Leiomyoma of Skull base

Case Report

Leiomyoma of skull base

Rajendra Prasad a,*, Amit Kapoor a, Shyam Sunder b

aDepartment of Neurosurgery, Apollo Hospital, New Delhi, IndiabDepartment of Neurosurgery, Udayan Hospital, Patna (Previously at Apollo Hospital, New Delhi), India

a r t i c l e i n f o

Article history:

Received 31 July 2013

Accepted 7 August 2013

Available online 6 September 2013

Keywords:

Benign mesenchymal tumor of skull

base

Leiomyoma of skull base

Rare intracranial tumors

a b s t r a c t

Leiomyoma of the skull base is an extremely rare tumor and is not well documented. In

this article, we report a case of a leiomyoma of the skull base in a 17 years old African male

whose initial diagnosis was a sphenoidal wing meningioma.

Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved.

1. Introduction

Leiomyoma is a benign tumor that originates from smooth

muscle cell. The most common sites are the uterus, gastro-

intestinal 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 leiomyo-

mas, 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). Forty-

eight hours later he underwent left frontotemporal crani-

otomy 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 rhi-

notomy 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).

Available online at www.sciencedirect.com

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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

it was decided to follow-up the small residual portion of the

tumor involving the cavernous sinus and not to give ste-

reotactic 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 operativeMRI showed: Residualmass 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 borderswith

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 pre-

served except for visual loss in the left eye and no prop-

tosis. There has been no increase in the size of the residual

lesion.

3. Discussion

Enzinger and Weiss (1995), analyzed a total of 7748 leio-

myomas, 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 histologi-

cally benign equivalent.3 These tumors were chon-

drosarcoma, 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 trans-

verse 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. 1 e Pre operative MRI (T1 contrast).

Fig. 2 e MRI T1 Brain Contrast after 1st surgery. Fig. 3 e Post-operative MRI after 2nd surgery.

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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.

r e f e r e n c e s

1. Osaki M, Osaki M, Kodani I. Vascular leiomyoma of the nasalcavity: case report and review of the literature. Yonago ActaMed. 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 basewith particular reference to surgical management andoutcome. 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.

Fig. 4 e Histopathology slides.

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