Hostname: page-component-586b7cd67f-dlnhk Total loading time: 0 Render date: 2024-11-20T15:29:29.342Z Has data issue: false hasContentIssue false
  • English
  • Français

Diagnostic and treatment challenges in neurofibroma concerning the external nose

Published online by Cambridge University Press:  15 December 2021

N Shakrawal Open the ORCID record for N Shakrawal [Opens in a new window] ,
M Chouhan  and
B Solanki
N Shakrawal*
Affiliation:
Department of Otorhinolaryngology and Head–Neck Surgery, Dr Sampurnanand Medical College, Jodhpur, India
M Chouhan
Affiliation:
Department of Otorhinolaryngology and Head–Neck Surgery, Dr Sampurnanand Medical College, Jodhpur, India
B Solanki
Affiliation:
Department of Otorhinolaryngology and Head–Neck Surgery, Dr Sampurnanand Medical College, Jodhpur, India
*
Author for correspondence: Dr Neha Shakrawal, Department of Otorhinolaryngology and Head–Neck Surgery, Dr Sampurnanand Medical College, Jodhpur, Rajasthan, India342005 Fax: +91 291 274 0531 E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background

This paper discusses clinical features and management aspects of an extremely rare entity, neurofibroma of the external nose.

Methods

Database searches were performed using PubMed, Embase and Google Scholar to identify previously published articles.

Results

Twelve articles comprising 13 patients with neurofibroma of the external nose were included. The mean age of presentation was 31 years. Sixty-nine per cent of patients were diagnosed at final histopathology. External approach rhinoplasty was performed in 76.9 per cent of patients, while the intranasal approach was used in 15.3 per cent of patients. There was a 15.3 per cent association with neurofibromatosis type 1. Recurrence was noted in 23 per cent of patients.

Conclusion

It may be challenging to diagnose this entity clinically because of its rarity and striking features on histopathology. Neurofibroma of the external nose should be kept in mind as a differential diagnosis for any soft progressive swelling over the nose. Management requires complete excision, with cosmesis and restoration of functions.

Keywords

NeurofibromaExternal NoseNeurofibromatosisNeurofibromatosis Type 1
Type
Review Article
Information
The Journal of Laryngology & Otology , Volume 136 , Issue 6 , June 2022 , pp. 473 - 478
Copyright
Copyright © The Author(s), 2021. Published by Cambridge University Press on behalf of J.L.O. (1984) LIMITED

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

Dr N Shakrawal takes responsibility for the integrity of the content of the paper

