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Open Access Case report

Rapid-developed primary malignant myoepithelioma in the cavernous sinus: a case report

Yuan Hong1, Song-Xue Guo2, Sheng Chen1, Damon Klebe3, Jian-Min Zhang1 and Qun Wu1*

Author Affiliations

1 Department of Neurosurgery, 2nd Affiliated Hospital, School of Medicine, Zhejiang University, 88# Jiefang Road, Hangzhou, Zhejiang 310009, China

2 Department of Neurosurgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, 3# East Qingchun Road, Hangzhou, Zhejiang 310016, China

3 Department of physiology, Loma linda university, Risley Hall 11041 Campus Street, Loma Linda, CA 92350, USA

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BMC Neurology 2013, 13:40  doi:10.1186/1471-2377-13-40

Published: 4 May 2013

Abstract

Background

Malignant myoepithelioma is a relatively rare malignant tumor occurring most frequently in the salivary glands. A few isolated cases have been described in other locations, including soft tissue, bone, lung, bronchus, oral cavity, nasopharynx, larynx, and maxillary sinus. Malignant myoepithelioma, however, is uncommonly involved within the cavernous sinus. To the best of our knowledge, this is the first report of malignant myoepithelioma arising from within the cavernous sinus.

Case presentation

Herein, we report a case of a 48-year-old woman who presented a 1-month history of diplopia and blepharoptosis as well as radiological evidence of a rapidly developing cavernous sinus tumor. The patient underwent a trans-sphenoidal biopsy and a histological diagnosis indicated a malignant myoepithelioma. After diagnosis, the tumor grew rapidly and her clinical condition deteriorated progressively. Therefore, a pterional craniotomy with partial tumor removal was performed. The patient’s clinical state was worsened, and she died two months after the initial operation. Because the malignant myoepithelioma could not be traced to an organ of origin, other than the cavernous sinus, this case was diagnosed as a primary intracranial malignant myoepithelioma.

Conclusion

The purpose of presenting this case report is to raise awareness among clinicians to consider malignant myoepithelioma as a differential diagnosis when a cavernous sinus mass is identified. Furthermore, an ideal management strategy for malignant myoepithelioma is not known and the prognosis seems to be unfavorable; therefore, more cases are needed to enhance our knowledge of the diagnosis, treatment, and prognosis of this rare intracranial lesion.

Keywords:
Myoepithelioma; Cavernous sinus; Treatment; Pathology