|Year : 2013 | Volume
| Issue : 4 | Page : 510-513
A silent tumor of the gingiva: An unusual case report and surgical management with 1 year follow-up
Babita Pawar, Avneesh Tejnani, Neha Kaur Sodhi, Gowri Pendyala
Department of Periodontology, Pravara Institute of Medical Sciences, Rural Dental College, Rahata, Ahmednagar, Loni, Maharashtra, India
|Date of Submission||01-Dec-2011|
|Date of Acceptance||30-Apr-2013|
|Date of Web Publication||17-Sep-2013|
Department of Periodontology, Pravara Institute of Medical Sciences, Rural Dental College, Rahata, Ahmednagar, Loni - 413 736, Maharashtra
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Neurofibroma is a benign tumor of the peripheral nerve sheath characterized by the proliferation of Schwann's cells, perineural cells, and endoneurial fibroblasts. Here, we present a case of unique variation in the observed characteristics of a neurofibroma, with no relation to neurofibromatosis (NF) type-1 or von Recklinghausen disease of the skin. A swelling was observed in the right maxillary gingiva in relation with 14-16 regions of a 25-year-old female patient. The lesion was smooth, with sessile base, painless, non-ulcerated, and with normal color. An excisional biopsy of the lesion was performed, and histologically the diagnosis was confirmed. The patient remains uneventful after 1 year of clinical follow-up. Oral cavity involvement by a solitary neurofibroma in patients with no other signs of NF is uncommon and the gingival involvement is very rare.
Keywords: Diagnosis, gingiva, mangement, neurofibroma, solitary
|How to cite this article:|
Pawar B, Tejnani A, Sodhi NK, Pendyala G. A silent tumor of the gingiva: An unusual case report and surgical management with 1 year follow-up. J Indian Soc Periodontol 2013;17:510-3
|How to cite this URL:|
Pawar B, Tejnani A, Sodhi NK, Pendyala G. A silent tumor of the gingiva: An unusual case report and surgical management with 1 year follow-up. J Indian Soc Periodontol [serial online] 2013 [cited 2021 Sep 28];17:510-3. Available from: https://www.jisponline.com/text.asp?2013/17/4/510/118326
| Introduction|| |
Neurofibroma is a benign tumor of the peripheral nerve sheath characterized by the proliferation of Schwann's cells, perineural cells, and endoneural fibroblasts. Localized or solitary neurofibromas develop along peripheral nerve as a focal mass with well-defined margins, but are not encapsulated. The majority of isolated or solitary neurofibromas are sporadic, and a small minority may be associated with the neurofibromatosis (NF)-1 syndrome.
Herewith, we present a rare case report on solitary neurofibroma of the gingiva. There are neither clinical findings nor personal/family history of NF type 1 so that the lesion seems to be independent of these syndromes.
| Case Report|| |
A 25-year-old female patient was referred to the department of Periodontology with a gingival swelling in the upper right back region. The lesion was an incidental finding by her dentist at a routine dental check-up. A history suggested the swelling had been present for approximately 6 months. This swelling had not caused any pain and no other symptoms were reported by patient. The medical history was non-contributory and the patient did not smoke or drink alcohol.
On local examination, the lesion was smooth, firm with sessile base, non-ulcerated, painless, with normal color measuring 4 mm × 10 mm, extending from the mesial of maxillary right first premolar to the mesial of maxillary right first molar involving the marginal, papillary, and attached gingiva [Figure 1]. Intra oral periapical radiograph showed a mild crestal bone loss between 13 and 14 and between 14 and 15 [Figure 2].
|Figure 1: Pre‑operative view showing the lesion present between 14‑16 region|
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Based on texture, differential diagnosis of granuloma or a possible fibroma was made. A punch biopsy of the lesion was conducted under local anesthesia and the specimen was submitted to routine histopathological analysis [Figure 3] and [Figure 4].
Histopathologically, hematoloxylin, and eosin stained biopsy sections showed spindle-shaped cells, with fusiform or wavy comma-shaped nuclei distributed on a background of delicate connective tissue matrix [Figure 5].
Having confirmed the occurrence of a neurofibrona, a complete exploration was made. There was no relevant family history and no evidence of NF, such as café-au-lait macules or freckling in the axillary and inguinal regions. No lisch nodules were detected during an ophthalmological study. The patient showed normal physical and mental development. With the above clinical and histological findings, the diagnosis of solitary neurofibroma of the gingiva was made and complete excision of the lesion was planned.
