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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 8  |  Issue : 4  |  Page : 175-178

Solitary diffuse neurofibroma of buccal mucosa


Department of Oral Pathology and Microbiology, Government Dental College and Hospital, Nagpur; MUHS, Nashik, Maharashtra, India

Date of Web Publication14-Dec-2017

Correspondence Address:
Rekha Bhaskar Chaudhari
Department of Oral Pathology and Microbiology, Government Dental College and Hospital, Nagpur, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/srmjrds.srmjrds_46_17

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  Abstract 


Neurofibroma (NF) is a benign peripheral nerve sheath tumor, arising from the mixture of Schwann cells and perineural fibroblasts. Diffuse NF (DNF) is an uncommon but distinct histological variant of NF. It typically involves skin and subcutaneous tissues, occurring primarily in children and young adults. DNF is seen either as a sporadic (solitary) lesion or as part of neurofibromatosis, usually type 1-NF1 (von Recklinghausen's disease of skin). Involvement of oral cavity is rare with isolated reports in the literature. Clinically, it appears as pedunculated or sessile nodular mass, exhibiting slow growth and mostly without pain. Histologically it differs from the conventional NF in that it has a very uniform matrix of fine-fibrillary collagen. Sheets or cords of neoplastic Schwann cells in the matrix are less elongated and has short fusiform or round contour. A characteristic feature is the presence of clusters of laminated Wagner–Meissner-like bodies, scattered throughout tumor mass, which distinguishes DNF from NF. A case of solitary DNF of buccal mucosa in a 23-year-old female patient is presented.

Keywords: Neurofibromatosis type-1, solitary diffuse neurofibroma, Wagner–Meissner bodies


How to cite this article:
Chaudhari RB. Solitary diffuse neurofibroma of buccal mucosa. SRM J Res Dent Sci 2017;8:175-8

How to cite this URL:
Chaudhari RB. Solitary diffuse neurofibroma of buccal mucosa. SRM J Res Dent Sci [serial online] 2017 [cited 2022 May 25];8:175-8. Available from: https://www.srmjrds.in/text.asp?2017/8/4/175/220808




  Introduction Top


Neurofibromas (NF) are benign, slow-growing nerve sheath neoplasms, which affect head and neck region only rarely.[1] It may occur either as a solitary lesion or as a part of the generalized syndrome of neurofibromatosis (usually NF type-1) NF-1, also called von Recklinghausen's disease of the skin.[2] The WHO has subdivided NF into two broad categories: dermal and plexiform. Dermal NF arises from single peripheral nerve, whereas plexiform NF is associated with multiple nerve bundles.[3] Based on morphological features, NF is classified into major (plexiform, diffuse, and Pacinian) and minor variants (epitheliod, cellular, myxoid, glandular, etc.).[4] Diffuse NF (DNF) is an unusual but distinctive form of NF that occurs principally in children and young adults.[3],[5] Clinically, this lesion is most common in the head and neck region. At least, 10% of patients with this lesion also have neurofibromatosis.[2] In the oral cavity, DNF may present as pedunculated or sessile nodular superficially located, painless, and slow-growing mass.[4],[6] Histologically, it is composed of Schwann cells which are less elongated and have short fusiform contour, scattered in a fibrillary collagen matrix, containing clusters of Wagner–Meissner-like structures.[2],[4],[5],[6] Intraoral DNF, not related to NF-1 are relatively uncommon. A close perusal of the literature on this lesion revealed only two reports of its occurrence in the oral cavity.

Here, a rare case of oral DNF of buccal mucosa, not associated with NF-1, in a 23-year-old female patient is presented.


  Case Report Top


A 23-year-old female patient reported to the hospital with a complaint of painless, small, pedunculated, slow-growing nodular mass, located on the right buccal mucosa; no prior and/or family history for tumors and cutaneous spots. It had been present for 5 months. It exhibited slightly irregular surface with color similar to that of adjacent mucosa and measured approximately 1 cm × 1 cm. It was nontender and firm in consistency. Medical and dental history was insignificant. Family history was nonrelevant. General physical examination was otherwise unremarkable. Presumptive clinical diagnosis of benign soft-tissue tumor was made – fibroma and lipoma. Complete hemogram was within normal limits. An excisional biopsy was performed. There was no recurrence after 1-year follow-up.

