CASE REPORT

Primary intracranial leiomyoma: a case report

and literature review
Abdullah E. Ali & Mahmood Fazl & Juan M. Bilbao
Received: 19 April 2006 / Accepted: 26 May 2006 / Published online: 8 August 2006
# Springer-Verlag 2006
Abstract We report a case of primary intracranial leio-
myoma in 29-year-old woman presented with severe
headache. The radiology diagnosis was consistent with
meningioma. However, histologically, the tumor had the
characteristic appearance of benign smooth muscle. This
was confirmed by immunohistochemistry and electron
microscopy. Benign metastasizing leiomyoma was exclud-
ed by thorough imaging. Although rare, leiomyoma should
be considered in the differential diagnosis of well-circum-
scribed intracranial lesion.
Keywords Intracranial
.
Benign
.
Leiomyoma
Abbreviations
AIDS Acquired immunodeficiency syndrome
CNS Central nervous system
CT Computerized tomography
EBV Epstein Barr virus
MRI Magnetic resonance imaging
Introduction
The majority of intracranial extraaxial neoplasms are
meningiomas. Primary intracranial mesenchymal neo-
plasms are extremely rare but when they occur, they should
be classified similarly to their systemic counterparts.
Leiomyomas are benign smooth muscle tumors common
to the uterus. Outside the uterus, leiomyomas have been
reported in almost every organ in the body [9]. In the
central nervous system, they can radiologically mimic
meningiomas [7].
Clinical history
A 29-year-old woman, previously healthy, presented com-
plaining of episodic clusters of severe hemicranial head-
aches for few years. The headache lasted for 10 min and
occurred 1020 times per day. She had no visual symptoms.
On examination, there were no focal findings. Magnetic
resonance image (MRI) of the brain showed a 1.2-cm
extraaxial lesion in the right frontal fossa floor, medial to
the sphenoid wing (Fig. 1). Although the lesion was small,
surgical resection was done because of the close proximity
to the optic nerve. The patient underwent frontal craniot-
omy. The tumor was attached to the dura. It was carefully
resected and sent to the anatomical pathology for
processing.
Materials and methods
The specimen was fixed in 10% formalin, embedded in
paraffin wax, and cut at 58 m. Immunohistochemical
staining was performed using the avidinbiotin peroxidase
method. The antibodies used in this study were directed
against: smooth muscle actin (1/500, monoclonal, Biogenex),
muscle-specific actin (1/25, monoclonal, Enzo), desmin
(1/100, monoclonal, Biogenex), caldesmon (1/200, mono-
clonal, Dako), collagen IV (1/50, monoclonal, Dako),
Virchows Arch (2006) 449:382384
DOI 10.1007/s00428-006-0252-z
A. E. Ali
:
J. M. Bilbao (*)
Department of Pathology, Sunnybrook Health Science Centre,
2075 Bayview Avenue, Room E-419,
Toronto, ON M4N 3M5, Canada
e-mail: Juan.Bilbao@sunnybrook.ca
M. Fazl
Department of Neurosurgery, Sunnybrook Health Science Centre,
Toronto, ON M4N 3M5, Canada
pancytokeratin (AE1/AE3, 1/300, monoclonal, Dako), epi-
thelial membrane antigen (1/1,200, monoclonal, Dako),
S100 protein (1/1,500, polyclonal, Dako), CD117 (1/400,
polyclonal, Dako), estrogen receptor (1/100, monoclonal,
Novocastra), progesterone receptors (1/100, monoclonal,
Novocastra), CD34 (1/50, monoclonal, Dako), CD30
(1/200, monoclonal, Dako), and Epstein Barr virus (EBV)
(1/100, monoclonal, Dako). The tissue for electron micros-
copy was obtained from the paraffin-embedded material,
routinely processed, stained with uranyl acetate, and exam-
ined in a ZEISS EM 109 electron microscopy.
Results
The tumor was well circumscribed. Microscopically, the
tumor consisted of a bland spindle cell neoplasm composed
of intersecting fascicles of cells with abundant eosinophilic
cytoplasm, elongated cigar-shaped nuclei without promi-
nent nucleoli (Fig. 2). Mitotic activity was very low, and
there was no necrosis. The tumor was immunoreactive to
smooth muscle actin, muscle-specific actin, desmin, cal-
desmon (Fig. 3), and collagen IV, and negative for keratin,
epithelial membrane antigen, S100 protein, CD117, estro-
gen and progesterone receptors, CD34, and CD30. The Ki-
67 stained <1% of tumor cells. Immunohistochemical stain
for EBV was nonreactive. Electron microscopy showed
tumor cells to possess abundant mitochondria and
pinocytotic vesicles. The cytoplasm was filled with
intermediate filaments (Fig. 4). The findings are charac-
Fig. 2 Histology showing bland spindle cell proliferation in fascicles
with abundant eosinophilic cytoplasm. No atypia, increased mitosis,
or necrosis is seen (H&E, 20)
Fig. 3 Immunohistochemistry showing positive caldesmon staining
for smooth muscle cells (20)
Fig. 4 Electron microscopy showing abundant pinocytotic vesicles as
well as cytoplasmic intermediate filaments (12,000)
Fig. 1 MRI scan of the brain showing an extraaxial lesion at the base
of the frontal fossa floor
Virchows Arch (2006) 449:382384 383
teristic of smooth muscle differentiation, and a diagnosis of
leiomyoma was made. The differential diagnosis of benign
metastasizing leiomyoma was entertained but the patient
had negative thoracic and abdominal computerized tomog-
raphy scan as well as gynecological sonography. The final
diagnosis was primary central nervous system (CNS)
leiomyoma.
