case report
A 19yr male patient came to the Department of Oral medicine, Meenakshi Ammal Dental college with a chief complaint of swelling in the upper front tooth region for the past 4 years. Swelling was painless and was present on the gingiva for the past 4 yes with no increase in size Air change in color. Apart from the oral cavity, no other similar swellings were found on the rest of the body. Family history was not relevant.
On local examination, a firm sessile swelling measuring 10mm * 4mm was present interdentally between 23 and 24. Labially it extended from the attached gingiva, involved the marginal gingiva passed interdentally to the palatal region of 23 and 24.23 and 24 were noticed to be separated by a distance of 5 mm due to the swelling, The color and the surface of the swelling were apparently normal and the swilling was not tender. The regional lymph nodes were not palpable. I.O.P.A showed a crestal bone loss between 23 and 24.
Differential Diagnosis of fibroma and fibro-epithelial polyp of the gingiva were made. Since it was a small solitary intra Oral lesion excisional biopsy was planned Under LA. the complete lesion along with 1mm of normal tissue was excised. The excised tissue was preserved in formalin and labeled and sent for histopathological examination.
Hematoloxylin and eosin stained biopsy sections showed bundles of nerves arranged in fascicles with thin wavy nuclei. Delicate intertwining collaeen fibrils avd fibroblasts were seen in the adjacent connective tissue few lymphocytes were scattered among the cells. Blood vessels were prominent Tumor was not encapsulated
Immunohistochemical staining for S-100 protein was positive, supporting an origin from the nerve sheath element.
With the above clinical. Histological and immunostainiug findings, the diagnosis of solitary neurolihroma of the gingiva was made.
discussion
Neurofibromas are benign, expansile swellings, contained within the epineurium. The exact etiology of solitary neurofibroma is unknown. It is thought to be originated front a mixture of cell types, including the schwann cells and the perineural fibroblass. These are most common in young adults(20-30yrs).
Oral neurofibromas usually present as submucosal, nontender uninflammed discrete masses that range from few millimeters to several centimeters. Tumors tend to grow slowly and patients are usually asyntptennatie. Common sites of the oral solitary netu'ofibromas include tongue (26%). buccal mucosa (8%).gingiva(2%) alveolar ridge (2%) labial mucosa(8%) palate (8%). nasopharynx, paranasalsinuses, Iarynx, floor of the mouth and salivary gland. Tumors may also arise within the bone. Neurolibromas on or adjacent to the cranial nerves, ailed the motor functions of the Facial and hypoglossal nerves, and sensory functions of Irigeminal nerve
Lesions affecting the gingivo-dento-alveolar complex can displace and cause the mobility of the erupted teeth. If they occur during primary or mixed dentition stages, They may lead to the displacement or impaction of erupting permanent teeth .The lesion is rarely painful until it is secondarily traunmatized The nodule is readily movable. if situated immediately beneath the mucosa hit it is less. when located on the deeper structures.
Radiographic features are seen mainly when it arises centrally within the bone. It may produce a well demarcated or poorly defined unilocular or multilocular radiolucency. It is also interesting to note that tf the lesion occurs in the mandible, the radiographic features such as enlargement of mandibular foramen. enlargement and branching of mandibular canal, increased bone density, concavity of the medial surface of ramus, and increased dimension ofcorunoid notch arc evident.
Clinically, the other diseases concerned of similar presentation arc granular cell tumor. fibroma, scar tissue. neurilemmoma, leiomyoma. and rhabdomyoma. A definitive diagnosis can only be rendered, after an incisional or an excisional biopsy followed by histopathological examination.
During the excision of the lesion, tissues from the marginal and attached gingiva were included in the biopsy specimen as inflammatory changes in the gingival margin tend to obscure any alterations that may he produced by systemic disturbances. Inclusion of attached gingiva in which the effect of local irritants is less likely to he present offers an opportunity to investigale tissue changes that may be produced by systemic disturbances.
Microscopically solitary neurofibroma often presents as a well circumscribed lesion especially. When proliferation occurs within the perincurium of the involved nerve. Tumors that proliferate outside the perineuium may not appear well demarcated and tend to blend with adjacent connective tissue,
Three histological forms of solitary neurofibroma can be diagnosed based on the content of cells, niticin and collagen. Common and characteristic form present. as interlacing bundles of elongated cells having wavy dark stained nuclei. Cells are intimately associated with wire like strands of collagen and small to moderate amounts of mucoid material separates the collagen. Stroma is dotted with occasional mast cells, lymphocytes and rarely xanthoma cells.
The second form shows a less frequent presentation. This type of neurofibroma is very cellular and consists of schwann cells set in a store unilunn collagen matrix devoid of mucosubstances. In such tenors, cells may be arranged in fascicles whorls or even a storiform pattern. In certain respects, these cellular ncurofibromas may resemble Antoni-A areas of nerilemmoma but unlike in neurilenunoma they are not encapsulated and lack of clear partition into two zones. Moreover small nearites call usually be demonstrated through out these tumours.
The third form is very rare and these tumours are Highly and easily confused with myxomas (usually occurs on extremeties).This hypocellular neoplastri. contain pools of acid mucopolysaccharides with widely spaced schwann cells. In contrast to myxoma. cells of neufibroma usually have greater degree of orientation. Vascularity is also morc prominent. With a careful searching, features of specific differentiation (eg. Wagner-Meissner bodies) may be found.
Immuno-histochemically the tumor cells show a scattered positive reaction for S-100 protein.
The lesion if large may infiltrate major/minor vessels causing weaking of vessels walls resulting in excessive haemorrhage during surgical excision. Therefore it is advisable to investigate with angiography prior to excision. Multiple lesions in the oral cavity can be managed using carbon dioxide laser which is an alternative mode of treatment and if present with in the skin, the cosmetic problem can be managed by dermabrasion
Treatment for solitary neurofibroma is local excision and recurrence is rare. Prognosis fur solitary neurofibroma is good when compared to the neurofibrometosis-I. since malignant transformation is common in neurofibromatosis and rare in solitary neurofibroma.
Malignant transformation of neurofibromas into neurogenic-sarcomas is seen in 5 to I5% of the patients with neurofibromatosis-I.Solitary neurofibroma may be seen as an initial presentation of neurofibromatosis-I in young patients with or without relevant family history (So. every individual presenting with asolitary ncurofibroma, below' 20yrs of age should be referred for genetic studies to rule out the possibility of neurofibronmatosis-1)
refference
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