|Year : 2019 | Volume
| Issue : 1 | Page : 65-67
Shredded carrots in the larynx: A rare case of neurofibroma of vocal cord
Feba Ann Sunny, J Jishana, Paresh Pramod Naik, Mary Kurien
Department of ENT, Pondicherry Institute of Medical Sciences, Puducherry, India
|Date of Submission||26-Oct-2018|
|Date of Acceptance||25-Nov-2018|
|Date of Web Publication||19-Jun-2019|
Feba Ann Sunny
Department of ENT, Pondicherry Institute of Medical Sciences, Ganapathichettikulam, Puducherry - 605 014
Source of Support: None, Conflict of Interest: None
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 <30 cases of laryngeal NF since its first description in 1950, and vocal cord manifestations without systemic features of NF are still rarer. Most of the cases were in children or associated with NF-1. Here, we present the case of a middle-aged gentleman with isolated neurofibroma of the vocal cord.
Keywords: Hoarseness, microlaryngeal surgery, neurofibromatosis, vocal cord polyp
|How to cite this article:|
Sunny FA, Jishana J, Naik PP, Kurien M. Shredded carrots in the larynx: A rare case of neurofibroma of vocal cord. J Curr Res Sci Med 2019;5:65-7
|How to cite this URL:|
Sunny FA, Jishana J, Naik PP, Kurien M. Shredded carrots in the larynx: A rare case of neurofibroma of vocal cord. J Curr Res Sci Med [serial online] 2019 [cited 2020 Feb 29];5:65-7. Available from: http://www.jcrsmed.org/text.asp?2019/5/1/65/260635
| Introduction|| |
Neurofibromatosis (NF) is a common genetic disorder that causes tumors to form on nerve tissue commonly in the brain, spinal cord, and nerves. Isolated laryngeal neurofibromas are extremely rare and account for only 0.03%–0.1% of benign tumors of the larynx. Among the 30 cases reported, most of these lesions had been reported in association with von Recklinghausen disease. Our patient is unique in that he does not have any features or family history of von Recklinghausen disease.
| Case Report|| |
A 49-year-old male presented to the Department of Otorhinolaryngology with a history of hoarseness of voice, associated with cough for 3 years. Hoarseness of voice was insidious in onset and initially intermittent and later became persistent. He also had associated persistent dry cough. He had no complaints of difficulty in breathing or noisy breathing. He had no complaints of throat pain or difficulty in swallowing. He was an accountant by profession and a chronic smoker and nonalcoholic.
General examination was normal and local examination of oral cavity and oropharynx was within normal limits. Video laryngoscopy was done which revealed a smooth, broad-based sessile mass of 1 cm × 1 cm at the anterior two-third of left vocal cord; both vocal cords were mobile and glottic chink was adequate [Figure 1].
A clinical diagnosis of vocal cord polyp or cyst was made, and he was taken up for microlaryngoscopy under general anesthesia, and the mass was excised using cold steel method and sent for histopathological examination.
Biopsy report revealed a well-circumscribed benign tumor with variable myxoid material, and randomly oriented thin-spindled cells which were bland with wavy, hyperchromatic nuclei and thin and thick collagen strands also called “shredded carrot appearance.” There was no dysplasia or evidence of malignancy as seen in [Figure 2].
These features were suggestive of NF and a diagnosis of laryngeal neurofibroma was made. Postoperatively, the patient was given absolute voice rest for 2 weeks and speech therapy; his voice improved, and a repeat video laryngoscopy revealed normal vocal cords with no residual tissue [Figure 3]. He was followed up for 2 months postoperatively and did not have any recurrence.
| Discussion|| |
Fifty percent of patients presenting to an otolaryngologist with complaints of voice change have benign vocal cord lesions among which the most common is vocal nodules. The most common risk factors associated with benign vocal cord lesions are voice overuse, smoking and alcoholism, acid reflux, systemic illnesses, insufficient fluid intake, and allergies.
Neurogenic tumors of vocal cord are quite rare and account for 0.0%–1.5% of all benign laryngeal tumors. These commonly include schwannomas and neurofibromas. Among them, neurofibromas are less common.
NF is autosomal dominant and results in tumors along nerves, spinal cord, and brain. Among the eight subtypes, two are best described. NF-1 causes peripheral neurofibromas characterized by cafe au lait spots, freckles, and Lisch nodules, and NF-2 causes vestibular schwannomas, meningiomas, and spinal ependymomas. Neurofibroma of the larynx wasfirst described in 1950 by Hollinger, and most of those cases were in pediatric populations or associated with NF. Within the larynx, they are most commonly seen in the supraglottis such as arytenoids and aryepiglottic folds wherein they are postulated to arise from superior laryngeal nerve and subglottis where they arise from recurrent laryngeal nerve.,
Ever since itsfirst description, <30 cases of isolated laryngeal neurofibromas have been reported with few cases of isolated vocal cord lesions presenting as tumors on vocal cords in adults with no systemic features of Von Recklinghausen's.
Studies reveal that they are slightly more common in women and children and have a variable presentation. Children may present with stridor while adults usually present with change in voice or dysphagia.
Histopathological features typical of neurofibroma are well–circumscribed, benign tumor composed of bundles of spindle cells with bland nuclear morphology admixed with fibroblasts and collagen fibers. All cases of vocal cord neurofibromas were diagnosed postoperatively after a biopsy, and though this is a rare entity, neurogenic tumors need to be considered in the differential diagnosis of smooth well-defined, mucosal mass in the vocal cord. Otolaryngologists need to be aware that laryngeal NF may not be associated with NF-1 or NF-2. The risk of malignant transformation of these tumors is <10%.
However, as complete treatment can be achieved by surgical excision of the mass, it is essential to be aware of this entity and plan for excision biopsy of all smooth mucosal masses of vocal cords.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
This paper was supported by the entire Department of Otorhinolaryngology, Pondicherry Institute of Medical Sciences. I would like to acknowledge Dr. Jishana and Dr. Aneesh Basheer for their valuable input and suggestions. Furthermore, Dr. Mary Kurien who is a constant support and moderated this paper and Dr. Paresh Naik for his comments which helped to improve the manuscript.
I would also like to thank Dr. Kiran from the Department of Pathology for his help in histopathology without whom the diagnosis and pathological features would be incomplete.
Financial support and sponsorship
This paper was supported by the entire Department of Otorhinolaryngology, Pondicherry Institute of Medical Sciences.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]