|Year : 2015 | Volume
| Issue : 2 | Page : 106-108
Intraoral desmoplastic fibroblastoma: A rare presentation
Santosh R Patil
Department of Oral Radiology and Medicine, College of Dentistry, Al Jouf University, Skaka, Aljouf, Kingdom of Saudi Arabia
|Date of Web Publication||14-May-2015|
Santosh R Patil
College of Dentistry, AlJouf University, Skaka, Aljouf
Kingdom of Saudi Arabia
Source of Support: None, Conflict of Interest: None
Desmoplastic fibroblastoma is an uncommon fibrous soft tissue tumor. Only a few cases have been reported in the literature till date. These slow growing, painless mass is most commonly seen in the upper extremities, but rarely in the head and neck region. This article presents a case report of a 45-year-old female who presented with a similar soft tissue mass in the oral cavity. The mass was excised and sent for histopathological evaluation, which confirmed the diagnosis as desmoplastic fibroblastoma.
Keywords: Desmoplastic fibroblastoma, gingiva, pre-maxillary region, soft tissue tumor
|How to cite this article:|
Patil SR. Intraoral desmoplastic fibroblastoma: A rare presentation. J Sci Soc 2015;42:106-8
| Introduction|| |
Desmoplastic fibroblastoma (DF) or collagenous fibroma was first described by Evans in 1995.  It is an uncommon, benign, slow growing, fibroblastic, soft tissue lesion which occurs in the subcutaneous tissue and skeletal muscle in the adults. , Nielsen et al., proposed the name "collagenous fibroma" based on the histopathological features and its benign nature.  It is seen commonly as subcutaneous or intramuscular lesions in the 5 th -6 th decade of life, with a strong predilection for males. The lesion shows wide anatomic variations.  It is a rarity in the oral cavity and commonly involves the hard palate, tongue and/or alveolar bone.  This article presents a case report of a slow growing, painful, benign swelling in the pre-maxillary region of a 45-year-old female patient.
| Case report|| |
A 45-year old female reported to us with a chief complaint of swelling and pain over the maxillary anterior gingiva since 2 years. The swelling was slow growing and the pain was intermittent and localized. Medical history and family history of the patient were not contributory. Intraoral examination revealed a well defined swelling in the pre-maxillary region measuring 2 × 1.5 cm extending from the mesial aspect of 11 to mesial aspect of 23. Superioinferiorly extending from mucogingival groove till the occlusal plane. The surface appeared smooth with few ulcerations [Figure 1]. Few erosive areas of 2 cm each over the labial aspect of growth were seen. 21 appeared to be displaced to opposite side and 22 and 23 were displaced distally. The tongue deviated on the right side during protrusion [Figure 2]. On palpation the swelling was sessile, firm in consistency and slightly tender on palpation and was fixed to the underlying structures. Involvement of regional lymph nodes was ruled out. Maxillary occlusal radiograph revealed a well defined dense shadow in the pre-maxillary region, displaced 21, 22, and 23. Regional bone loss was also appreciated [Figure 3]. The mass was completely excised under local anesthesia and sent for histopathological examination which showed a dense, homogenous, eosinophilic connective tissue with fibroblastic proliferation. Multinucleated giant cells were also observed in the dense collagen bundles. A few fat cells were also observed, which confirmed the diagnosis as desmoplastic fibroblastoma [Figure 4]. Involved teeth were extracted followed by placement of prosthesis. No recurrence was observed in the present case during the 1 year follow-up.
| Discussion|| |
Desmoplastic fibroma, clinically presents as a firm, well-circumscribed subcutaneous or intramuscular, round to oval, or lobulated mass, which appears fibrous with a glistening gray to white, on cut surface. They are most commonly seen in the upper extremities. Approximately 80 cases have been discussed in the literature till date, among which the largest series of 63 cases of collagenous fibroma have been discussed by Miettinen and Fetsch.  Mesquita et al., were the first to report an intraoral collagenous fibroma.  The lesion is frequently seen to affect males >50 years of age. On gross examination, the lesion measures from 1cm to 20cm and presents as a painless tumor of long duration.  Collagenous fibromas characteristically present as slowly enlarging non-tender masses that are noted to increase in size for over a year in approximately one-third of cases. The tumor is rarely seen in the oral cavity and the most common site of occurrence is the tongue. Cases involving the tongue, alveolar bone and buccal mucosa have also been mentioned in the literature. 
The lesion represents a neoplasm, given that no associated event was clinically mentioned and no specific cause could be microscopically identified for the fibrous proliferation.  Similarly in the present case, neither a clinically evident event nor any history of a traumatic injury was elicited by the patient. A cytogenetically abnormal clone, involving the 11q12 breakpoint has been identified with the neoplastic nature of the lesion in a few cases.  Oral desmoplastic fibroblastoma presents clinically as a painless, slow growing, firm to elastic in consistency, well-circumscribed mass, with yellowish, reddish, or normally colored mucosa, with a size >1 cm and no associated history of trauma.  When lesion enlarges as in the present case, it may lead to difficulty in speech, mastication, and mouth closing, and displacement of adjacent teeth.  The current case is in accordance with these clinical features.
The differential diagnosis of desmoplastic fibroblastoma includes a variety of soft tissue neoplasms that enlarge slowly over a period of time and are of a medium size. Neurofibroma, schwannoma, fibromatosis, nodular fasciitis, fibroma of tendon sheath and myofibroma can be considered in the clinical differential diagnosis. Other rare possibilities may include benign fibrous histiocytoma and granular cell tumor, as well as leiomyoma, which rarely involve the buccal mucosa. The pleomorphic adenoma should be included as a possible differential diagnosis as well. 
Microscopically, the lesion can be characterized by a paucicellular proliferation of spindle to stellate-shaped cells, present in a highly hyalinized collagenous stroma with or without focal fibromyxoid degeneration.  Few case reports have observed the presence of binucleation and a few multinucleated giant cells. Other common and distinguishing histological findings include adjacent skeletal muscle or fat tissue entrapment.  However, the recognition of collagenous fibroma is easier if the age of the patient, site of occurrence, size and depth of the lesion is evaluated along with the histological findings. Immunohistochemistry also aids in establishing the diagnosis, when doubt persists. DF is known to have strong and diffusely positive affinity to vimentin and preferably negative to S-100 protein, CD34, desmin, cytokeratins, epithelial membrane antigen and CD68.  The treatment of desmoplastic fibroblastoma is total surgical excision with a good prognosis. No tumor recurrence has been reported in the literature during the follow-up.
| Conclusion|| |
Desmoplastic fibroblastoma is rarely seen in oral cavity. These are rare fibrous soft tissue tumours that usually arise in subcutaneous tissue or skeletal muscle in a wide variety of anatomical sites. Despite this case having some similar features with the previously been reported cases in the literature, this case has been reported to increase awareness of these rare lesions amongst dental practitioners. It is essential to know the characteristics of this rare tumor in an attempt to prove differential diagnoses when compared to other similar oral lesions.
| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]