Laryngeal Neurofibroma Research Articles

Incidentally Diagnosed Supraglottic Neurofibroma in Adult

Neurogenic tumors of the larynx are extremely rare, especially in adults. Most cases reported to date have been in children with neurofibromatosis. Because of their slow growth and variability in location and size, laryngeal neurofibromas may remain asymptomatic for years or become symptomatic at birth. Symptoms include stridor, hoarseness, dysphagia, dysphonia, globus sensation, dyspnea, hoarseness, and feeding difficulties and are usually progressive. There is little information about these tumors, but knowing this diagnosis is very important, especially for physicians in charge of airway management, and missing it can have fatal consequences. We present a case of a laryngeal neurofibroma that was discovered incidentally in a 36-year-old adult male.

Application of peroral endoscopic-assisted laryngeal microsurgery in children with laryngeal neurofibromas

Objective:To investigate the clinical manifestations and the effect of peroral endoscopic-assisted laryngeal microsurgery for children with laryngeal neurofibroma, and to provide clinical reference for the diagnosis and treatment of this disease. Methods:The clinical data of 4 children with laryngeal tumors admitted to the Department of Otorhinolaryngology, Children's Hospital of Chongqing Medical University from January 2021 to June 2023 were retrospectively analyzed. Laryngeal tumors were removed by peroral endoscopic-assisted laryngeal microsurgery. One case underwent tracheotomy at the same time, and one case was simultaneously performed with laryngeal T tube placement and tracheotomy. Results:Surgical resection is the best treatment for laryngeal neurofibroma, and laryngeal microsurgery should be actively used for patients with surgical indications.This surgical method has the advantages of good efficacy, minimal invasion, aesthetics and preservation of laryngeal function, which not only ensures safety, but also improves the quality of life after surgery, and has the value of development and promotion.

Shredded carrots in the larynx: A rare case of neurofibroma of vocal cord

Neurofibromatosis (NF) is a common disorder resulting in tumors on nerve tissue. It rarely affects the larynx and even rarer is an isolated laryngeal neurofibroma. Literature reveals

Isolated paraglottic neurofibroma; lateral thyrotomy approach: a case report

Isolated neurofibromas are rare tumours of the paraglottic space. A 59 year old lady presented with progressive hoarseness for 5 years. Examination revealed a smooth submucosal globular mass in the glottosupraglottic region on the left side. After confirming findings on a contrast CT scan lateral thyrotomy approach was employed to excise the tumour in toto. Histopathology revealed a neurofibroma. Further workup to exclude neurofibromatosis (NF1) was done. Isolated laryngeal neurofibromas present a challenge to surgeons for providing complete excision of tumour while giving a good voice quality and lateral thyrotomy approach is the ideal approach for satisfying both these parameters.

The efficacy and safety of the flexible fiber CO2 laser delivery system in the endoscopic management of pediatric airway problems: Our long term experience

ObjectiveTo report the use of flexible fiber CO2 laser in the endoscopic management of pediatric airway cases. MethodsA retrospective review was conducted of patients who underwent CO2 laser-assisted airway procedures between September 2007 and January 2014 at a tertiary pediatric hospital. ResultsSixty-eight patients underwent 80 procedures utilizing flexible fiber CO2 laser. Procedures included supraglottoplasty (n = 32), laryngeal cleft repair (type I [n = 10], type II [n = 7], type III [n = 6]), suprastomal granuloma excision (n = 6), cordotomy (n = 4), laryngeal neurofibroma excision (n = 4), laryngeal granulomatous mass excision (n = 1), subglottic stenosis excision (n = 6), division of glottic web (n = 2), subglottic cyst excision (n = 1), and supraglottic biopsy (n = 1). Ages ranged from 8 days to 21 years (median 11 months). No intraoperative or postoperative complications related to the use of laser were noted. ConclusionsThe flexible fiber CO2 laser can be safely and effectively used to address a variety of pediatric airway lesions. Previously, the use of CO2 laser in minimally invasive airway surgery has been limited due to the articulating arm carrier, absence of a hand piece, and the direct line-of sight view required. The fiber allows the cutting beam to be directed at the site of the lesion and bypasses limitations posed by other laser systems.

