Elsevier

World Neurosurgery

Volume 103, July 2017, Pages 951.e5-951.e12
World Neurosurgery

Case Report
The Unusual Presentation of a Myxoma Within the Sphenoid Sinus: Case Report and Review of the Literature

https://doi.org/10.1016/j.wneu.2017.04.019Get rights and content

Background

We describe a rare case of a sphenoid sinus myxoma that was resected via an endoscopic endonasal skull base approach. We review the literature regarding these rare tumors of the paranasal sinuses.

Case Description

A 72-year-old woman was diagnosed with an incidental sphenoid sinus tumor and left sphenoid wing meningioma during a workup for left-sided proptosis and diplopia. Biopsies of the sphenoid wing and sphenoid sinus tumors were obtained. After undergoing surgical resection of the meningioma, the patient then underwent definitive resection of the sphenoid sinus myxoma via endoscopic endonasal skull base approach. Postoperative imaging demonstrated a gross total resection. The patient suffered postoperative thromboembolic complications due to underlying hypercoagulable state but made a complete recovery and returned to her neurologic baseline. There has been no evidence of recurrent myxoma in the sphenoid sinus 24 months after surgery.

Discussion

Myxomas are benign tumors derived from primitive mesenchyme. Myxomas very rarely present in the paranasal or skull base location. Complete surgical resection is the primary treatment for these tumors. The endoscopic endonasal approach is an effective technique for resecting various benign and more aggressive extradural skull base tumors.

Conclusions

Myxomas of the sphenoid sinus are rare. The endoscopic endonasal skull base approach is an effective and minimal access technique for resection of this rare tumor of the sphenoid sinus.

Introduction

Myxomas are benign neoplasms that histologically resemble the mucinous appearance of the umbilical cord.1 In 1948, Stout2 characterized myxomas as tumors that are derived from primitive mesenchyme that do not metastasize. Myxomas most frequently are found in the atria but can also present in bone, skin, skeletal muscle, and subcutaneous tissue. Head and neck myxomas are rare and are more likely to be present in the mandible, maxilla, and soft tissue of the face.3 There are even rarer reports of myxomas occurring in the nasal cavity, paranasal sinuses, and nasopharynx.4, 5, 6, 7 To diagnose a myxoma, other neoplasms with myxoid elements such as schwannomas, liposarcomas, rhabdomyosarcomas, meningiomas, chordomas, and chondroblastic and fibroblastic tumors must be excluded.8 Sato et al.8 and Moore et al.9 described cases of myxoma arising in the sphenoid sinus that were resected via an endoscopic endonasal approach and a transfacial approach, respectively. We now describe in detail another rare case of a sphenoid sinus myxoma that was resected successfully via the endoscopic endonasal skull base approach and review the literature regarding these rare skull base tumors.

Section snippets

History and Presentation

A 72-year-old woman presented with proptosis, left periorbital fullness, and diplopia. She underwent magnetic resonance imaging (MRI), which demonstrated a sphenoid wing meningioma as well as a sphenoid sinus tumor. The lobulated 4.5 × 3.0 × 4.4 cm mass in the sphenoid sinus was heterogeneously hyperintense on T2 sequences and enhanced avidly and heterogeneously on T1 sequences with gadolinium. There was central hypointensity on T1-enhanced sequences. The lesions appeared discontiguous and

Discussion

Myxomas of the head and neck are very rare benign neoplasms derived from primitive mesenchyme.2 In the head and neck, these tumors most likely are present in the mandible and maxilla.3 Myxomas of the paranasal sinus constitute an even smaller minority of cases within the head and neck.8, 9, 12, 13, 14 Paranasal sinus myxomas are usually soft, painless masses15 and are very difficult to diagnose and treat. Most paranasal sinus myxomas develop in the maxillary sinus. These neoplasms have a

Conclusions

Myxoma of the sphenoid sinus is a rare tumor of the paranasal sinuses and skull base. The literature regarding paranasal sinus myxomas is reviewed. We describe a (rare) case of a sphenoid sinus myxoma. Myxomas are benign tumors that are treated by complete surgical resection. We describe and demonstrate the feasibility of a gross total resection of a skull base myxoma via a minimally invasive endoscopic endonasal approach that is performed with the aid of an endoscopic endonasal specific

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    Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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