- Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan
- Department of Otolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan
- Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan
Correspondence Address:
Toshiki Endo
Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan
DOI:10.4103/2152-7806.181902
Copyright: © 2016 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.How to cite this article: Ogita S, Endo T, Nomura K, Ogawa T, Watanabe M, Higashi K, Katori Y, Tominaga T. Nasal cavity epithelioid hemangioendothelioma invading the anterior skull base. Surg Neurol Int 06-May-2016;7:53
How to cite this URL: Ogita S, Endo T, Nomura K, Ogawa T, Watanabe M, Higashi K, Katori Y, Tominaga T. Nasal cavity epithelioid hemangioendothelioma invading the anterior skull base. Surg Neurol Int 06-May-2016;7:53. Available from: http://surgicalneurologyint.com/surgicalint_articles/nasal-cavity-epithelioid-hemangioendothelioma-invading-the-anterior-skull-base/
Abstract
Background:Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that frequently occurs in soft tissues. Patients suffer from local recurrence and remote metastasis because of its malignant potential. Here, we present a rare case of EHE that originated from nasal cavity and invaded intracranially through the anterior skull base.
Case Description:This is a 27-year-old woman who presented a local physician with intermittent epistaxis and a facial pain around her nose. Preoperative studies demonstrated that the tumor invaded into anterior skull base and the dura matter. Therefore, we performed combined skull base and transnasal surgery, which achieved complete resection of the tumor. Postoperative course of the patient was uneventful. No recurrence or distant metastasis was observed in the patient for 2 years following the radical resection.
Conclusions:To date, four cases of EHE in the nasal cavity were reported. This is the first case in which EHE demonstrated invasive potentials with intracranial extension. Radical surgical resection plays an important role for better management of invasive paranasal EHE.
Keywords: Anterior skull base, epithelioid hemangioendothelioma, nasal cavity, vascular tumor
INTRODUCTION
Epithelioid hemangioendothelioma (EHE) is a relatively rare vascular tumor, which was first described by Weiss and Enzinger in 1982.[
EHE frequently occurs in the soft tissue, liver, breast, lung, and bone but rarely in head and neck.[
CASE REPORT
History and presentation
A 27-year-old woman presented to a local physician with an intermittent epistaxis and a facial pain around her nose. Her medical history was unremarkable. Computed tomography (CT) scan demonstrated a heterogeneously enhanced mass lesion centered in the left nasal cavity [
Figure 1
Computed tomography scans at presentation to a local physician (a) and on referral to our institution (b). (a) A heterogeneously enhanced mass was detected in the nasal cavity (arrow). (b) Computed tomography scan without contrast demonstrated rapid progression of the tumor. The tumor invaded into the left orbit (arrow)
She was referred to our institute for surgical treatment of a nasal cavity tumor. On admission, she suffered from anosmia and diplopia. CT scan, performed 3 months after the first scan, demonstrated that the enlarged mass lesion breaking through the medial wall of the left orbit and pushed the eye laterally [
Pathological study of surgical specimen
Hematoxylin and eosin-stained sections showed neoplastic cells forming small intracellular lumina that included red cells. The endothelium covering blood vessels was hyperplastic. No mitosis and necrosis were observed [
Surgical intervention
Magnetic resonance images indicated that the tumor had invaded the anterior skull base dura matter [
Before the operation, spinal drainage was introduced to reduce brain retraction. Neurosurgeons conducted bilateral frontal craniotomy and excised the anterior skull base dura where the tumor had invaded. The left anterior and posterior ethmoidal arteries were coagulated and cut. Following skull base dissections, otolaryngologists achieved complete removal of the tumor through the canine fossa using an endoscope. We confirmed that the tumor was adhesive but had not invaded into the orbit. Intraorbital component was left intact as we had planned preoperatively. Following resection of the tumor and the dura matter, skull base reconstruction was performed using the pericranial flap, fascia lata, and titanium mesh plate.
Postoperative course
The patient recovered uneventfully from the radical resection of the tumor. Her diplopia improved. She has been free from local recurrence or distant metastasis for 26 months after the surgery.
DISCUSSION
EHE is an uncommon vascular tumor of the soft tissues characterized by the proliferation of endothelial cells with an epithelioid morphology.[
Based on the reported cases, it is difficult to establish therapeutic protocols for paranasal EHE. Because the data are limited, the role of radiotherapy and chemotherapy cannot be completely evaluated. However, as suggested in previous literatures,[
A recent study of 49 soft tissue EHEs indicated the higher mitotic activity (>3/50 HPF) as one of the poor prognostic factors.[
CONCLUSION
In this case, paranasal EHE invaded the anterior skull base and dura matter. Although the tumor was histologically aggressive, combined anterior skull base and transnasal approaches achieved total removal of the tumor. Radical surgical resection was important to provide a favorable outcome in EHE.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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