Intradiploic Meningioma With Inward and Outward Extensions in a Rheumatoid Arthritis Patient

Abstract

Background: Meningiomas usually arise from clusters of arachnoidal cap cells. Originating of a meningioma in an intraosseous region is rare condition. There are several theories about occurrence of these types of meningiomas. Here we report a case of an intradiploic meningioma in a rheumatoid arthritis patient and discuss the mechanism of developing intraosseous meningiomas, differential diagnosis of intraosseous lesions of calvarium, relations of rheumatoid arthritis and extensions of meningioma outside the diploe.Case description: A sixty three-year-old female patient who had been under the treatment for rheumatoid arthritis was admitted to the hospital with a swelling around her right eye. Bony window of preoperative CT showed an expansile right sphenoid bone lesion. The mass was excised with internal and external tables of the temporal bone by craniectomy.Conclusion: We advocate a wide resection, followed by cranioplasty with acrylic. If the subtotal resection is possible, residual lesion should be followed for adjuvant radiation therapy.

Full text

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Journal of Neurological Sciences [Turkish] 27:(1)# 22;079-083, 2010
http://www.jns.dergisi.org/text.php3?id=341

Case Report

Intradiploic Meningioma With Inward and Outward Extensions in a Rheumatoid
Arthritis Patient

Uygur ER1, Aslan GÜZEL2, Mehmet TATLI2, Adnan CEVİZ3, Aydın SAV4

1Ministry of Health, Diskapi Education and Research Hospital, 2nd Neurosurgery Clinic,
Ankara, Türkiye 2Dicle University, Neurosurgery Clinic, Diyarbakır, Türkiye 3Dicle
University, Pathology Clinic, Diyarbakır, Türkiye 4Marmara University, Pathology Clinic,
İstanbul, Türkiye

Abstract

Background: Meningiomas usually arise from clusters of arachnoidal cap cells. Originating
of a meningioma in an intraosseous region is rare condition. There are several theories about
occurrence of these types of meningiomas. Here we report a case of an intradiploic
meningioma in a rheumatoid arthritis patient and discuss the mechanism of developing
intraosseous meningiomas, differential diagnosis of intraosseous lesions of calvarium,
relations of rheumatoid arthritis and extensions of meningioma outside the diploe.
Case description: A sixty three-year-old female patient who had been under the treatment for
rheumatoid arthritis was admitted to the hospital with a swelling around her right eye. Bony
window of preoperative CT showed an expansile right sphenoid bone lesion. The mass was
excised with internal and external tables of the temporal bone by craniectomy.
Conclusion: We advocate a wide resection, followed by cranioplasty with acrylic. If the
subtotal resection is possible, residual lesion should be followed for adjuvant radiation
therapy.

Keywords: Ectopic meningioma, Intradiploic meningioma, Intraosseous meningioma,
Rheumatoid arthritis

Römatoid artrit hastasında içe ve dışa uzanımlı intradiploik meningioma

Özet

Amaç: Meningiomalar genellikle araknoid cap hücre kalıntılarından gelişirler. İntraosseöz
meningioma gelişimi nadirdir. Bu tip meningioma gelişimi için bir kaç teori mevcuttur. Bu
yazıda römatoid artritli bir hastada gelişen intradiploik meningiomayı sunarak, gelişim
mekanizmalarını, ayırıcı tanısını, römatoid artrit ile ilişkisini ve kalvaryum dışına uzanımını
tartışmayı amaçladık.
Olgu sunumu: Römatoid artrit nedeniyle tedavi altında olan altmış üç yaşındaki kadın hasta
sağ göz etrafında şişlik ile başvurdu. Bilgisayarlı tomografide ekspansil sağ sfenoid kemik
lezyonu izlendi. Kitle kraniyotomi ile çıkarıldı.
Sonuç: Bu tip olgularda geniş rezeksiyon ve akrilik kraniyoplasti önermekteyiz. Kısmi
çıkarım ancak olanaklı ise radyasyon tedavisi uygun olur.

Anahtar Kelimeler: Ektopik meningioma, İntradiploik meningioma, İntraosseöz
meningioma, Römatoid artrit

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INTRODUCTION
Meningiomas usually arise from clusters of
arachnoidal cap cells located in the
external layer of
membrane(13,15). Although bone invasion
and hyperostosis are frequently seen in
meningiomas, primary intraosseous ones
are rare, less than
meningiomas(3,6,13).
meningiomas constitute 14% of primary
extraneuraxial meningiomas(10). Clinical
and radiological
intraosseous meningiomas are different(9).
Here, we describe a case of an intradiploic
meningioma with extensions inwards and
outwards in a rheumatoid arthritis (RA)
patient and discuss
diagnosis, cellular origin, pathogenetic
mechanisms involved in its formation, and
possible associations with RA.

CASE PRESENTATION
A sixty three-year-old female patient who
had been under the treatment for RA was
admitted to the hospital with a swelling
around her right eye for three and a half
years. On physical examination, a palpable
mass lesion under the right temporalis
muscle was detected. Ulnar deviation of
her hands and
deformities were observed. A mild
papilledema was detected on neurological
examination.

Bony window of preoperative CT showed
an expansile right sphenoid bone lesion
(Fig. 1). On MRI 4x5 cm hyperostotic
mass lesion in right greater wing of the
sphenoid bone and right frontotemporal
region were seen (Fig. 2). The mass which
was compressing on the right optic canal
was enhanced intensively after iv contrast
administration.

