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March 19, 2020

An Atypical Cause of Difficulty Swallowing

Author Affiliations
  • 1Division of Otolaryngology–Head & Neck Surgery, Department of Surgery, University of California, San Diego
JAMA Otolaryngol Head Neck Surg. Published online March 19, 2020. doi:10.1001/jamaoto.2020.0133

Schwannomas are benign nerve sheath cell tumors. Whereas 25% to 45% of schwannomas occur in the head and neck region, laryngeal schwannomas are rare.1 Representing 0.1% of all benign laryngeal tumors, schwannomas are most commonly found in the aryepiglottic folds and arytenoids, with most arising from the superior laryngeal nerve.2-4 In 1 series3 of 55 laryngeal schwannomas, only 2 were reported in the pyriform sinus. We present here the third reported case of pyriform schwannoma, to our knowledge.

Report of a Case

A woman of Indian descent in her 70s was referred with a 10-month history of globus pharyngeus, intermittent dysphagia, odynophagia, and unintentional weight loss. The remaining medical history was unremarkable.

Flexible laryngoscopy and flexible endoscopic evaluation of swallow were unremarkable preoperatively. Computed tomographic findings demonstrated a 2×2-cm hypodense mass in the cervical esophagus, with its superior aspect approximating the inferior border of the cricoid cartilage (Figure 1A). Additional radiographic characteristics included soft tissue fullness at the left pyriform sinus, anterior displacement of the membranous trachea, and scalloped contour of the anterior C7 vertebral body.

Figure 1.  Contrast-Enhanced Computed Tomographic Images
Contrast-Enhanced Computed Tomographic Images

Operative direct microlaryngoscopy and rigid esophagoscopy was performed. A Dedo laryngoscope was suspended in the postcricoid space. Only a subtle stalk was visualized extending into the esophageal inlet (Figure 2A). A right-angle laryngeal probe was passed distal to the mass and used to deliver a pendulous mass into the hypopharynx. The mass was firm, 3 cm in cranio-caudal dimension, and was encapsulated with mucosal tissue. It was transected at its pedicle on the medial surface of the left pyriform sinus with an AccuBlade CO2 laser (Figure 2B). Histopathologic examination revealed a schwannoma positive for S-100, negative for desmin, with Ki67 proliferation index of 1% and negative margins (Figure 2C and D). One week postoperatively, she was tolerating a mechanical soft diet and had mild odynophagia. Examination findings showed a healing left pyriform eschar and normal vocal fold movement. At 3 months, she had complete resolution of symptoms and a normal examination.

Figure 2.  Laryngoscopic, Clinical, and Histopathologic Images
Laryngoscopic, Clinical, and Histopathologic Images

A, Laryngoscopic image with 0° telescope via Dedo laryngoscope of a mass pedicled at the left pyriform sinus and prolapsing into the esophagus. B, Clinical image of gross specimen postresection. C, Photomicroscopic image of specimen showing Antoni A regions, consisting of compact bundles of cells with palisading nuclei, and Antoni B regions, edematous, loosely arranged spindle cells in a myxoid matrix, consistent with schwannoma (original magnification ×20). D, Immunohistochemical stain results were positive for S-100 (original magnification ×2).

Discussion

Patients with laryngeal schwannoma most commonly present with dysphonia.3 The true vocal fold ipsilateral to the lesion is often immobile or hypomobile, often secondary to nerve compression.2,3 Other mass effect–related symptoms of laryngeal schwannoma include dysphagia, globus pharyngeus, and stridor.1,2 One case of asphyxial death has been reported.4

The rare nature of laryngeal schwannoma makes diagnosis challenging. These nerve sheath tumors can arise from the pharyngeal plexus, the internal branch of the superior laryngeal nerve, or branches of the recurrent laryngeal nerve, all of which innervate the mucosal and submucosal layers of the pyriform sinus.5 On flexible laryngoscopy, these tumors appear as round submucosal tissue fullness.3 Cystic change has been described3 and was observed in this case. Imaging may be used to establish mass extent and differentiate benign from malignant tumors, but these modalities are not always able to differentiate schwannomas from other benign laryngeal tumors.3 Definitive histopathologic diagnosis is based on the presence of a clear capsule, Antoni A and Antoni B regions, and S-100 positivity.2,3

As with other head and neck schwannomas causing considerable mass effect, en bloc resection is the favored therapeutic modality.3,4 Depending on tumor size and location, excision can be achieved via transoral or open cervical approach.3 Prognosis and outcome after excision are very good with a low chance of recurrence.3

This case represents, to our knowledge, the third report of schwannoma arising from the pyriform sinus and demonstrates an example of a large laryngeal schwannoma excised via microdirect laryngoscopic laser resection approach without preliminary tracheostomy. Efforts to define standard airway management for these tumors are made difficult by the exceeding rarity of laryngeal schwannoma. Nonetheless, schwannoma should be considered in the differential diagnosis for postcricoid masses.

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Article Information

Corresponding Author: Philip A. Weissbrod, MD, Division of Otolaryngology–Head & Neck Surgery, Department of Surgery, University of California San Diego, 8899 University Center Lane, San Diego, CA 92122 (pweissbrod@ucsd.edu).

Published Online: March 19, 2020. doi:10.1001/jamaoto.2020.0133

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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