako, 1:50), progesterone
receptors (Neomarkers, 1:50), estrogen receptors (Neo-
markers, 1:50), CD34 (Dako, 1:100), bcl-2 (Neomark-
ers, 1:100), CD99 (Dako, 1:100), desmin (Dako, 1:50),
glial fibrillary acidic protein (GFAP; Dako, 1:1500), beta
catenin (BD Transduction, 1:200), p53 protein (Santa
Cruz Biotechnology, 1:1000), and proliferation marker
MIB-1 (Dako, 1:200) were used. For assessing the MIB-
156
A. Kakkar et al.
Fig. 2. Case 2. An axial T2-weighted MR image shows a well-de-
fined hypointense mass in the region of the floor of the anterior cranial
fossa (A). No foci of calcifications are observed on a T2-weighted gra-
dient echo image (B). On contrast administration, the peripheral por-
tion of the tumor shows dense contrast enhancement on a T1-weighted
image (C). A postoperative T1-weighted image after Gd administration
shows residual tumor (D).
1 labeling index, at least 500 cells were counted using
a 1-square-mm eyepiece pinhole. For each batch, appro-
priate positive and negative controls were taken. Electron
microscopic examination was performed on paraffin-em-
bedded tumor tissue, after deparaffinization, rehydration,
and postfixation in osmium tetroxide.
Microscopic Examination. Sections stained with H
& E from tumors from both patients showed similar fea-
tures (Fig. 3). The tumors were sparsely cellular, com-
posed of elongated spindled-shaped cells with a scant to
moderate amount of pale eosinophilic cytoplasm, and
normochromatic oval to elongated nuclei with delicate
chromatin and inconspicuous nucleoli. The tumor cells
were arranged in long parallel fascicles that were embed-
ded in a densely collagenized matrix. Areas of hyaliniza-
tion were present and were more prominent around blood
vessels. Sections from both cases showed focal myxoid
change in the stroma. The tumor cells did not demon-
strate nuclear atypia, pleomorphism, mitoses, or areas of
necrosis. There was no evidence of meningothelial dif-
ferentiation in the form of whorl formation or sheeting of
the tumor cells. Mast cells were observed interspersed be-
tween the tumor cells. Review of sections prepared from
tumors excised at the first and second surgeries in Case 2
showed similar histomorphological features.
The tumor cells were immunopositive for vimentin;
J Neurosurg: Pediatrics / Volume 14 / August 2014
Meningeal fibroma

Fig. 3. Photomicrographs showing tumor cells in long intersecting fascicles (A and B) with areas of hyalinization (C), focal
myxoid change (D), and thick bundles of collagen in the stroma (E). High magnification shows bland spindle-shaped cells with
elongated nuclei (F), perivascular hyalinization (G), and interspersed mast cells (H). H & E, original magnification 40 (A), 100
(B and C), 200 (D and E), and 400 (FH).

however, they were immunonegative for EMA, S100 pro-
tein, claudin-1, estrogen receptors, progesterone recep-
tors, CD34, CD99, bcl-2, beta catenin, desmin, SMA,
pan cytokeratin, and GFAP (Fig. 4). The MIB-1 labeling
index was less than 1% in both tumors, and no immu-
nopositivity for p53 protein was noted. Ultrastructural
examination (Fig. 5) of paraffin-embedded tumor tissue
from both cases showed the presence of artifacts; how-
ever, the features were sufficient to recognize the tumor
cells as fibroblasts. Spindle-shaped tumor cells with

