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| CASE REPORT |
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| Year : 2014 | Volume
: 4
| Issue : 2 | Page : 63-65 |
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Pediatric laryngeal schwannoma: Case report and literature review
Abdelrahman E. M. Ezzat
Department of ENT, Faculty of Medicine, Al-Azhar University Hospitals, Cairo, Egypt
| Date of Web Publication | 21-May-2015 |
Correspondence Address:
Dr. Abdelrahman E. M. Ezzat
Department of ENT, Faculty of Medicine, Al-Azhar University Hospitals, Cairo
Egypt
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2230-9748.157473
 Abstract | | |
Neurogenic tumors of the larynx, especially the schwannomas are rare. Hence, we report a case of a schwannoma in a 15-year-old male child, which was excised endoscopically. The goal of this report is to advert to this rare disease and to review and discuss diagnostic methods and treatment options. The definite diagnosis is histopathological examination. Endoscopic resection for smaller lesions and external approaches for larger lesions are recommended treatment options, and adjuvant treatment is not required. Keywords: Laryngoscopic surgery, pediatric laryngeal tumors, schwannoma
How to cite this article:
Ezzat AE. Pediatric laryngeal schwannoma: Case report and literature review. J Laryngol Voice 2014;4:63-5 |
| Introduction | |  |
The majority of schwannomas present in the parapharyngeal space, with schwannomas in the head and neck accounting for 25-45% of those identified clinically. The larynx remains a rare site. [1] They represent 0.1% to 1.5% of all benign laryngeal tumors. Indeed, it represents <1.5% of all benign tumors (the majority being papillomas and chondromas), schwannoma being slightly more frequent than neurofibroma. [2] Eighty are located in the aryepiglottic fold and 20% in the false or true vocal cords. [3] Almost all neurogenic tumors of the larynx arise from the supraglottis, the true vocal cord representing a rare origin site. [4] These tumors in the larynx may present at any age, with a slight female preponderance. [1]
| Case report | | |
A 15-year-old male child was referred to the Otolaryngology clinic for progressive dyspnea and hoarseness over the last 3 months with no weight loss, progressed to stridor and severs dyspnea and dysphagia in last few days. Through the history, the condition started from more than 2 years with dysphonia and globus sensation. Fiberoptic laryngoscopy revealed a large submucosal mass within the right half of larynx obstructing the view [Figure 1]a. The mobility of the vocal cords cannot be assessed. On examination of the neck, there was no palpable lymph node. Computerized tomography showed an isoattenuated with mild heterogeneously enhanced mass in the larynx mainly on the right side, with compression of the glottic area [Figure 2]a and b, the mass extended in the supraglottic area [Figure 3]a and b. | Figure 1: (a) A preoperative flexible fiberoptic nasopharyngoscopy, show large submucosal mass within the right part larynx obstructing the view (b) Early postoperative flexible fiberoptic nasopharyngoscopy shows mobile both vocal cords with complete removal of the tumor, (A = arytenoids, T = tumor, E = epiglottis and G = glottis)
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 | Figure 2: (a) Computerized tomography scan axial cuts shows, an isodense mass in the larynx mainly on the right side (b) Computerized tomography scan axial cuts shows, an isodense mass in the larynx mainly on the right side, with compression of the supraglottic area, (T = tumor, G = glottis and AE = aryepiglottic fold)
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 | Figure 3: (a) Computerized tomography scan sagittal shows, an isodense mass in the larynx extended in the supraglottic area, (T = tumor) (b) Computerized tomography scan coronal cuts shows, an isodense mass in the larynx extending in the supraglottic area, (T = tumor)
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Under general anesthesia, endoscopic laryngeal microsurgery was performed. The lesion measured 30 mm × 20 mm × 15 mm, thick walled and firm in consistency [Figure 4]. After excision, a mucosal microflap was replaced over the raw area. Histological examination revealed a nodular lesion comprising hypercellular spindle cells with prominent palisading of their nucleus. Postoperatively, the patient's voice improved. Repeat fibroptical examination at 6 months after surgery showed mobile both vocal cords [Figure 1]b.