Gross examination of the specimen – single excised specimen measured 1 cm × 1 cm × 0.9 cm, greyish with irregular surface [Figure 1]. Cut section was homogenous, grayish-white.
Figure 1: Grayish-yellow tumor mass with irregular surface

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H and E section revealed diffuse replacement of mucosa and submucosa by tumor mass [Figure 2] which was unencapsulated and composed of short fusiform or round cells with twisted nuclei and fibroblasts arranged randomly, within uniform fibrillary collagen matrix with numerous pale eosinophilic globules containing parallel slits [Figure 3]. These globules comprised of narrow elongated cells stacked in a lamellar arrangement, identical to tactile corpuscles like Wagner–Meissner bodies. The nuclei of the lamellar cells were located at the periphery of the corpuscles. Mature entrapped fat tissue, interspersed among tumor cells was present [Figure 4]. Overlying surface epithelium was thin and stretched out with flattening of rete ridges [Figure 1]. On the basis of these histological features, the lesion was diagnosed as “solitary DNF.”
Figure 2: Diffuse replacement of mucosa by tumor mass (H and E, ×40)

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Figure 3: Diffuse neurofibromas composed of randomly arranged, spindle to ovoid cells with wavy nuclei within fibrillar collagen matrix, and numerous clusters of pale eosinophilic globules (arrow) of Wagner–Meissner-like bodies (H and E, ×100)

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Figure 4: Entrapped adipose tissue (thin arrow) and whorled laminated structures resembling Wagner-Meissner corpuscles (thick arrow), comprising of narrow elongated cells with nuclei located at periphery (H and E, ×400)

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  Discussion Top


NFs are composed of Schwann cells, perineural cells, fibroblasts within collagenous or myxoid matrix.[1] These lesions are considered to be the most common type of peripheral neoplasms seen on the skin, but are uncommon intraoral neoplasms. DNF is an unusual but distinctive variant of NF that is recognized as a separate entity from 1997.[7] Isolated or solitary NF may be noted as soft-tissue growth when smaller peripheral nerve is involved. It is believed that frequency of oral solitary DNF, not associated with NF-1 is low. NF-1 is the most common form of neurofibromatosis. In general, DNF is closely related with NF-1 which is an autosomal dominant disease, affecting 1 in 3500 individuals worldwide. It is caused by a mutation in NF-1 gene, located on chromosome 17 q 11.2 that encodes the protein neurofibromin. Patients with NF-1 may develop tumor at any site in the body including skin, internal nerve trunk, and viscera. It seems likely that loss of function alteration in the NF-1 gene play a role in both NF-1 associated and sporadic NFs.[2],[7] In the present case, there was no evidence of various manifestations such as cafe-au-lait spots, Lisch nodules, and axillary freckling.[2] NF-1 was ruled out due to the absence of these indicators. A comparative review of literature is presented in [Table 1].
Table 1: Comparative review of literature

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As the name implies, DNF is ill-defined and spreads extensively along connective tissue septae and between fat cells.[2] It typically involves skin and subcutaneous tissues, occurring primarily in children and young adults. Histologically, it is characterized by diffuse replacement of dermis by interlacing bundles of spindle (Schwann cells) cells with round- or fusiform-buckled nuclei and eosinophilic cytoplasm within loose fine fibrillary collagen matrix and many clusters of Wagner–Meissner-like bodies.[2],[8] Despite infiltrative growth, it does not destroy but rather envelopes normal structures such as adipose tissue, muscle fibers, and skin adnexal structures. Histological features in the present case were identical to that described by Enzinger and Weiss [2] and consistent with those noted in earlier reports.[4],[5],[6],[7],[9] The most prominent finding, in this case, was whorled lamellated structures resembling Wagner–Meissner corpuscles, considered to be characteristic of DNF. These are virtually seen in almost all cases in contrast to NFs.[8] The Wagner–Meissner corpuscles are touch receptors of skin. They are located in dermal papillae, especially on the palmer and planter surfaces.[10] Tactile corpuscle-like bodies are microscopic Schwannian structures that simulate the superficial mechanoreceptors of peripheral nervous system. They have been described nearly exclusively in peripheral nerve sheath tumors, namely, DNF.[11] Touch corpuscles are composed of closely piled laminar cells. These are characteristic but are not always present.[10] Immunohistochemically, neuron-specific enolase, vimentin, and protein S-100 could be demonstrated in the tactile corpuscle, as reported in earlier cases.[4],[6],[7] On the basis of histopathological and immunohistochemical profile, it is speculated that aberrant transition of neoplastic Schwann cells and unusual proliferation of Meisner bodies might be related to tumor growth.[4]

