4 6 Vol. 6 No.

1, January-March 2004

JK SCIENCE
From the Deptt. of Otorhinolaryngology & Head and Neck Surgery, Govt. Medical College, Srinagar (J&K). Correspondence to : Dr. Shafkat A. Lone, Deptt. of ENT & Head & Neck Surgery, Govt. Medical College, Srinagar (J&K)

Sub Glottic Schwannoma of the Larynx

Shafkat A. Lone, Masood A. Khan, Mohd. Lateef, Rauf Ahmad Introduction Schwannoma is a tumor derived from the schwan cells which are embeded in the neurilemal sheath as a multinucleated syncytial network (1,2). Approximately 25% to 35% of all reported schwannomas occur in the head and neck region (1).However, schwannomas involving the larynx arerare (2,3). Shouchnek (4) is credited with the original report in 1925. Almost all laryngeal schwannomas present insiduously and arise from the aryepiglottic fold or arytenoids (5). Case report An 80 year old male, farmer was admitted in the Deptt. of Otorhinolaryngology and Head and Neck Surgery, Government Medical College, Srinagar with chief complaints of hoarseness of voice of 12 years duration in May, 2001. Examination of the larynx showed normal aryepiglotic folds, arytenoids, ventricles and left vocal cord. Mobility of the right vocal cord was impaired. A mucosa-covered swelling occupying the right subglottic region was seen. The surface of the swelling was smooth and a few prominent blood vessels were present over it. There was no involvement of lymph nodes.CT scan of the neck showed a soft tissue hyperdense lesion in the Abstract Schwannomas are rare neurogenic tumors. Their laryngeal location is uncommon.We present a case of subglottic schwannoma which is very rare. Key words Schwannoma, sub-glottis. subglottic region on the right side extending to the right vocal cord. The airway was adequate occluding less than 50% of the subglottis (Fig 1). Direct laryngoscopy was done which confirmed the findings on indirect laryngoscopy. Biopsy was taken and sent for histopathological examination, which revealed it to be a schwannoma (Fig 2). Surgery was advised which was refused by the patient and he left against advise.Patient was re-admitted in the department on 24th of December, 2001 with severe airway obstruction. Emergency tracheostomy was done. MRI was done which showed a hyperintense mass in the subglottic region almost occluding the whole lumen (Fig 3). Surgical excision of the tumor by laryngofissure was performed under general anaesthesia. The tumor was found to arise from the right side of the subglottis extending to the undersurface of vocal cords and into the tracheal lumen (first and second tracheal rings). The tumor was pale yellow, globular, smooth surfaced. The tumor was removed in toto and measured 5x2.5cms. Histopathologic characteristics of

the tumor revealed it to be a schwannoma.The immuno histochemistry for S100 protein was positive.The postoperative course was unremarkable and the patient was

CASE REPORT
Vol. 6 No. 1, January-March 2004 4 7

JK SCIENCE decanulated on 10th post-operative day.The patient is under regular follow up and is doing well. Discussion Schwanomas arise from the schwan cells of peripheral, cranial or sympathetic nerves.They were first described by Verocay in 1910 who called them neurinomas (6). Schwannomas involve males and females equally and can occur at any age (7). With expansion of the tumor, the nerve fibers become splayed over the outer aspect of the capsule, rather than getting incorporated within the substance of the tumor. The most common nerve of origin of laryngeal schwannoma is the internal branch of superior laryngeal nerve (8). The nerve of origin, however, is difficult to identify at operation (9), as occurred in our patient. Still the anatomical location of the tumor indicated that it arose from the branches of recurrent laryngeal nerve. Various surgical approaches for the removal of laryngeal schwannoma have been mentioned in the literature, varying from endoscopic removal, laryngofissure and anterior and lateral pharyngotomies to lasers (10,11). The prognosis is good if surgical excision is complete. Malignant transformation is rare (8). Radiation should not be employed as a form of therapy, for schwanomas are highly radio-resistant.
References 1. Putney FJ, Moran JJ, Thomas K. Neurogenic tumors of head and neck. Laryngoscope 1954; 24: 1, 037. 2. Caix, Dong P,Wang T.Neurilemommas of larynx (article in Chinese). Lin Chuang Ex Bi Yau Hou Ke Za Zhi 1998; 12 (3): 111-12. 3. Plantet MM, Hagay C, De Maulmont C, Mahe E, Banal A, Gentile A,Cherel P, Mayras C. Laryngeal schwannomas. Eur J Radiol 1995; 21(1): 61-66. 4. Schouchnek, quoted by Whittman DE et al. Neurilemommas of the larynx. J Laryngol Otol 1979; 84: 747-49. 5. Supance JS, Quenelle DJ,Crisman J.Endolaryngeal neurofibromas. Otol Head Neck Surg 1980; 88: 74-78. 6. Das, Gupta TK et al. Benign solitary schwannomas. Cancer 1969; 24: 355-66. 7. Hawkins DB, Luxford WM. Schwanomas of head and neck in children. Laryngoscope 1980; XC (12): 1921-26. 8. Mehta S. Neurilemomma of the larynx. Ear, Nose Throat J 1991; 70 (2): 114. 9. Nauson EMC. Neurilemomma of the larynx. Head Neck Surg 1978; 69-74. 10. Sanghvi V, Lala M, Borges A, Rodrigues G, Pathak KA, Parikh D. Lateral thyrotomy for neurilemomma of the larynx. J Laryngol Otol 1999; 113 (4): 346-48.
Fig. 1. CT scan of the neck showing a hyperdense lesion in the subglottic region on the right side. Fig. 2. Microphoto showing features suggestive to schwannoma (H%EX40). Fig. 3. MRI of the neck showing a hyperintense lesion in the subglottis occluding whole of the lumen.