A Case of Plexiform Neurofibroma Arising from Laryngeal Surface of Epiglottis

Neurofibroma is characterized as a benign, slow growing neoplasm, originating from Schwann cells or fibroblast in peripheral nerve sheaths. It may appear as a solitary tumor or have multiple localizations in von Recklinghausen disease. They are commonly found in the gastrointestinal tract and laryngeal neurofibromas are extremely rare, accounting for only 0.03 to 0.1% of benign tumors of the larynx. The aryepiglottic fold and arytenoid are the common site of occurrence for laryngeal neurofibroma, because the branch of the superior laryngeal nerve is involved. We present a case of solitary plexiform neurofibroma arising from the laryngeal surface of epiglottis in a 55-year old female who found the lesion incidentally. We removed the tumor completely by transoral laser surgery and no recurrence was found after 7 months. The case of solitary neurofibroma arising from laryngeal surface of epiglottis has not been reported in Korea. We report this case regarding the diagnosis and treatment with review of literatures. Key words: Plexiform neurofibroma ㆍ Larynx ㆍ Epiglottis ㆍ CO2 laser 핵심용어: 망상형 ì‹ ê²½ì„¬ìœ ì¢ ã† 후두 ㆍ 후두개 ㆍ ë ˆì´ì €ìˆ˜ìˆ

English

Neurofibromatosis originates from the Schwann cells of the nerve sheath of the nerve which is benign in nature. In von Recklinghausen's disease multiple masses arises all over the body. It may occur from internal nerves sporadically. These are the white glistening round or lobulated masses arise in the course of nerves involved. Neurofibroma of larynx is seldom reported. Women are more affected than men in the ratio 3:2. Hoarseness is the earliest and common symptom, the patient present with. If the mass increase in the course of time, the dyspnoea, later dysphagia symptoms wills result. The arytenoid is the common site of origin, later aryepiglottic fold, false vocal cord, ventricle of the larynx. As the tumor is benign in nature, total excision of tumor is recommended. In the literature, very few cases of laryngeal involvement are reported. This article presents a case report of neurofibroma of larynx involving false vocal cord and ventricle presented submucosally with symptom of hoarseness, primarily with mild stridor on exertion. In this large endolaryngeal mass, laryngofissure surgical approach is selected as a choice.

Neurofibromatosis of the larynx causing stridor and sleep apnea

BackgroundNeurofibromatosis type 1 can rarely present in the larynx. Patients typically do not present with complete obstructive symptoms, but partial obstruction and stridor. We review our health centers’ case series of two patients, the first of whom presented with persistent sleep apnea post tonsillectomy and adenoidectomy, and the second who presented with noisy breathing. Additionally, we will review the literature on the management and treatment options for children with this rare clinical entity. MethodsRetrospective case review. Case report & resultsA two-year old male underwent a sleep endoscopy following persistent evidence of obstructive sleep apnea on polysomnography after initial tonsillectomy and adenoidectomy. Family elicited concerns about noisy breathing at night and an accompanying video documented stridor while sleeping during the monitored polysomnography. Flexible fiberoptic laryngoscopy in the operating room revealed what appeared to be a cystic mass along the right aryepiglottic fold causing deviation of the laryngeal introitus towards the contralateral side. Subsequent direct laryngoscopy and excisional biopsy revealed pathology results consistent with a plexiform neurofibroma.A six-month-old patient with stertor and stridor was found to have a laryngeal mass, subglottic stensosis, and progressive airway obstruction due to plexiform neurofirboma in the supraglottis, subglottis, and trachea.We present a series of two patients incidentally diagnosed with neurofibromatosis type 1 by way of a laryngeal neurofibroma and review the literature on management options. Both patients were found to have accompanying café au lait spots. Both patients required tracheostomy for airway management, and one was successfully decannulated. ConclusionLaryngeal neurofibroma is a rare anomaly that can manifest with airway obstruction. Both patients presented here subsequently were noted to have café au lait spots on physical examination. The Otolaryngologist should be reminded of this anomaly when evaluating a child with evidence of a submucosal laryngeal mass. We present our series including that of a patient whose diagnosis was prompted by persistent sleep apnea following adenotonsillectomy tonsillectomy and a patient with airway obstruction and subglottic stenosis due to a neurofibroma. The treatment of choice is complete excision of the neurofibroma while maintaining functionality of the larynx. This can lead to successful decannulation.