The patient was operated on under general
anesthesia. After a right pterional skin
incision the tumor which was seen under
the right temporalis muscle was adherent
to it. The tumor was localized mainly in
the diploe of the temporal bone. The mass
the arachnoid
1% of all
Intraosseous
presentations of
its differential
multiple phalange
was excised with internal and external
tables of the temporal
craniectomy. The tumor was extending to
the underlying dura via eroding the internal
table. It was a highly vascular mass, mildly
soft in its center and peripherally harder.











































bone by
Fig 1: Bony window of preoperative axial CT
shows expansile right sphenoid bone lesion and
irregularity of inner and outer tables.
Fig 2: Preoperative axial T1-W MRI discloses
hypointense homogenous lesion compress to
right orbit and temporal
heterogeneously hyperintense
around the lesion.
region,
thickenings
and

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Histopathologically the tumor was a
meningothelial meningioma
infiltrated to the muscle.

The enhanced MRI of the patient showed
no recurrence or residual mass (Fig. 3) and
the patient was neurologically intact 11
months after the operation.

DISCUSSION
Meningiomas are classified into three main
groups; primary
extraneuraxial and
metastasize(10). Meningiomas originating in
an extraarachnoid region without contact
with the arachnoid layers may be
considered as ectopic,
intradiploic or epidural meningiomas(4,13).
Several theories have been proposed to
explain the mechanism of developing
ectopic meningiomas. Existence of the
arachnoidal cells at the foramina of cranial
nerves, presence of similar cells in the
arterial sheaths where vessels pierce the
skull or the periosteum could explain the
origin of the ectopic meningiomas found in
relation to cranial and spinal nerves(6,10,12).
Direct extensions or metastasis of an
intracranial meningioma is another theory.
Arachnoid cell capture as a result of
trauma which includes head molding
which
neuraxial,
secondary
primary
or
intraosseous,
during birth is also a possible mechanism
for further developing
meningioma(1,11). The sphenoid bone is one
of the most commonly affected sites of
intraosseous meningiomas because its
great number of articulations may be a nest
for captured arachnoid cells(2). Some
arachnoidal cell clusters found in autopsy
specimens of some patients without
meningioma at the level of internal
auditory meatus,
geniculate ganglion or the roof of the
Eustachian tube may be considered
reasonable for
meningiomas(6,13). Mesenchymal origin of
meninges may explain the development of
meningioma from
mesenchymal cell precursors in uncommon
locations(5,9,10,13,15).

On the other hand a relationship has been
demonstrated between
meningiomas and other neoplasms that
neuroectodermal origin
neurofibromatosis(11).

Although the
meningioma is defined as a lesion that does
not involve the dura, in the course of the
tumor growth, intradiploic meningiomas
may break through the bony borders,
invade the muscles and dura of either
side(6). It may be speculated that erosive
changes and decalcifications of various
bones in a RA patient is considered a
facilitative factor for tumors breaking
through the inner and outer tables. Both of
these would explain why the presented
tumor invaded the muscle and extending
but not involved the dura. Although it is a
well known fact that destructive type
meningiomas exhibits more aggressive
course(10), association of the RA in this
case may explain why this meningioma has
some destructive features but no aggressive
behavior. Bony destructions of this patient
may be supposed to be the result of RA.

It is known that osteolytic lesions of the
skull such as
histiocytosis X,
hemangiomas, myelomas and osteogenic
ectopic
jugular foramen,
ectopic temporal
multipotential
ectopic
such as
true intraosseous
malignant tumors,
epidermoids,
Fig 3: No residual or recurrent tumor is seen on
postoperative axial T1-W MRI after 11 months.

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sarcomas can facilitate the infiltration of
tumor cells into some bones(10,11). RA may
be one of the facilitative diseases for
developing meningioma
destruction mechanisms. To the best of our
knowledge, there is no epidemiologic
study in the literature on this topic. Since
hyperostosis is considered as the most
common radiographic
intraosseous meningioma and is found
60% of the series of Inagaki(8), coexistence
of this type of meningioma and RA may be
explained by frequent bony lesions in RA.
Because these bony lesions might be a well
ground for developing a neoplasia. At the
other hand, intraosseous meningioma is
developed frequently in patients with some
diseases such as Paget's disease, marble
bone disease, chronic inflammation of the
sinuses and mucormycosis(7). The common
feature of these diseases is that cause some
degree of bone destructions. Underlying
bony destructions can be considered as an
aiding factor for inoculation of tumor cells
into the diploe.

Coexistence of these two pathologies may
be found co-incidentally in our patient
even though after
explanations. There is not any strong
evidence for explaining this coexistence.
Aggressive behavior of our case may
justify that this condition is not a simple
association although
intraosseous meningiomas have clinically
benign course. Abnormal bones of the
skull in RA may assist spreading to the
nearby structures and developing the tumor
because of their weak barrier effect.

The differential diagnosis of intradiploic
meningioma includes osteoma, solitary
osteoblastic metastasis of osteoblastic
tumors, myeloma, eosinophilic granuloma,
fibrous dysplasia, myxoid tumors, Paget's
disease of bone and marble bone
disease(6,12,13,14). Although MRI is accepted
the best imaging method for detecting
meningiomas, CT may be better in
disclosing the bony
reactions(7,12).

by similar
finding in
all the above
most of the
borders and
Treatment of intradiploic meningiomas is
simply surgical tumor removal with
excision of both of the bony tables. We
advocate a wide resection, followed by
cranioplasty with acrylic. If the subtotal
resection is possible, residual lesion should
be followed for adjuvant radiation therapy.



Correspondence to:
Uygur Er
E-mail: uygurer@gmail.com



Received by: 18 March 2007
Revised by: 30 September 2007
Accepted: 08 December 2007



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