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SOLITARY NEUROFIBROMA OF TONGUE

Haritosh K Velankar, Ka Pradhan
Lecturer; HOD, Department of ENT, Rajawadi Hospital, Padmashri DY Patil Medical College, Ghatkopar.


Neurofibromatas, are observed in oral environment, especially in tongue. Neurofibroma may occur as an isolated lesion or as part of Von Reclinghausen’s neurofibromatosis. They may develop along any of the cranial or peripheral nerves as well as the nerve roots. Treatment of solitary neurofibroma, not associated with any syndrome is surgical excision, with minimal risk of recurrence. Oral lesions seldom transform into sarcoma but may become large enough to interfere with proper function.

INTRODUCTION

Neurofibromas may develop along any of the cranial or peripheral nerves as well as the nerve roots. Often multiple tumours are present, in Von Recklinghausen’s disease, but can occur as solitary lesion also. One such case of solitary neurofibroma in the tongue is reported.

CASE REPORT

14 year old boy came with history of swelling of the tongue, which was noticed since 5 years. It was very small when first noticed, but gradually increased to its present size, which is about 3 cms by 3 cms (Fig. 1).

Fig 1
Fig 1. Swollen tongue Lt side


H/o increased difficulty in swallowing, due to restricted tongue movements; hot potatoe speech, which was gradually worsening; reccurent tongue bites, due to the bulky size of the tongue.

No h/o any other such swelling on the body.

There was no family history suggestive of multiple swellings on the body.

O/E 3 cms by 3 cms swelling was seen to the left of midline of the tongue. On palpation, it was a firm swelling. Transillumination test was negative. Mucosa over the swelling could be lifted up from the dorsum of the tongue. Mobility of the tongue was restricted towards left side.

FNAC done, was inconclusive, as the aspirate was blood stained.

Local excision of the tumour was done under general aneasthesia, by making an incision on left lateral border of the tongue (Figs 2 & 3).

Fig 2
Fig 2. Intra-operative look of tumour


Fig 3
Fig 3. Tumour after removal measuring 3cm x 3cm

Histopathology report was suggestive of neurofibroma.

DISCUSSION

The neurofibroma is the most common type of peripheral nerve sheath neoplasm. It can be seen as a solitary lesion or can be seen in multiple neurofibromatosis (Von Recklinghausen’s disease).

These tumours are derived from schwann cells, fibroblast and occasionally perineural cells. Microscopic cellular pattern of neurofibroma shows, delicate spindle cells with elongated slender, and sometimes wavy nuclei arranged in a loose, disorganised pattern. Fibrous stands are numerous within the lesions, which stain positively for reticulin and collagen.

This tumour is typically observed in young adults and presents as a slow growing, soft, painless lesion. Although this tumour is most commonly observed on the skin, it can be observed intraorally over the tongue and buccal mucosa which are the most common intraoral sites.

The oral or pharyngeal neurofibromatas are usually diagnosed in teenagers and young adults, although at all ages they may be present. There is no gender predilection and most of them arise from the tongue, buccal and labial mucosa. The lesion presents as a slowly enlarging, painless, soft nodule which is readily movable if situated immediately beneath the mucosa but is less so whenlocated in deeper tissues. In Von Recklinghausen’s disease, involvement of the tongue is seen in almost more than 50% cases.

Treatment of solitary neurofibroma not associated with any syndrome is surgical excision, with minimal risk of recurrence. Oral lesions seldom transform into sarcoma but may become large enough to interfere with proper function.

REFERENCES

1.Neville BW, Hann J, Narang R, Garren P. Oral neurofibrosarcoma associated with neuro firbomatosis. Oral Surg Oral Med Oral Pathol 1991; 72 : 546-61.

2.Alatil C, Oncer B, Uner M, Erseven G. Solitary plexiform neurofibroma of oral cavity - a case report. Internat J Oral Maxillofascial Surg 1996; 25 : 378-80.

3.Sohata JS, Vishwanathan A, Nayak DR, Hazarika P. Neurofibroma of Tongue. Internat J Paed Otorhinolaryngol 1996; 34 : 153-57.

4.Anderson WAD, John M Kissane. Pathology 7th edition. 2 : 1242.



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