J Neurosurg: Pediatrics / Volume 14 / August 2014 157


Fig. 4. Tumor cells in both cases were found to be immunoposi-
tive for vimentin (left), and the MIB-1 labeling index was less than 1%
(right). Original magnification 400 (left) and 200 (right).
elongated nuclei, some of which appeared indented, and
abundant dilated rough endoplasmic reticulum were iden-
tified. Some of the cells showed the presence of elongated
processes. Bundles of collagen fibers were present in the
extracellular matrix. Intercellular junctions and interdigi-
tations were not observed, excluding the possibility of a
meningothelial origin.
Discussion
Intracranial fibromas are rare, benign, fibrous lesions
that arise from the meninges or from within the brain pa-
renchyma.1,4,5 Less than 20 such cases have been described
to date, and their incidence remains unknown.1,2,4,5,7,9,1113
At our institute, they accounted for 0.12% (2/1648) of all
meningeal tumors excised over a 10-year period. Intra-
cranial fibroma was first described in the English litera-
ture by Koos et al. in 1971 in an 11-month-old boy.4 These
fibromas are frequently noted in the first 2 decades of life,
and show a male preponderance.12 These tumors are usu-
ally bulky, well-circumscribed masses that, on histologi-
cal examination, are found to be composed of elongated
spindle cells in a dense, eosinophilic, hyalinized collag-
enous matrix.1 Due to the rarity of these tumors, no defi-
nite management protocol is defined for meningeal fibro-
mas. Because these tumors are benign lesions, complete
resection is curative. However, incomplete resection can
lead to recurrence or regrowth of the tumor, as noted in
one of our cases.
The differential diagnoses of meningeal fibroma in-
clude meningioma, SFT, fibromatosis, cranial fasciitis,
meningeal myxoma, and meningeal glioma (Table 1).
While psammomatous calcification and osseous and chon-
Fig. 5. Electron photomicrographs showing a tumor cell with elongated nucleus and dilated endoplasmic reticulum (asterisk,
A). Higher magnification shows an indentation in the nucleus and margination of chromatin (B). Bundles of collagen fibers are
noted around the tumor cell (C).
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A. Kakkar et al.
droid metaplasia may be encountered in these tumors,1
the absence of whorls and meningothelial features helps
to distinguish them from fibroblastic meningiomas.12 Im-
munoreactivity with EMA, claudin-1, S100 protein, and
progesterone receptors in meningiomas further aids in this
distinction.12 Another close differential diagnosis is SFT.
These tumors also arise from the meninges, but are usually
observed in adults.1 Solitary fibrous tumors are more cellu-
lar than fibromas, and are composed of interlacing fascicles
of spindle cells rather than long parallel fascicles.1 They are
immunopositive for CD34, CD99 and bcl-2, while fibro-
mas are negative for these markers.12 Meningeal gliomas
are extremely rare; the absence of collagen accompanied
by immunopositivity for GFAP helps to differentiate be-
tween the two tumors.12 Fibromatosis is a histologically
benign but locally invasive condition involving the dura,
but it has infiltrative margins, whereas fibromas are well
circumscribed.10 Fibromatosis may occur de novo or at the
site of previous surgery or trauma.6 Intracranial myxomas
are rare lesions that arise from the skull bones and may
show attachment to the dura. Radiologically, they are usu-
ally limited to the inner and outer tables of the skull, while
histologically, they are typified by a loose myxoid matrix
containing spindled- to stellate-shaped cells with minimal
pleomorphism.8 Cranial fasciitis is a reactive proliferation
of fibroblastic and myofibroblastic cells in a variably hya-
linized and myxoid stroma, which involves the soft tissue
of the scalp and adjacent cranium, and may extend to in-
volve the dura. Its predilection for the first 6 years of life,
presence of a lytic skull lesion, prominent myxoid matrix,
and inflammatory cell infiltrate on histology help to distin-
guish it from meningeal fibroma.3
Nonmeningothelial tumors of the meninges, includ-
ing fibromas, are much less common than meningiomas.
Knowledge of these entities is essential for pathologists
to include them in the differential diagnosis of menin-
giomas, especially in younger patients; this knowledge is
also essential for neurosurgeons because incomplete re-
section may lead to tumor recurrence, and these patients
need to be closely followed up.
Disclosure
The authors report no conflict of interest concerning the mate-
rials or methods used in this study or the findings specified in this
paper.
J Neurosurg: Pediatrics / Volume 14 / August 2014
Meningeal fibroma
TABLE 1: Immunohistochemical panel for differential diagnosis of fibroma from other meningeal tumors*
Tumor Vimentin EMA S100 SMA
fibroma + focal +/
meningioma + + +/-
SFT + focal +/
glioma + +
* PR = progesterone receptors; + = positive; = negative.
Author contributions to the study and manuscript preparation
include the following. Conception and design: Sharma, Kakkar.
Acquisition of data: Kakkar, Goyal, Kale, A Suri. Analysis and inter-
pretation of data: Sharma, Kakkar, Sarkar, V Suri, Garg. Drafting
the article: Kakkar, Goyal. Critically revising the article: Sharma.
Reviewed submitted version of manuscript: all authors. Approved
the final version of the manuscript on behalf of all authors: Sharma.
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Manuscript submitted October 17, 2013.
Accepted May 7, 2014.
Please include this information when citing this paper: pub-
lished online June 20, 2014; DOI: 10.3171/2014.5.PEDS13556.
Address correspondence to: Mehar Chand Sharma, M.D.,
F.R.C.Path., Department of Neuropathology, All India Institute of
Medical Sciences, New Delhi 110029, India. email: sharmamehar@
yahoo.co.in.
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