Abstract Neurogenic tumours of the larynx, particularly schwannomas are rare. We report a case report of a schwannoma in a 30-year-old woman which was excised endoscopically. The aim of this paper is to highlight this rare condition and management options to the otolaryngological community. 1. Case Report A 30-year-old lady presented to the outpatient clinic with progressive dysphonia and exertional dyspnea over a 2-year period. There were no associated systemic symptoms. She was a nonsmoker and consumed alcohol occasionally. On examination, she was dysphonic but not stridulous or dyspneic. Flexible nasoendoscopy revealed a soft tissue swelling in the supraglottic region starting from the posterior commissure blocking the view of the vocal cords. Computer tomography (CT) showed a well circumscribed 1 . 5 1 . 4 cm low attenuation midline supraglottic mass, commencing just below the aryepiglottic folds and extending onto sclerotic arytenoids (Figures 3 and 4 ). The attenuation of this mass was slightly greater than that of fluid, suggesting this could represent a retention cyst containing proteinaceous secretions. Suspension microlaryngoscopy and biopsy was performed. Intraoperatively, a solid, well-encapsulated lesion was shelled out using cold steel instruments (Figures 1 and 2 ). The patient was extubated postoperatively and did not require a tracheostomy. Histopathological examination confirmed the presence of an arytenoid schwannoma. 3 months following surgery, no recurrence was evident. Figure 1: Mass presenting as a submucosal bulge obstructing view of vocal cords. Figure 2: Postoperative view following complete excision of the schwannoma. Figure 3: Serial CT axial images of the midline supraglottic mass commencing just below the aryepiglottic folds and extending onto arytenoids. Figure 4: Serial CT axial images of the midline supraglottic mass commencing just below the aryepiglottic folds and extending onto arytenoids. 2. Introduction Schwannomas are benign slow-growing tumours that arise from the Schwann cells of any nerve (peripheral, cranial, or autonomic). Those affecting the head and neck are commonly intracranial (e.g., vestibular nerve) or parapharyngeal (arising from glossopharyngeal, vagal, accessory, hypoglossal nerve, and sympathetic chains). Neurogenic tumors of the larynx (e.g., schwannoma or neurofibroma) therefore remain extremely rare. Indeed, it represents less than 1.5% of all benign tumours (the majority being papillomas and chondromas) [ 1 ]. It is a slow, growing encapsulated tumour commonly presenting in the 4th and 5th decade of life, especially women. However, it can present at any age. Over 130 cases have been reported in the worldwide literature since it was first described in 1925 [ 2 ]. The origin of the schwannoma is presumed to be in the internal branch of the superior laryngeal nerve [ 3 ]. Often however, this is not discernible intraoperatively and likely to originate from the smaller distal nerve fibres in the laryngeal submucosa. The most common anatomical site is the aryepiglottic fold, followed by the arytenoids, ventricular folds, and vocal cord [ 4 , 5 ]. The risk of malignant transformation of laryngeal schwannoma is very rare [ 6 ]. From a clinical perspective, it is vital to differentiate a schwannoma from a neurofibroma for 2 reasons. Firstly, neurofibromas have a higher potential of recurrence and malignancy (approximately 10%). Secondly, the presence of neurofibromas should also alert the physician to the exclusion of neurofibromatosis. The definitive diagnosis is established histologically. Patients typically present with dysphonia, stridor, dyspnea, dysphagia, globus sensation, or lateral neck lump. On laryngoscopy, most lesions appear as a smooth submucosal lesion, usually confined to the aryepiglottic fold or false vocal cord. It may obstruct the view of the laryngeal inlet and result in reduced mobility of the vocal cord. The latter may be due to pseudofixation of the cricoarytenoid joint as a result of mass effect of the lesion [ 5 ]. Almost all neurogenic tumors of the larynx arise from the supraglottis, the true vocal cord representing a rare origin site [ 7 ]. CT and 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 [ 8 ]. Calcification which reflects degenerative change, though rare, has been reported in ancient schwannomas. Schwannoma and neurofibromas cannot be distinguished radiologically due to similar findings. The differential diagnosis includes laryngeal cyst and internal laryngocele. 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 [ 9 ]. 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 fibres. An important feature is that schwannoma grows extrinsic to the nerve fibre whilst in neurofibroma, the tumour is entwined with the parental nerve fascicles [ 5 ]. Surgery forms the mainstay of treatment of laryngeal schwannoma. A tracheostomy may be required to secure the airway. The surgical approach depends on the size and location of the lesion. Smaller lesions can be approached endoscopically with or without a laser. Larger tumours may require an external approach, for example, lateral thyrotomy, lateral pharyngotomy, or laryngofissure technique [ 10 ]. Wide excision is necessary to prevent recurrence. Rapid regrowth can occur within months following incomplete resection of the schwannoma [ 5 ]. Following surgery, restoration of vocal cord mobility has been reported independent of the approach [ 11 , 12 ]. 3. Conclusion Despite its rarity, neurogenic tumors of the larynx need to be recognised. The primary management involves securing the airway. Despite various imaging modalities, the distinction between Schwannoma and neurofibromas can only be made histologically. Complete resection of the lesion is necessary to prevent recurrence. < h4>References</h4> S. R. Jones, E. N. Myers, and L. Barnes, Benign neoplasms of the larynx,

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