Discussion
Primary intracranial mesenchymal tumors are uncommon.
Primary CNS smooth muscle tumor is exceedingly rare.
Rhabdomyosarcoma accounts for most primary myogenic
neoplasms of the CNS reported to date [9]. There is a high
incidence of primary CNS smooth muscle neoplasms in
immunocompromised individuals. An association with
EBV had been demonstrated [1, 9]. The first case of
primary intracranial leiomyoma was reported by Kroe et al.
in 1968 [6]. The majority of the reported cases were in the
sellar region [8]. It has been mistaken as meningioma
radiologically [7]. The differential diagnosis of an extra-
axial intracranial lesion would include meningioma,
schwannoma, hemangiopericytoma, and solitary fibrous
tumor. The histology, immunohistochemical profile, and
the electron microscopical findings confirmed the smooth
muscle origin of this tumor. Another rare differential
diagnosis that should not be forgotten, giving the female
gender of the patient, is benign metastasizing leiomyoma
[3]. This was excluded by thorough imaging.
Several hypotheses were suggested for the origin of this
tumor in the CNS. It is presumed that smooth muscle
tumors in the CNS may arise from embryonic rests,
pluripotential mesenchymal cells, perivascular connective
tissue, leptomeninges, or smooth muscle cells of the blood
vessels [6, 10]. A separate study suggested the latter origin
because of the finding of actin inclusions in the proliferat-
ing smooth muscle cells of the adjacent blood vessels
similar to those found in the tumor cells [8].
Leiomyomas were recently added to the list of neoplasms
showing increasing incidence in acquired immunodeficiency
syndrome (AIDS) especially in children [1, 4]. There is an
association between EBV infection and the development of
leiomyoma in those individuals. One report documents a
benign leiomyoma but with EBV positivity in a young
female with AIDS. Her tumor behaved aggressively, and she
died of extensive intracranial disease [2].
To our knowledge this is the seventh case of primary
intracranial leiomyoma [2, 5, 6, 8, 10]. Table 1 summarizes
the clinicopathological data of the reported cases of primary
intracranial leiomyoma. Although rare, leiomyoma should be
considered in the differential diagnosis of a well-circum-
scribed intracranial extraaxial lesion especially at the sellar
or suprasellar regions.
References
1. Bargiela A, Rey JL, Diaz JL (1999) Meningeal leiomyoma in an
adult with AIDS: CT and MRI with pathological correlation.
Neuroradiology 41(9):696698
2. Bette K, Kleinschmidt-DeMasters BK, Mierau GW (1998)
Unusual dural and skull-based mesenchymal neoplasms: a report
of four cases. Hum Pathol 29(3):240245
3. Goyle KK, Moore DF Jr, Garrett C (2003) Benign metastasizing
leiomyomatosis: case report and review. Am J Clin Oncol 26
(5):473476
4. Karpinski NC, Yaghmai R, Barba D (1999) Case of the month:
March 1999a 26 year old HIV positive male with dura based
masses. Brain Pathol 9(3):609610
5. Kim SH, Youm JY, Song SH, Kim Y, Song KS (1999) Primary
intracranial leiomyoma. Case illustration. J Neurosurg 90(1):171
6. Kroe DJ, Hudgins WR, Simmons JC (1968) Primary intrasellar
leiomyoma. J Neurosurg 29(2):189191
7. Lai PH, Yang CF, Huang CH (1998) Primary intracranial
leiomyoma. Neuroradiology 40(4):238241
8. Lin SL, Wang JS, Huang CS (1996) Primary intracerebral
leiomyoma: a case with eosinophilic inclusions of actin filaments.
Histopathology 28(4):365399
9. Rosai J (2004) Rosai and Ackermans surgical pathology, 9th edn.
Mosby, St. Louis, MO
10. Thierauf P, Weiland H (1978) Intracranial leiomyoma. Med Welt
29(33):12121280
Table 1 Reported cases of primary intracranial leiomyoma
Author Age Sex Site Size
(cm)
Presentation EBV
status
HIV
status
1 Kroe et al. [6] 68 F Intrasellar 2.53.0 Visual defect NA NA
2 Thierauf and Weiland [10] Child F Suprasellar 45 Visual defect NA NA
3 Lin et al. [8] 20 F Left superior temporal and
insular gyri
321 Limb numbness
and pain
NA NA
4 Bette et al. [2] 56 F Intra- and suprasellar 3.2 Visual defect NA NA
5 Bette et al. [2] 34 F Cavernous sinus 1.20.8 Right-sided sixth nerve
palsy
Positive Positive
6 Kim et al. [5] 40 F Temporoparietal convexity 74.54 Headache NA NA
7 Present case 29 F Right frontal lobe floor 1.2 Headache Negative NA
NA Not available
384 Virchows Arch (2006) 449:382384