Nerwiakowłókniak krtani jako przyczyna duszności – opis przypadku i przegląd literatury

Neurofibromas of the larynx occur extremely rarely and present in 0.5% of laryngeal benign tumors. Schwannomas occur more frequently (1,5% of laryngeal benign tumors)and its extracranial prevalence in head and neck is between 25–45%.Laryngeal neurofibroma usually develops in places rich in terminal nerve plexuses. The nerve of origin is usually an internal branch of the superior laryngeal nerve. Hence, aryepiglottic folds and arytenoids are most commonly affected.Symptoms of neurofibroma are determined by the location. Hoarseness, dysarthria, dyspnea, globus sensation are most commonly associated with neurofibroma. The other symptoms include: cough, obstructive sleep apnea, dyspnea on exertion. Because of its benign character, neurofibroma can remain asymptomatic for years.In computed tomography scans neurofibroma shows as a hypodense heterogenic tumor, well-delineated, with smooth edges. Heterogenic and medium enhancement is observed after contrast administration. A “target” appearance is typical for the plexus subtype of neurofibroma, this consists of low signal intensity centrally, high signal peripherally and lobulated pattern.The choice of treatment for laryngeal neurofibroma depends on its location, size and associated symptoms. Currently there are no specific guidelines with regard to the management of laryngeal neurofibromas. Tumor resection is possible by both endoscopic or external approach. Endoscopic CO2 laser excision is recommended for patients who present small localized lesions, well visible in direct laryngoscopy.

Laryngeal myxoid neurofibroma misdiagnosed as myxoma: a case report

Neurogenic tumours constitute a very small percentage of all neoplastic lesions of the head and neck region. Neurogenic tumors of the larynx are extremely rare. Two types of neurogenic tumors must be distinguished: Schwannomas and neurofibromas. Neurofibromas arise from the sheath of nerve fibers and are characterized by prominent collagen production and trapped nerve fibers. In the larynx, the most common sites of origin are arytenoids and aryepiglottic folds. Because of benign nature of this tumor, conservative surgery is recommended as the management of choice. Only few cases of laryngeal myxoid neurofibroma have been reported in literature and this article presents one case of laryngeal myxoid neurofibroma, which at first was misdiagnosed as myxoma, with brief review of literatures.

Recurrent Laryngeal Neurofibroma Unrelated to NF Type 1

Objective: Although rare, neurofibromas arising from the recurrent laryngeal nerve in neurofibromatosis type I patients have been cited in the literature. However, recurrent laryngeal neurofibromas unrelated to this inherited disorder have not been reported. We describe a new entity of a recurrent laryngeal neurofibroma arising at the level of the glottis. Method: A case report and literature review is presented. A patient with progressive dysphonia, dysphagia, and mild dyspnea was referred to our cancer center in November 2011 for assessment. Flexible laryngoscopy revealed right vocal cord paresis and a submucosal mass causing medial displacement of the right arytenoid. Results: CT imaging revealed a well-defined, nonenhancing mass at the level of the posterior glottis. Transoral endoscopic surgical excision of the mass was performed. Pathology revealed a benign peripheral nerve sheath tumor consistent with a neurofibroma. The patient had resolution of his dyspnea and achieved significant improvement of his voice with a 6-week course of speech therapy, despite postoperative right vocal cord paralysis. Conclusion: Isolated neurofibromas of the recurrent laryngeal nerve, without the appearance of other characteristic features of von Recklinhausen disease have not been reported. Although benign, early recognition and surgical excision of these lesions is necessary to prevent airway obstruction, dysphagia, and aspiration.

A Case of Laryngeal Neurofibroma with Sleep Obstructive Apnea

' disease), which is characterized by cafe-au-lait spots and cutaneous neurofibromas. Neurofibromas in the laryngeal area are extremely rare. Most patients with a laryngeal neurofibroma present with dyspnea, dysphagia, stridor, or hoarseness, depending on the location and size of the tumor. We present a case of a laryngeal neurofibroma in a boy with neurofibromatosis type 1. A 30-month-old boy with neurofibromatosis presented to our hospital with respiratory difficulty and obstructive sleep apnea. Magnetic resonance imaging demonstrated a round mass over the left carotid space, and its location made surgical resection impossible. Hence, tracheostomy was performed to maintain airway patency. The patient s symptoms improved after tracheostomy. Long ' -term follow-up is es- sential owing to the possibility of recurrence. The patient has thus far shown no obstructive airway symptoms for one year after closure of the tracheostomy. (Pediatr Allergy Respir Dis(Korea) 2011;21:131-136)

Supraglottic Neurofibroma Presenting as Obstructive Sleep Apnea: A Case Report

Introduction: Sleep disordered breathing and obstructive sleep apnea are commonly encountered in the pediatric population. In many cases it is secondary to adenotonsillar hypertrophy. The differential diagnosis is long, and as clinicians we have to determine who needs to undergo fiberoptic laryngoscopy in the clinic setting and other extensive testing. The differential diagnosis of a supraglottic mass in sleep disordered breathing needs to be considered in patients who fail conservative treatmenteven a diagnosis as rare as neurofibroma. Materials and Methods: A case report. A 16 yearold female presented with snoring, obstructive sleep apnea (OSA), and “asthma” secondary to a neurofibroma of the aryepiglottic fold without associated NF1. Conclusions: This is the first case of an adolescent aged patient of African descent diagnosed with a solitary laryngeal neurofibroma. Her prolonged treatment with asthma medications and attempt to treat her sleep apnea with CPAP emphasize the importance of considering obstructive lesions in these situations.

