Leiomyoma is a benign tumor that originates from smooth
muscle cell. The most common sites are the uterus, gastrointestinal tract & skin. Leiomyoma is a relatively uncommon smooth muscle tumor rarely found in the head and neck. Enzinger and Weiss (1995), analyzed a total of 7748 leiomyomas, 95% of the tumors occurred in the female genitalia (uterus), 3% in the skin, 0.9% in the gastrointestinal tract and the remainder at various sites including skull base.
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Case Report
Leiomyoma of skull base
Rajendra Prasad a,*, Amit Kapoor a, Shyam Sunder b
a
b
Department of Neurosurgery, Apollo Hospital, New Delhi, India
Department of Neurosurgery, Udayan Hospital, Patna (Previously at Apollo Hospital, New Delhi), India
article info
abstract
Article history:
Leiomyoma of the skull base is an extremely rare tumor and is not well documented. In
Received 31 July 2013
this article, we report a case of a leiomyoma of the skull base in a 17 years old African male
Accepted 7 August 2013
whose initial diagnosis was a sphenoidal wing meningioma.
Available online 6 September 2013
Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved.
Keywords:
Benign mesenchymal tumor of skull
base
Leiomyoma of skull base
Rare intracranial tumors
1.
Introduction
Leiomyoma is a benign tumor that originates from smooth
muscle cell. The most common sites are the uterus, gastrointestinal tract & skin. Leiomyoma is a relatively uncommon
smooth muscle tumor rarely found in the head and neck.3
Enzinger and Weiss (1995), analyzed a total of 7748 leiomyomas, 95% of the tumors occurred in the female genitalia
(uterus), 3% in the skin, 0.9% in the gastrointestinal tract and
the remainder at various sites including skull base.
We review the literature of mesenchymal tumors of the
skull base. We report an extremely rare case of a leiomyoma of
the skull base.
2.
Case report
A 17-year-old African male presented to us with a history of
protrusion of the left eye ball of one year duration, along
with progressive loss of vision in the left eye of three
months duration. On examination he had axial proptosis of
the left eye. There was no perception of light in the left eye.
There was no motor or sensory deficit in the limbs. He was
referred for surgery with a diagnosis of sphenoidal wing
meningioma. He underwent staged excision of the lesion.
Since the lesion appeared vascular on MRI he underwent
tumor embolization of the feeders from both the internal
carotid artery (ICA) and external carotid artery (ECA). Fortyeight hours later he underwent left frontotemporal craniotomy and microscopic decompression of the tumor. As the
medial portion of tumor was attached to the cavernous
sinus and was very vascular it was decided to stage the
tumor resection. One week after the first surgery there was
no improvement in vision in the left eye and visual evoked
potential did not elicit any response. He then underwent left
ophthalmic artery embolization followed by left lateral rhinotomy through a transfacial approach and decompression
of the residual lesion. Since the tumor was benign in nature,
* Corresponding author. Tel.: þ91 09810048369.
E-mail address: neurosurgeryindia@gmail.com (R. Prasad).
0976-0016/$ e see front matter Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.apme.2013.08.008
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241
Fig. 1 e Pre operative MRI (T1 contrast).
it was decided to follow-up the small residual portion of the
tumor involving the cavernous sinus and not to give stereotactic radiation till there was evidence of tumor growth.
Pre operative MRI (T1 contrast) Brain (Plain and Contrast)
showed a well defined extra axial intensely enhancing mass
lesion in left parasellar region extending into the left orbit, left
infratemporal fossa, left ethmoid, sphenoid and maxillary
sinuses with erosion of adjacent bones [Fig. 1].
Post operative MRI showed: Residual mass in left parasellar
region with intraorbital extension through left optic nerve
canal and displacing left optic nerve [Fig. 2].
Post-operative (T1 contrast) showing a very small residual
tumor [Fig. 3].
Histopathology showed a cellular neoplastic lesion,
composed of neoplastic cells arranged in the form of sheets,
whorls and fascicles. The cells had indistinct cells borders with
oval to spindle shaped nuclei. Foci of dystrophic calcification
were seen. No mitotic activity was evident. It was reported to
be a leiomyoma, a benign mesenchymal neoplasm [Fig. 4].
At 3 years follow-up the patient is neurologically preserved except for visual loss in the left eye and no proptosis. There has been no increase in the size of the residual
lesion.
Enzinger and Weiss (1995), analyzed a total of 7748 leiomyomas, 95% of the tumors occurred in the female genitalia
(uterus), 3% in the skin, 0.9% in the gastrointestinal tract and
the remainder in various sites including the skull base.1,2
Tumors of mesenchymal origin that affect the skull base
are rare and in a series of 322 lateral skull base procedures,
ten (0.3%) were performed for sarcomas, or their histologically benign equivalent.3 These tumors were chondrosarcoma,
chondroid
chordoma,
leiomyosarcoma,
synovial sarcoma, myxoma and fibrous dysplasia. In a case
report of four cases of unusual dural and skull-based
mesenchymal neoplasms, there was case of leiomyoma of
the suprasellar region in a 57-year-old woman and a patient
of EBV-associated leiomyosarcoma of the left dural transverse sinus.4
Leiomyomas of skull base are rare tumors. As these tumors
can be vascular preoperative embolization is of immense help
in surgical decompression of these lesions. Complete surgical
Fig. 2 e MRI T1 Brain Contrast after 1st surgery.
Fig. 3 e Post-operative MRI after 2nd surgery.
3.
Discussion
4. 242
a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 2 4 0 e2 4 2
Fig. 4 e Histopathology slides.
excision should be the aim. We do not recommend radiation,
as these are benign tumors.
Conflicts of interest
All authors have none to declare.
Acknowledgment
We acknowledge the help of our researcher Ms. Meenakshi
Mohan who helped in the follow-up and compiling data of
these patients.
references
1. Osaki M, Osaki M, Kodani I. Vascular leiomyoma of the nasal
cavity: case report and review of the literature. Yonago Acta
Med. 2002;45:113e116.
2. Fisch U, Mattox D. Microsurgery of the Skull Base. New York:
Thieme; 1988.
3. Shotton JC, Fisch U. Mesenchymal tumors of the skull base
with particular reference to surgical management and
outcome. Skull Base Surg. 1992;2(2):112e117.
4. Kleinschmidt-demasters BK, Mierau GW, Sze CI, et al.
Unusual dural and skull-based mesenchymal neoplasms:
a report of four cases. J Human Pathology. 1998;29(3):
240e245.
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