Under local anesthesia complete excision was achieved using no. 15 blade. The growth was carefully dissected and separated from the underlying tissues [Figure 6] and [Figure 7]. The excised specimen was almost 0.5 cm × 1 cm in size [Figure 8]. The bed of the lesion was examined for remnants, hemostasis was achieved and Coe pack was placed [Figure 9] and [Figure 10]. The post-operative period was uneventful. The patient was kept on a constant follow-up [Figure 11] and after 12 months, there were no signs or symptoms derived from the lesion [Figure 12].
|Figure 9: Bed of the lesion examined for remnants and hemostasis achieved|
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| Discussion|| |
Oral neurofibroma is an uncommon benign tumor of the oral cavity derived from the cells that constitute the nerve sheath. Neurofibroma occurs as a solitary lesion or as part of the generalized syndrome of NF (von Recklinghausen disease of the skin). The solitary form does not differ from the disseminated or multiple forms of the disease except that systemic and hereditary factors present in the disseminated form are absent in the solitary type 1-3. 
The cell of origin for neurofibroma has not been definitively identified. Some believe it arises from the Schwann cell. Perineural fibroblasts are neuroectodermal tissue cells that synthesize collagen and create a network that envelops the individual axis cylinders of the nerves with their associated Schwann cells. Some investigators believe that perineural fibroblasts give rise to the neurofibroma. 
The cause of solitary neurofibroma is unknown. However, NF is inherited as an autosomal dominant trait with a high degree of penetrance, but variable expressivity. As many as 50% of cases are reported to be the result of spontaneous mutation. 
Most neurofibromas in the head and neck region tend to be solitary tumors,  but the occurrence of isolated solitary neurofibroma affecting peripheral nerves without any other stigmata of NF-1, as in the case reported, is unusual in the oral cavity with gingival involvement being all the more rare.
Little information is available regarding the relative frequency of solitary oral neurofibromas. Common sites of the oral solitary neurofibromas include tongue (26%), buccal mucosa (8%), alveolar ridge (2%), labial mucosa (8%), palate (8%), gingiva (2%), nasopharynx, paranasalsinuses, Iarynx, floor of the mouth and salivary gland. Tumors may also arise within the bone. 
Oral neurofibromas usually present as non-tender, uninflammed, submucosal discrete masses that range from few millimeters to several centimeters. Tumors tend to grow slowly and patients are usually asymptomatic, but depending on the locality (e.g., tongue, palate), they may be traumatized and give rise to symptoms. There is no predilection towards race or sex.  As in the present case the lesion was an incidental finding by the patient's dentist during a routine dental check-up.
Manifestations of NF specific to the oral cavity include enlarged fungiform papillae on the dorsum of the tongue and diffuse enlargement of the gingiva. In patients with mandibular involvement, enlargement of the inferior alveolar canal in the mandible and a flaring of the inferior alveolar foramen have been reported. However, in the present case, there was a localized enlargement giving it an appearance of a granuloma or a fibroma.
Solitary oral neurofibromas are most frequently treated by surgical excision, depending on the extent and the site. However, for neurofibromas associated with NF, surgical removal is attempted only for functional or cosmetic reasons.  Usually, the prognosis for solitary neurofibroma is extremely good, with only rare instances of recurrence after resection. The present case was kept under observation and was recalled every 3 months up to 1 year and showed no signs of recurrence.
Malignant transformation of neurofibromas into neurogenic-sarcomas is seen in 5-15% of patients with NF-I. Solitary neurofibroma may be seen as an initial presentation of NF-I in young patients with or without relevant family history. So every individual presenting with a recurrence of solitary neurofibroma, below 20 years of age should be referred for genetic studies to rule out the possibility of NF-1. ,
Distinguishing between isolated neurofibromas and those associated with NF-1 is important because the treatment and prognosis differ greatly. Furthermore, neurofibromas associated with NF-1 are more likely to recur or undergo malignant transformation. ,
| Conclusion|| |
The present case is reported because of the unusual mode of presentation, and it also shows how large lesions can be tolerated and goes undetected in the oral cavity if asymptomatic. Although these are benign tumors and have a fairly good prognosis and since, they have a propensity for malignant transformation and also chances of progressing into NF, these lesions must be monitored carefully and treated meticulously. Even though, they are a rare lesion in the oral cavity, solitary neurofibromas must be considered in the list for differential diagnosis in cases of intraoral swellings and intraosseous lesions of the jaws.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]