References

Wilson, J, McLaren, K, McIntyre, M, von Haacke, N, Maran, A. Nerve sheath tumors of the head and neck. Ear Nose Throat J 1988;67:103–10Google ScholarPubMed
Ferner, R, O'Doherty, M. Neurofibroma and schwannoma. Curr Opin Neurol 2000;15:679–84CrossRefGoogle Scholar
Rameh, C, Husseini, S, Tawil, A, Fuleihan, N, Hadi, U. Solitary plexiform neurofibroma of the nasal tip: case report and review of the literature. Int J Pediatr Otorhinolaryngol Extra 2007;2:116–19CrossRefGoogle Scholar
Batsakis, JG. Tumors of the Peripheral Nervous System. Tumors of the Head and Neck. Baltimore: Williams and Wilkins, 1979;313–33Google Scholar
New, G, Devine, K. Neurogenic tumors of nose and throat. Arch Otolaryngol 1947;46:163–79CrossRefGoogle ScholarPubMed
Das Gupta, TD, Brasfield, R, Strong, E, Hajdu, S. Benign solitary schwannoma (neurilemomas). Cancer 1969;24:355–603.0.CO;2-2>CrossRefGoogle Scholar
Ferner, RE, Huson, SM, Thomas, N, Moss, C, Willshaw, H, Evans, DG et al. Guidelines for the diagnosis and management of individuals with neurofibromatosis 1. J Med Genet 2007;44:81–8CrossRefGoogle ScholarPubMed
Upadhyaya, M, Cooper, DN, eds. Neurofibromatosis Type 1. Berlin: Springer-Verlag, 2012CrossRefGoogle ScholarPubMed
Friedman, JM. Neurofibromatosis 1. In: Pagon, RA, Adam, MP, Ardinger, HH et al. , eds. GeneReviews [internet]. Seattle: University of Washington, 1998Google ScholarPubMed
National Center for Biotechnology Information (NCBI). GTR: Genetic Testing Registry. In: http://www.ncbi.nlm.nih.gov/gtr/tests [2 February 2021]Google Scholar
Spurlock, G, Bennett, E, Chuzhanova, N, Thomas, N, Jim, HP, Side, L et al. SPRED1 mutations (Legius syndrome): another clinically useful genotype for dissecting the neurofibromatosis type 1 phenotype. J Med Genet 2009;46:431–7CrossRefGoogle ScholarPubMed
Lee, JH, Bae, JH, Kim, KS. A case of solitary neurofibroma of the nasal dorsum: resection using an external rhinoplasty approach. Eur Arch Otorhinolaryngol 2005;262:813–15CrossRefGoogle ScholarPubMed
Chander, V, Surya Rao, RVM, Sekhar, G, Raja, A, Sridevi, M. Diffuse neurofibroma of nose associated with neurofibromatosis type 1: a rare case report with review of literature. Indian J Dermatol 2015;60:573–7Google ScholarPubMed
Kim, KS. Complete removal of external nasal neurofibroma by endonasal approach. J Craniofac Surg 2015;26:e562–4CrossRefGoogle ScholarPubMed
Nataraj, R, Jagade, M, Parelkar, K, Hanawte, R, Singhal, A, Rengaraja, D et al. Plexiform neurofibroma of nasal tip. Int J Otolaryngol Head Neck Surg 2015;4:423–8CrossRefGoogle Scholar
Trieu, D, Drosou, A, Schwartz, MR, Goldberg, LH. Myxoid neurofibroma treated with Mohs micrographic surgery. Letters and communications. Dermatol Surg 2015;41:166–8CrossRefGoogle Scholar
Chintale, SG, Bhise, SD, Kirdak, V, Shaikh, K. A rare case of neurofibroma of the nasal tip and dorsum of nose. Otolaryngology Online Journal 2016;6:110–14Google Scholar
Gupta, N, Kaur, R, Rai, A, Wadhwa, N, Singh, PP. Neurofibroma of the cheek and nasal dorsum: a case report and a mini review. Int J Otorhinolaryngol Clin 2016;8:62–4Google Scholar
Min, HJ, Kim, KS. Removal of solitary neurofibroma of external nose by intranasal approach. Eur Ann Otorhinolaryngol Head Neck Dis 2017;134:273–5CrossRefGoogle ScholarPubMed
Gómez, OJ, Bernal, M, Martínez, DC. Neurofibroma of the nasal ala: pediatric nasal reconstruction. J Craniofac Surg 2018;29:e314–15CrossRefGoogle ScholarPubMed