Management of advanced laryngeal and hypopharyngeal plexiform neurofibroma in adults

Laryngeal neurofibromas are rare. The plexiform subtype is less common and often presents during childhood in association with neurofibromatosis type I. Because it is unencapsulated and more aggressive, plexiform neurofibroma presents a management dilemma. Imaging, particularly magnetic resonance imaging, can aid in diagnosing neurofibroma, differentiating the nonplexiform and plexiform subtypes, and planning the subsequent operative approach. The importance of conservative surgery to relieve symptoms, but preserve laryngopharyngeal function, and close follow-up are stressed. We report 2 adult cases of plexiform neurofibroma involving the larynx and hypopharynx and discuss the controversies in management.

経口的に切除し得た小児巨大喉頭神経線維腫の1例

A neurofibroma of the larynx is a rare disease that usually involves the arytenoids and the aryepiglottic fold. Treatment options for laryngeal neurofibroma are either the transoral or pharyngotomy approaches. The transoral approach had been indicated only for small tumor due to its limited operative field and difficulty in managing massive hemorrhaging. New device, such as the laser, distending laryngoscopy, videoendoscopy and special forceps enable us to resect larger tumors of the larynx.Since laryngeal neurofibromas are benign tumor, transoral surgery could be the treatment of choice in order to reduce surgical invasion. We reported on a case of a huge laryngeal neurofibroma resected by a novel transoral approach. A five-year-old girl complaining of stridor and dyspnea and suffering from type 1 neurofibromatosis (von Recklinghausen's disease) was referred to our clinic. A tracheostomy and biopsy had already been performed at another institution. A flexible laryngoscopic examination revealed a 35 by 25 mm smooth pinkish mass covered by mucosa on the left arytenoid which was obstructing the airway. The left vocal cord was fixed and the right one was restricted by mass effect. The tumor was completely resected by the transoral approach. No intraoperaive and postoperative complications were noted. Oral intake commenced two days after the operation without swallowing disturbance. There was no recurrence of the tumor during a one year follow up and her tracheostoma was closed successfully.

The Biology and Management of Laryngeal Neurofibroma

To review the presentation of laryngeal neurofibroma, including its association with neurofibromatosis types 1 and 2, and present guidelines for its management. Retrospective study. Five pediatric patients with laryngeal neurofibroma, 4 girls (80%) and 1 boy (20%), were treated at a tertiary pediatric medical center from 1973 through 2003. Recorded data included age at initial presentation, sex, symptoms, significant medical and family history, preoperative evaluation, location of the tumor, surgical procedure, complications, outcome, and recurrence. The 5 patients presented with stridor and cafe-au-lait spots at or shortly after birth. All patients were diagnosed as having neurofibromatosis type 1 by the established criteria. Studies evaluating the disease processes included plain radiography, computerized tomography, magnetic resonance imaging, barium swallow, and laryngoscopy and bronchoscopy under anesthesia. Pathologic examination of biopsy specimens from all patients showed neurofibromas with plexiform and/or diffuse features. Treatments included tracheotomy (n = 4), carbon dioxide laser excision (n = 4), modified neck dissection (n = 3), partial pharyngectomy (n = 1), supraglottic laryngectomy (n = 1), and endoscopic hemilaryngectomy (n = 1). Three patients were successfully decannulated. Follow-up ranged from 1 to 15 years. One patient was lost to follow-up. No evidence of malignant degeneration was noted. Neurofibroma of the larynx is a rare condition that should be considered in the differential diagnosis of children with a submucosal laryngeal mass. In our series, all patients had associated neurofibromatosis type 1. Complete surgical excision is the treatment of choice in cases of localized small lesions. To prevent debilitating outcomes due to aggressive surgery, minimally invasive procedures (partial excision via endoscopic approach) may be preferable for larger lesions that infiltrate the surrounding vital structures. Long-term follow up of these patients is essential owing to the possibility of malignant transformation.