| Discussion | | |
Schwannomas are benign slow-growing tumors that arise from the Schwann cells of any nerve (peripheral, cranial, or autonomic). Those affecting the head and neck are commonly intracranial (e.g., the vestibular nerve) or parapharyngeal (arising from glossopharyngeal, vagal, accessory, hypoglossal nerve, and sympathetic chains). [1]
Patients typically present with dysphonia, stridor, dyspnea, dysphagia or/and globus sensation also lateral neck lump may be present. On laryngoscopy, most lesions appear as a smooth submucosal lesion. It may obstruct the view of the laryngeal inlet and result in reduced mobility of the vocal cord. The latter may be due to "pseudo-fixation" of the cricoarytenoid joint as a result of mass effect of the lesion. [5]
Computerized tomography and magnetic resonance imaging (MRI) are valuable in defining the nature and extent of the lesion, with MRI offering superior soft tissue delineation. Typically, the lesion is sharply demarcated, round or oval, isoattenuated with muscle, and often heterogeneously enhanced. [6]
The definitive diagnosis is performed histologically. Enger and Weiss established three histological criteria for the diagnosis of schwannoma: Encapsulation, presence of Antoni A and/or Antoni B stroma, and S-100 protein positivity. [7] In Antoni A (cellular region), the spindle-shaped Schwann cells are compactly arranged with nuclei occasionally lining up in palisades to form Verocay bodies. Antoni B (less cellular) describe loosely arranged spindle Schwann cells within a myxoid matrix. In contrast, neurofibromas are unencapsulated and comprise a variety of cell types: Elongated spindle Schwann cells interwoven with axons and collagen fibers. An important feature is that schwannoma grows extrinsic to the nerve fiber whilst in neurofibroma, the tumor is entwined with the parental nerve fascicles. [5]
The only effective therapeutic option in benign neurogenic laryngeal tumors is complete resection. Since the diagnosis can only be made histologically, direct laryngoscopy with biopsy of the lesion will usually be the first step in treatment. [8] The surgical separation of the tumor from the nerve is theoretically possible in schwannoma, whilst in neurofibroma it is impossible. [9] Following surgery, restoration of vocal cord mobility has been reported independent of the approach. [8] Complete surgical excision of the tumor should be planned according to the individual requirements of each case. Most authors favor external approaches with alternative airway provisions such as a preliminary tracheotomy in larger tumors. Median or lateral thyrotomy or median or lateral pharyngotomy are recommended. [10] In smaller tumors, endoscopic (laser-assisted) resection of the tumor can be a reasonable treatment option. Independent of the approach, restoration of the vocal cord mobility is possible even if it was immobile prior to surgery. [8]
| Conclusions | | |
The most common site of schwannoma in larynx is aryepiglottic fold and false cords. The definite diagnosis is histopathological examination. Endoscopic resection for smaller lesions and external approaches for larger lesions are recommended treatment options, and adjuvant treatment is not required.
| References | | |
| 1. | Patrick J. Benign neoplasms and other tumours of the larynx. In: Anniko M, editors. Otorhinolaryngology, Head and Neck Surgery. Germany: Springer; 2010. p. 487-98. r  |
| 2. | Jones SR, Myers EN, Barnes L. Benign neoplasms of the larynx. Otolaryngol Clin North Am 1984;17:151-78. |
| 3. | Fini-Storchi I, Frosini P. Laryngeal neurinoma. A case report and review. ORL J Otorhinolaryngol Relat Spec 1997;59:182-5. |
| 4. | Thomas RL. Non-epithelial tumours of the larynx. J Laryngol Otol 1979;93:1131-41. |
| 5. | Rosen FS, Pou AM, Quinn FB Jr. Obstructive supraglottic schwannoma: A case report and review of the literature. Laryngoscope 2002;112:997-1002. |
| 6. | Lin J, Martel W. Cross-sectional imaging of peripheral nerve sheath tumors: Characteristic signs on CT, MR imaging, and sonography. AJR Am J Roentgenol 2001;176:75-82. |
| 7. | Woodruff JM, Kourea HP, Louis DN, Schethauer BW. Schwannoma. In: Kleihues P, editors. WHO Classification of Tumours: Pathology and Genetics of Tumorus of Nervous System. 2 nd ed. Lyon: IARC Press; 2000. p. 164-6. |
| 8. | Taylor J, Stiefel M, Park SY. Schwannoma of the true vocal fold: A rare diagnosis. Ear Nose Throat J 2006;85:52-3, 59. |
| 9. | Jones SR, Myers EN, Barnes L. Benign neoplasms of the larynx. Otolaryngol Clin North Am 1984;17:151-78. |
| 10. | Elias MM, Balm AJ, Peterse JL, Keus RB, Hilgers FJ. Malignant schwannoma of the parapharyngeal space in von Recklinghausen's disease: A case report and review of the literature. J Laryngol Otol 1993;107:848-52. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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