|Year : 2017 | Volume
| Issue : 1 | Page : 18-20
Suprahyoid transverse pharyngotomy for schwannoma of larynx in an adolescent male: A case report
Kavita Sachdeva, Vineet Kaul
Department of Otorhinolaryngology and Head and Neck Surgery, NSCB. Medical College and Hospital, Jabalpur, Madhya Pradesh, India
|Date of Web Publication||14-May-2018|
Dr. Kavita Sachdeva
Department of ENT, NSCB. Medical College and Hospital, Jabalpur - 482 003, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Schwannomas are benign encapsulated nerve sheath tumors and are unusual in the larynx. Only about 100 cases have been reported worldwide. We report a case of an 18-years-old male having progressive dysphagia and dysphonia for 5 months. We have discussed clinicopathological and radiological findings and surgical management.
Keywords: Larynx, schwannoma, suprahyoid pharyngotomy
|How to cite this article:|
Sachdeva K, Kaul V. Suprahyoid transverse pharyngotomy for schwannoma of larynx in an adolescent male: A case report. J Laryngol Voice 2017;7:18-20
| Introduction|| |
A schwannoma is a benign tumor originating from the Schwann cell of the neural sheath. The head and neck region account for 25%–40% of the schwannomas. They are characteristically slowly progressive, solitary, and well-encapsulated tumors. They affect both genders equally and occur most commonly in the fifth and sixth decades of life. About 80% are believed to arise from aryepiglottic fold (AEF), but cases have been reported affecting false and true vocal cords and arytenoids  as well.
| Case Report|| |
An 18-year-old adolescent male Labourer by occupation presented with complaint of dysphagia only for solids and dysphonia for 5 months. He also had a history of snoring for 4 months, pain in neck for 3 months, and cough with expectoration for 2 months. He had developed vomiting along with cough after meals for 15 days. Dysphagia and dysphonia were progressive and medically intractable. The patient was addicted to betel nut chewing.
Neck examination revealed enlarged laryngeal framework. Laryngeal crepitus was present. Indirect laryngoscopy (IDL) revealed a smooth globular mass originating in the left AEF compressing the laryngeal inlet and reaching up to the base of laryngeal surface of epiglottis. The overlying mucosa was intact, smooth, and pink in color. On palpation, it was soft-to-firm tender and did not bleed.
Direct laryngoscopy revealed globular mass arising from left AEF and left pyriform fossa (PF) touching postwall of laryngopharynx, shifting glottic chink to the right along with compression of vocal cords which are difficult to visualize and narrow glottic aperture. Biopsy was taken from the mass which revealed benign spindle cell neoplasm possibly Neurofibroma with nonspecific chronic inflammatory cells.
Routine blood investigations showed no abnormality. Ultrasonography of the neck showed evidence of 3 cm × 3.5 cm size heterogeneous mass with well-defined margins with calcification and few necrotic areas, posterior to thyroid with internal vascularity suspicious of paratracheal mass.
Computed tomography (CT) scan revealed large well-defined heterogeneously enhancing soft-tissue density, involving upper aerodigestive tract [Figure 1], measuring 5.4 cm × 3.3 cm in axial plain, craniocaudal extension approximately in 5.8 cm in sagittal plain, predominantly involving hypopharynx possibility of benign mass lesion extending from C4 level to D1 level. It extended superiorly up to epiglottis involving left AEF, inferiorly involving cricoid cartilage, encasing right PF with partial effacement of the left PF, and posteriorly touching posterior pharyngeal wall. No evidence of erosion of hyoid was noted. Nonspecific lymph nodes were present in jugular chain.
|Figure 1: First image shows radiopaque mass on the lateral soft-tissue neck skiagram; three subsequent images are sagittal cuts of plain computed tomography neck showing soft-tissue mass at C5–C7 level (white arrow)|
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The patient was managed surgically by tumor removal through a suprahyoid transverse pharyngotomy approach with tracheostomy under general anesthesia. A transverse suprahyoid incision was given. Geniohyoid and mylohyoid were separated from hyoid upper borders. The mucosa was incised for approach. The lesion was visualized and needle aspiration was done which yielded a dry tap. The mass was dissected submucosally by blunt dissection. Solid tissue mass was delivered out which was found attached to the left AEF and medial wall of PF. Mass was encapsulated, friable, and removed in toto [Figure 2]. Wound was closed in two layers. Postoperative histopathological report showed the peripheral nerve sheath tumor suggestive of schwannoma.
|Figure 2: Intraoperative image showing excision of mass by suprahyoid pharyngotomy approach; inset image showing mass after complete excision|
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Planned tracheostomy decannulation was done after IDL examination. Repeat IDL examination done after 1 week showed restoration of normal laryngeal anatomy. The patient was followed up at 12 and 24 months showing no complications.
| Discussion|| |
Schwannomas were first described by Verocay in 1908, who coined the term neurinomas. There are two histological types of schwannoma – a typical biphasic pattern with areas of hypercellularity called Antoni A and a hypocellular type wherein a highly myxoid matrix predominates known as Antoni B. The tumor is positive for S-100 protein. Schwannomas should be differentiated from neurofibromas as the latter has a high malignant potential (about 10%) and is associated with neurofibromatosis. Definitive diagnosis is established histologically where schwannoma is well encapsulated and grows extrinsic to nerves while neurofibroma is unencapsulated and is intertwined with nerve fascicles.
Ultrasonography of larynx is a useful tool that can give additional information about anatomical relations of mass, texture, vascularity, and presence or absence of thyroid cartilage invasion. It can also be used for guided fine-needle aspiration cytology. CT and magnetic resonance imaging (MRI) give similar information but neither can differentiate schwannoma from other neck masses. “ Target sign “ has been described in CT and MRI scan findings of schwannoma – a circumscribed solid mass with nerve bundle entering into its center. It has a sensitivity of 50%.
Since schwannoma is a radioresistant tumor, surgical excision is the treatment of choice. Microlaryngoscopic removal and CO2 laser removal have been described previously in literature for small and transorally accessible lesions.,, For bulky tumors, external approach through midline pharyngotomy or thyrotomy with tracheostomy is preferable  as postoperative laryngeal edema can ensue leading to respiratory distress. In all cases, surgery for schwannoma should be radical as residual tissue can rapidly recur leading to airway compromise.
| Conclusion|| |
The present case of laryngeal schwannoma in an adolescent male presenting with a bulky tumor leading to characteristic findings of dysphagia with vomiting and dysphonia has not been described previously in literature after extensive search conducted online by us. The case was unique as the tumor was bulky thus narrowing the glottic opening and was managed by suprahyoid pharyngotomy approach. This approach helped in complete excision of the tumor and complete relief from all symptoms with no recurrence in follow-up period.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]