DNF differs from the conventional NF in that it has spindle to ovoid Schwann cells with twisted nuclei embedded in a uniform matrix of fine fibrillary collagen and mature fat tissue. In addition clusters of elongated cells, stacked in a lamellar arrangement similar to the tactile corpuscles like Wagner–Meissner body has a peculiar feature. However, its emergence and significance is still enigmatic.[3],[7] Ohno et al.[4] did not label their case as DNF, because of location of the lesion on gingiva which lacks abundant adipose tissue. However, entrapment of submucosal fat tissue was noted in the present case which supports the diagnosis of DNF. Conventional histological analysis is conclusive when classic microscopic features are observed.

The case is reported for its rarity and unique presentation-illustrative of its indistinctive clinical characteristic and highlighting histopathological features which revealed diffuse replacement of mucosa and submucosa by tumor mass. Neurogenic tumors are uncommon in the oral cavity. Any patient with a lesion diagnosed as DNF should be evaluated for NF-1. In the differential diagnosis of small, oral solitary soft-tissue tumors, NF (histological variants) should be kept in mind. The case described underlines the importance of histopathological examination of such lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Richards D. Neurofibroma of the oral cavity. Br J Oral Surg 1983;21:36-43.  Back to cited text no. 1
[PUBMED]    
2.
Enzinger FM, Weiss SW. Soft Tissue Tumors. 3rd ed. St. Louis, Toronto, London: The C.V. Mosby Company; 1995. p. 610-4.  Back to cited text no. 2
    
3.
Von Deimling A, Foster R, Krone W. Neurofibromatosis type -1. In: Kleihues P, Cavenee WK, editors. WHO Classification of Tumors. Pathology & Genetics of Tumors of Nervous System. Lyon, France: IARC; 2000. p. 216-8.  Back to cited text no. 3
    
4.
Ohno J, Iwahashi T, Ozasa R, Okamura K, Taniguchi K. Solitary neurofibroma of the gingiva with prominent differentiation of meissner bodies: A case report. Diagn Pathol 2010;5:61.  Back to cited text no. 4
[PUBMED]    
5.
Chander V, Rao RS, Sekhar G, Raja A, Sridevi M. Recurrent diffuse neurofibroma of nose associated with neurofibromatosis type 1: A rare case report with review of literature. Indian J Dermatol 2015;60:573-7.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Chiu YW, Lui MT, Kao SY, Chen YW. Solitary diffuse neurofibroma of tongue – A case report. Taiwan J Oral Maxillofac Surg 2012;23:187-96.  Back to cited text no. 6
    
7.
Dikov T, Ivanova V, Karaivanov M, Yardanova I. Solitary neurofibroma featuring prominent Wagner-Meissner bodies and floret-like multinucleated giant cells: A case Report. Sci Technol Med 2013;3:73-6.  Back to cited text no. 7
    
8.
Macias VC, Rafael M, Fernandes C, Rosa JC. Diffuse neurofibroma – An uncommon cause of alopecia. An Bras Dermatol 2013;88:166-9.  Back to cited text no. 8
[PUBMED]    
9.
Nair PA, Kora RK. Solitary neurofibroma over cheek showing Wagner-Meissner bodies. Egypt J Dermatol Venerol 2017;37:26-7.  Back to cited text no. 9
  [Full text]  
10.
van Zuuren EJ, Posma AN. Diffuse neurofibroma on the lower back. J Am Acad Dermatol 2003;48:938-40.  Back to cited text no. 10
[PUBMED]    
11.
Celeiro-Muñoz C, Huebner TA, Robertson SA, Pittman ME, Singhi AD, Arnold CA, et al. Tactile corpuscle-like bodies in gastrointestinal-type mucosa: A Case series. Am J Surg Pathol 2015;39:1668-72.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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