Otolaryngology -- Head and Neck Surgery

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Myxoid Neurothekeoma of the Caudal Nasal Septum and Tip John Robert Scott, Martin Joseph Bullock, Matthew Hall Rigby and S. Mark Taylor Otolaryngology -- Head and Neck Surgery 2012 146: 868 originally published online 16 November 2011 DOI: 10.1177/0194599811429245 The online version of this article can be found at: http://oto.sagepub.com/content/146/5/868

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Case Report

Myxoid Neurothekeoma of the Caudal Nasal Septum and Tip John Robert Scott1, Martin Joseph Bullock, MD, FRCPC2, Matthew Hall Rigby, MD3, and S. Mark Taylor, MD, FRCSC3

Otolaryngology– Head and Neck Surgery 146(5) 868–869 Ó American Academy of Otolaryngology—Head and Neck Surgery Foundation 2012 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0194599811429245 http://otojournal.org

No sponsorships or competing interests have been disclosed for this article.

Keywords myxoid neurothekeoma, rhinoplasty, nasal tip Received September 20, 2011; revised October 17, 2011; accepted October 18, 2011.

N

eurothekeoma is a rare benign soft-tissue neoplasm of probable peripheral nerve sheath origin.1 It occurs primarily in the head, neck, and upper extremity regions of young adults, predominantly affecting females.1,2 Patients present with a nondescript, painless, firm nodule ranging in size from a few millimeters to several centimeters.3 Located very superficially, these tumors are slow growing and display characteristic histopathological patterns. We present here a unique case of a myxoid neurothekeoma involving the caudal nasal septum and tip. Megahed et al4 reported a case in German literature involving the nasal tip, but to our knowledge, this is the first case reported with septal involvement.

Case Report A previously healthy 48-year-old woman was referred to our clinic with a 6-month history of a mass in her right nasal tip. The lesion was not painful and was nonulcerative, and there was no history of preceding trauma. On examination, the nasal tip appeared bulbous, and there was an obvious softtissue mass in the right side of the caudal septum measuring 1 cm in its greatest dimension. A magnetic resonance imaging scan was performed to determine the extent of the patient’s lesion and to rule out a potential intracranial connection. The imaging correlated with the tentative diagnosis of a lymphatic malformation. After discussion of management options, the patient decided against surgical excision. Two years after her initial consultation, the patient returned with concerns about the mass increasing in size. On examination, the mass had grown considerably since her last appointment and almost completely obstructed the right nostril (Figure 1). Because of decreased right-sided air entry and the overall appearance of her nose, the patient decided to proceed with the surgery.

Figure 1. Preoperative frontal view of the nasal mass.

1

Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada 3 Division of Otolaryngology–Head & Neck Surgery, Dalhousie University, Halifax, Nova Scotia, Canada 2

Corresponding Author: John Robert Scott, Faculty of Medicine, Dalhousie University, Suite 3044, Dickson Building, 5820 University Avenue, Halifax, Nova Scotia, Canada, B3H 1V7 Email: [email protected]


Scott et al

869 The differential diagnosis for neurothekeoma includes a dermoid cyst, lymphangioma, fibrolipoma, dermatofibroma, dermal nevus, encephalocele, neurofibroma, and leiomyoma.2 The curative treatment for all of these benign lesions is surgical excision, and thus, a specific biopsyproven diagnosis is not always necessary once potentially worrisome causes are ruled out. The present case demonstrates this point, as a definitive diagnosis was not achieved until after surgical resection. The above presentation of a slow-growing, painless subcutaneous facial mass is standard for a myxoid neurothekeoma. The defining feature of this case is the unique location of the tumor. Neurothekeomas have been described on dermal surfaces, mucosal surfaces, and even on the tongue, but this is the first case in the world literature to involve not only the nasal tip but also septum. The above case demonstrates that myxoid neurothekeomas, although rare, can occur within the nasal tip and septum and should be included in the differential diagnosis for benign softtissue tumors in this area. The Capital Health Research Ethics Board has approved this case report.

Figure 2. Open rhinoplasty approach showing the tumor (left) and its en bloc removal (right)

An open rhinoplasty approach was used to resect the mass. The lower lateral cartilages were dissected from the mass, and the caudal septal component was freed, removing the cystic 3 3 2 cm tumor en bloc (Figure 2). A columellar strut was used to provide tip support, and despite redundant mucosa, the nasal airway returned to normal. The patient’s postoperative course was uneventful, and at the 6-month follow-up, the patient’s nasal tip appeared remarkably normal, and her septum was midline. The specimen was hypocellular, had diffuse S100 protein positivity, and was largely epithelial membrane antigen (EMA) negative. The final pathological diagnosis of the nasal mass was myxoid neurothekeoma.

Discussion Historically, neurothekeomas can be subclassified into myxoid, cellular, or mixed types.3 In most cases, the diagnosis of a myxoid neurothekeoma is not problematic, whereas the identification of the cellular variant can be difficult because it can mimic other skin lesions including melanoma.1,3 Local excision is the mainstay of treatment for neurothekeomas, and recurrence is due to incomplete removal.1-5 Myxoid neurothekeomas are regarded as having low cellularity, are EMA negative, and show strong S100 positivity among their myxoid stroma histopathologically.5 These tumors are usually located on or around the face, and a preoperative diagnosis is helpful to avoid wide surgical margins.

Author Contributions John Robert Scott, manuscript author; Martin Joseph Bullock, pathology supervisor and manuscript preparation; Matthew Hall Rigby, manuscript preparation; S. Mark Taylor, supervisor, surgeon, and manuscript preparation.

Disclosures Competing interests: None. Sponsorships: None. Funding source: None.

References 1. Levin KA, Hayden R, Hanau CA, Galindo LM. Cytologic findings of myxoid neurothekeoma: case report based on fineneedle aspiration cytology, immunohistochemistry, and correlating histopathology. Diagn Cytopathol. 2002;27:173-176. 2. Wong BYH, Hui Y, Lam KY, Wei WI. Neurothekeoma of the paranasal sinuses in a 3-year-old boy. Int J Pediatr Otorhinolaryngol. 2002;62:69-73. 3. Plaza JA, Torres-Cabala C, Evans H, Diwan AF, Prieto VG. Immunohistochemical expression of S100A6 in cellular neurothekeoma: clinicopathologic and immunohistochemical analysis of 31 cases. Am J Dermatopathol. 2009;31:419-422. 4. Megahed M, Behrendt H, Schaffetter-Kochanek K, Plewig G. Neurothekeoma—a light and electron microscopy, histochemical and immunohistochemical study. Der Hautarzt. 1992;43: 781-785. 5. Lopez-Cepeda LD, Navarrete-Franco G, Novales-Santacoloma J, Enriquez-Merino J. Scar-like lesion on dorsal nose (cellular neurothekeoma). Head Face Med. 2007;3:39.
