Abstract
The neurofibroma occurs as isolated or multiple lesions frequently associated with neurofibromatosis type 1 (NF-1). The aim of this study was to analyze the clinical and histopathological features of neurofibromas, particularly the plexiform variant, in the skin and oral mucosa, discussing their pathogenesis as well as clinical management of isolated lesion unassociated with NF1. The clinicopathologic features of 66 neurofibromas in the head and neck region diagnosed at the pathology laboratories of the Bauru Dentistry School and Lauro de Souza Lima Research Institute from 1970 to 2003 were reviewed. The clinical data, therapy, and follow-up information were obtained from the medical records. The results showed a high frequency of cutaneous lesions (81.8%) occurring mainly in females older than 40 years. Isolated neurofibromas were found in 51.2% of patients, and multiple lesions were often associated with the NF-1. The histopathological analysis demonstrated that diffused neurofibromas occur more frequently than the plexiform type. However, one case of plexiform neurofibroma was detected in the oral mucosa as an isolated lesion non-associated with the NF-1. The indolent clinical behavior of isolated neurofibromas in the head and neck region and the absence of NF-1 association reinforce that sporadic lesion could be hyperplastic or hamartomatous rather than neoplastic in nature.
Similar content being viewed by others
References
Alatli C et al (1996) Solitary plexiform neurofibroma of the oral cavity: case report. J Maxillofac Surg 25:379–380
Albernaz MS, Pratt MF, Garen PD (1990) Intraparotid facial nerve neurofibroma: a case report and literature review. Otolaryngol Head Neck Surg 102:413–415
Aloi FG, Massobrio R (1989) Solitary plexiform neurofibroma. Dermatológica 179:84–86
Bocca M et al (1990) Neurofibroma della mandibola: revisione della letteratura e prezentaziona di um caso clinico. Minerva Stomatol 39:675–677
Cherrick HM, Eversole LR (1971) Benign neural sheath neoplasm of the oral cavity. J Oral Surg 32:900–901
Ellis GL, Auclair PL, Gnepp DR (1991) Benign mesenchymal neoplasms. In: Ellis GL, Auclair PL, Gnepp DR (eds) Surgical pathology of the salivary glands. Saunders, Philadephia, pp 496–497
Fisher DA, Chu P, McCalamont T (1997) Solitary plexiform neurofibroma is not pathognomonic of von Recklinghausen s neurofibromatosis: a report of a case. Int J Dermatol 36:439–442
Friedman JM (1999) Epidemiology of neurofibromatosis type I. Am J Med Genet 89:1–6
Goldblum JR, Weiss SW (2001) Benign tumors of peripheral nerves. In: Goldblum JR, Weiss SW (eds) Soft tissue tumors. Mosby, Missouri, pp 1111–1146
Gorlin RJ, Cohen MM, Levin LS (1990) Hamartoneoplastic syndromes. In: Gorlin RJ, Cohen MM, Levin LS (eds) Syndromes of the head and neck. Oxford University Press, Oxford, pp 392–399
Hadju SI (1993) Peripheral nerve sheath tumors. Cancer 72:3549–3552
Happle R (2001) Large plexiform neurofibromas may be explained as a type 2 segmental manifestation of neurofibromatosis 1. Am J Med Genet 98:363–364
Liapis H, Dehner LP, Gutman DH (1999) Neurofibroma and cellular neurofibroma with atypia: a report of 14 tumors. Am J Surg Pathol 23:1156–1163
Lin V, Daniel S, Forte V (2004) Is a plexiform neurofibroma pathognomonic of neurofibromatosis type I? Laryngoscope 114:1410–1414
Loufty WG et al (1977) Trigeminal nerve neurofibroma: case report. J Oral Maxillofac Surg 35:733–738
Needle MN et al (1994) Prognostic signs in the surgical management of plexiform neurofibroma: the Children’s Hospital of Philadelphia experience, 1974–1994. J Pediatr 131:678–682
(1988) Neurofibromatosis: conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 45:575–578
Nicol JW, Yardley MPJ, Parker AJ (1994) Plexiform neurofibroma: an unusual cause of neck lump in a child. Br J Clin Pract 48:110–111
Packer RJ, Gutmann DH, Rubenstein A (2002) Plexiform neurofibromas in NF1: toward biologic based therapy. Neurology 58:1461–1470
Peetermans JFE et al (1991) Neurofibroma of the vagus nerve in the head and neck: a case report. Head Neck 13:56–61
Perry A et al (2001) NF1 deletions in S-100 protein-positive and negative cells of sporadic and neurofibromatosis 1 (NF1)-associated plexiform neurofibromas and malignant peripheral nerve sheath tumors. Am J Pathol 159:57–61
Pollack RP (1990) Neurofibroma of the palatal mucosa: case report. J Periodontol 61:456–458
Prescott GH, White E (1970) Solitary, central neurofibroma of the mandible: report of case and review of the literature. J Oral Surg 28:305–309
Richards D (1983) Neurofibroma of the oral cavity. Br J Oral Surg 21:36–43
Ruggieri M, Huson SM (2001) The clinical and diagnostic implications of mosaicism in the neurofibromatoses. Neurology 56:1433–1443
Rutkowski JL et al (2000) Genetic and cellular defects contributing to benign tumor formation in neurofibromatosis type 1. Hum Mol Genet 9:1059–1066
Scheithauer BW, Woodruff JM, Erlandson RA (1999) Tumors of the peripheral nervous system. Atlas of tumor pathology. Armed Forces Institute of Pathology, Washington
Shack RB, Reilley AF, Lynch JB (1985) Neurofibromas of head and neck. South Med J 78:801–804
Sharawy A, Springer J (1968) Central neurofibroma occurring in the mandible. Oral Surg Oral Med Oral Pathol 25:817–821
Sharma SC, Srinivasan S (1998) Isolated plexiform neurofibroma of tongue and oropharynx: a rare manifestation of von Recklinghausen’s disease. J Otolaryngol 27:81–84
Shimoyama T et al (2002) Solitary neurofibroma of the oral mucosa: a previously undescribed variant of neurofibroma. J Oral Sci 44:59–63
Skouteris CA, Sotereanos G (1988) Solitary neurofibroma of the maxilla: report of a case. J Oral Maxillofac Surg 46:701–705
Stewart A, Bailey MW (1992) Neurofibroma of the inferior alveolar nerve: diagnostic and management difficulties. Br J Oral Maxillofac Surg 30:56–58
Tsutsumi T, Tsuneyuki O, Komatsuzaki A (1996) Solitary plexiform neurofibroma of the submandibular salivary gland. J Laryngol Otol 110:1173–1175
Weber A, Montandon C, Robson CD (2000) Neurogenic tumors of the neck. Radiol Clin North Am 38:1077–1090
Weedon D (2002) Neural and neuroendocrine tumors. In: Weedon D (ed) Skin pathology. Churchill Livingstone, London, pp 983–986
Weiss SW et al (1994) Neural tumours. In: Weiss SW et al (eds) Histological typing of soft tissue tumours. Springer, Berlin Heidelberg New York, pp 38–39
Wright BA, Jackson J (1980) Neural tumors of the oral cavity. Oral Surg 49:509–522
Acknowledgment
The authors thank CAPES (Coordenação de Aperfeiçoamento de Pessoal de Nível Superior) for supporting this study.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Marocchio, L.S., Oliveira, D.T., Pereira, M.C. et al. Sporadic and multiple neurofibromas in the head and neck region: a retrospective study of 33 years. Clin Oral Invest 11, 165–169 (2007). https://doi.org/10.1007/s00784-006-0096-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00784-006-0096-6