Bartolo, A, Spiteri, MA, Ayoub, O. Trouble at the tip: a case report on neurofibroma of nasal tip and review of literature. The Rhinologist 2018;1:26–31Google Scholar
Fakhim, SA, Rezaeian, S, Feizi, HH, Behzad, MN, Piri, R. A case of solitary neurofibroma in outer surface of nose. J Anal Res Clin Med 2016;4:233–7CrossRefGoogle Scholar
Kannan, N, Patil, R, Pattipati, S. Neurofibroma of lip: report of a rare case. J Indian Acad Oral Med Radiol 2010;22:113–15CrossRefGoogle Scholar
Abell, MR, Hart, WR, Olson, JR. Tumors of the peripheral nervous system. Hum Pathol 1970;1:503–51Google ScholarPubMed
Lassmann, H, Jurecka, W, Lassmann, G, Gebhart, W, Matras, H, Watzek, G. Different types of benign nerve sheath tumors. Light microscopy, electron microscopy and autoradiography. Virchows Arch A Pathol Anat Histol 1977;375:197–210CrossRefGoogle ScholarPubMed
Skovronsky, DM, Oberholtzer, JC. Pathologic classification of peripheral nerve tumors. Neurosurg Clin N Am 2004;15:157–66CrossRefGoogle ScholarPubMed
Verocay, J. Multiple tumors as a systemic disease of the nervous system. In: Festschrift fur Chiari, Vienna and Leipzig, 1908;378Google Scholar
Berlucchi, M, Piazza, C, Blanzuoli, L, Battaglia, G, Nicolai, P. Schwannoma of the nasal septum: a case report with review of the literature. Eur Arch Otorhinolaryngol 2000;257:402–5CrossRefGoogle ScholarPubMed
Perzin, KH, Panyu, H, Wechter, S. Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx. A clinicopathologic study. XII: Schwann cell tumors (neurilemoma, neurofibroma, malignant schwannoma). Cancer 1982;50:2193–2023.0.CO;2-0>CrossRefGoogle ScholarPubMed
Gnepp, D. Diagnostic Surgical Pathology of the Head and Neck. Philadelphia: WB Saunders, 2001;196–7Google Scholar
Hernández-Martín, A, Duat-Rodríguez, A. An update on neurofibromatosis type 1: not just café-au-lait spots, freckling, and neurofibromas. An update. Part I. Dermatological clinical criteria diagnostic of the disease. Actas Dermosifiliogr 2016;107:454–64CrossRefGoogle ScholarPubMed
Lemmerling, M, Moerman, M, Govaere, F, Praet, M, Kunnen, M, Vermeersch, H. Schwannoma of the tip of the nose: MRI. Neuroradiology 1998;40:264–6CrossRefGoogle ScholarPubMed
Hassell, DS, Bancroft, LW, Kransdorf, MJ, Peterson, JJ, Berquist, TH, Murphey, MD et al. Imaging appearance of diffuse neurofibroma. AJR Am J Roentgenol 2008;190:582–8CrossRefGoogle ScholarPubMed
Wasa, J, Nishida, Y, Tsukushi, S, Shido, Y, Sugiura, H, Nakashima, H et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol 2010;194:1568–74CrossRefGoogle ScholarPubMed
Chen, W, Jia, JW, Wang, JR. Soft tissue diffuse neurofibromas: sonographic findings. J Ultrasound Med 2007;26:513–18CrossRefGoogle ScholarPubMed
Campora, DE, Radii, M, Campora, DL. Neurogenic tumours of head and neck in children. Int J Pediatr Otolaryngol 1999;49:S231–3CrossRefGoogle ScholarPubMed
Glimer-Hill, HS, Kline, DG. Neurogenic tumours of cervical vagus nerve report of 4 cases and review of literature. Neurosurgery 2000;46:1498–503CrossRefGoogle Scholar
Miettinen, M, Weiss, SW. Soft tissue tumors. In: Damjanov, I, Linder, J, eds. Anderson's Pathology, 10th edn. St Louis: Mosby, 1995;2507–10Google Scholar
Pablo, MM, Diego, HM. Solitary neurofibroma of the inferior nasal turbinate. Auris Nasus Larynx 1998;25:329–31Google Scholar
