neurofibroma is a benign peripheral nerve sheath tumor arising from the schwann cells and perineural fibroblasts .
oral involvement is noted in 3.4 - 92% of adults and 40% of children with nf1 .
a 28-year - old male patient reported [ figure 1 ] with the chief complaint of a painless swelling in the right upper back tooth region since 3 years .
history revealed swelling was of insidious in onset , which increased to attain the present size .
family history revealed the patient 's father [ figure 3 ] also had multiple swellings all over the body and face . on general examination ,
multiple swellings all over the trunk region and arms were observed [ figure 2 ] .
solitary well - defined oval shaped swelling [ firm in consistency and nontender on palpation figure 4 ] approximately 4 cm  3 cm was present in the right maxillary posterior region extending from distal aspect of maxillary first premolar to mesial aspect of third molar .
. however , multiple , discrete , sessile cutaneous masses which had started appearing since childhood were observed .
histopathological examination of incisional biopsy of the oral lesion showed spindle cells with elongated wavy nuclei .
the lesional area was separated from the overlying epithelium by mature fibrous connective tissue [ figure 5 ] .
cutaneous neurofibroma over the trunk patient 's father with cutaneous neurofibroma gingival neurofibroma h and e stained section of the lesion under , 40
neurofibromatosis type 1 is due to alteration of nf1 gene , which is a tumor suppressor gene located in the long arm of chromosome 17 .
the clinical criterion for the diagnosis of nf1 encompasses presence of six or more cafau lait spots ( > 5 mm in children or > 15 mm in adults ) , two or more cutaneous or subcutaneous neurofibromas or one plexiform neurofibroma , freckles in the axilla or groin optic glioma , two or more lisch nodules ( pigmented hamartomas of the iris ) , bony lesion with sphenoid wing dysplasia or bowing of the long bones with or without pseudoarthrosis , and/or first degree relative with nf1 .
other features that have been described to occur variably in individuals with nf1 include short stature , large head size , failure to gain weight , precocious puberty , vascular disease including childhood hypertension , neural problems secondary to the spinal cord involvement , headaches , cognitive problems , stroke , brain tumors , and rarely tumors such as pheochromocytoma , and juvenile chronic myeloid leukemia .
common sites of the oral solitary neurofibromas include tongue ( 26% ) , buccal mucosa ( 8% ) , alveolar ridge ( 2% ) , labial mucosa ( 8% ) , palate ( 8% ) , gingiva ( 2% ) , nasopharynx , paranasal sinuses , larynx , floor of the mouth and salivary gland .
this patient reported with gingival localization of neurofibroma , which is extremely rare and unique .
gingival neurofibromas can cause periodontal disease , as tissue growth is an obstacle in carrying out routine oral hygiene measures .
oral radiographic findings unique to nf include lengthening , narrowing and rarefaction of coronoid and articular process , deepening of sigmoid notch , an enlarged mandibular canal , mandibular foramen and mental foramen .
other findings are shortening of the ramus , notching of the inferior border of the mandible . in the case
usually , the prognosis for solitary neurofibroma is extremely good , with only rare instances of recurrence .
the present case was kept under observation and was recalled every 3 months for a period of 1 year and showed no signs of recurrence .
malignant transformation of neurofibromas with nf1 into neurogenic sarcomas bears a very bad prognosis with a 5 years survival rate of just 15% .
long - term review of patients and genetic counseling is recommended owing to the likelihood ( 50% ) of vertical transmission .
it is imperative for the general physicians and dermatologists to be aware of the oral manifestations of nf1 considering the risk of malignant transformation and the poor prognosis .
 neurofibroma is a benign peripheral nerve sheath tumor and is the most frequent tumor of neural origin . 
 its presence is one of the clinical criteria for the diagnosis of neurofibromatosis type 1 ( nf1 ; a common hereditary disease occurring in one out of every 3000 births ) . 
 the diagnosis can sometimes be made at birth , while in others the diagnosis is made later in life after the appearance of additional clinical criteria . 
 majority of the solitary neurofibromas are sporadic , while a few are associated with nf1 syndrome . oral hard and soft tissue 
 are affected by the tumor ; however , the tongue is the most affected site . 
 gingival neurofibroma is an uncommon oral manifestation of nf . here , we report a rare case of gingival neurofibroma in nf1 patient . one of the most feared complications of nf1 is its transformation into neurofibrosarcoma , which bears a very poor prognosis . 
 treatment of neurofibroma is surgical resection . 
INTRODUCTION
CASE REPORT
DISCUSSION