Hillstrom, RP, Zarbo, RJ, Jacobs, JR. Nerve sheath tumors of the paranasal sinuses: electron microscopy and histopathologic diagnosis. Otolaryngol Head Neck Surg 1990;102:257–63CrossRefGoogle ScholarPubMed
Macias, VC, Rafael, M, Fernandes, C, Rosa, JC. Diffuse neurofibroma--an uncommon cause of alopecia. An Bras Dermatol 2013;88:166–9CrossRefGoogle ScholarPubMed
Donnelly, M, Al-Sader, M, Blayney, A. View from beneath: pathology in focus, benign nasal schwannoma. J Laryngol Otol 1992;106:1011–15CrossRefGoogle Scholar
Megahed, M. Histopathological variants of neurofibroma. A study of 114 lesions. Am J Dermatopathol 1994;16:486–95CrossRefGoogle ScholarPubMed
Ravi, AK, Ram, R, Lindberg, MR, Pandey, T. Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum. Skeletal Radiol 2014;43:1773–8CrossRefGoogle ScholarPubMed
Marocchio, LS, Oliveira, DT, Pereira, MC, Soares, CT, Fleury, RN. Sporadic and multiple neurofibromas in the head and neck region: a retrospective study of 33 years. Clin Oral Investig 2007;11:165–9CrossRefGoogle ScholarPubMed
da Rosa, MR, Ribeiro, AL, de Menezes, SA, Pinheiro, JJ, Alves-Junior, SM. Solitary giant neurofibroma of the mental nerve: a trauma-related lesion? J Craniofac Surg 2013;24:e247–51CrossRefGoogle ScholarPubMed
Fisher, ER, Vuzevski, VD. Cytogenesis of schwannoma (neurilemoma), neurofibroma, dermatofibroma and dermatofibrosarcoma, as revealed by electron microscopy. Am J Clin Pathol 1968;49:141–5CrossRefGoogle Scholar
Hirao, M, Gushiken, T, Imokawa, H, Kawai, S, Inaba, H, Tsukuda, M. Solitary neurofibroma of the nasal cavity: resection with endoscopic surgery. J Laryngol Otol 2001;115:1012–14CrossRefGoogle ScholarPubMed
Wise, JB, Cryer, JE, Belasco, JB, Jacobs, I, Elden, L. Management of head and neck plexiform neurofibromas in pediatric patients with neurofibromatosis type 1. Arch Otolaryngol Head Neck Surg 2005;131:712–18CrossRefGoogle ScholarPubMed
Bernthal, NM, Putnam, A, Jones, KB, Viskochil, D, Randall, RL. The effect of surgical margins on outcomes for low grade MPNSTs and atypical neurofibroma. J Surg Oncol 2014;110:813–16CrossRefGoogle ScholarPubMed
Cook, JL. Nasal reconstruction. In: Rohrer, TE, Cook, JL, Migden, MR, Nguyen, TH, Mellette, JR, eds. Flaps and Grafts in Dermatologic Surgery. New York: Elsevier, 2008;191–215Google Scholar
Burget, GC, Menick, FJ. Repair of small surface defects. In: Burget, GC, Menick, FJ, eds. Aesthetic Reconstruction of the Nose. St Louis: Mosby, 1994;117–56Google Scholar
Burget, GC, Menick, FJ. Aesthetics, visual perception, and surgical judgment. In: Burget, GC, Menick, FJ, eds. Aesthetic Reconstruction of the Nose. St Louis: Mosby, 1994;1–55Google Scholar
Fattahi, TT. An overview of facial aesthetic units. J Oral Maxillofac Surg 2003;61:1207–11CrossRefGoogle ScholarPubMed
Bloom, J, Antunes, M, Becker, D. Anatomy, physiology, and general concepts in nasal reconstruction. Facial Plast Surg Clin North Am 2011;19:1–11CrossRefGoogle ScholarPubMed
Staser, K, Yang, FC, Clapp, DW. Pathogenesis of plexiform neurofibroma: tumor-stromal/hematopoietic interactions in tumor progression. Annu Rev Pathol 2012;7:469–95CrossRefGoogle ScholarPubMed
Kebudi, R, Cakir, FB, Gorgun, O. Interferon-α for unresectable progressive and symptomatic plexiform neurofibromas. J Pediatr Hematol Oncol 2013;35:e115–17CrossRefGoogle ScholarPubMed
Weiss, S, Goldblum, J. Enzinger's and Weiss's Soft Tissue Tumors, 4th edn. St Louis: Mosby, 2001;1146–67Google Scholar