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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 4  |  Page : 214-217
Desmoplastic ameloblastoma in the maxilla: A case report and review of literature


Department of Oral Medicine and Radiology, College of Dental Sciences, Davangere - 577 004, India

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Date of Submission14-Jun-2005
Date of Decision24-Jul-2006
Date of Acceptance26-Jul-2006
 

   Abstract 

Desmoplastic ameloblastoma (DA) is a rare variant of ameloblastoma (AM). The location of this lesion, its histology and radiological features differ from those of conventional AM. We report a case of DA in the canine / premolar region of the left maxilla of a 32-year-old woman and present a brief review of the literature. Radiographically, it had a mixed radiolucent / radiopaque appearance with ill-defined margins. Histologically, the tumor was characterized by extensive stromal desmoplasia and small tumor islands of odontogenic epithelium in the stroma, along with a few areas of reactive bone formation. The tumor was treated by partial maxillectomy and the patient was disease free after 1 year.

Keywords: Desmoplastic ameloblastoma, odontogenic tumor, stromal desmoplasia

How to cite this article:
Shashikanth M C, Neetha M C, Ali I M, Shambulingappa P. Desmoplastic ameloblastoma in the maxilla: A case report and review of literature. Indian J Dent Res 2007;18:214-7

How to cite this URL:
Shashikanth M C, Neetha M C, Ali I M, Shambulingappa P. Desmoplastic ameloblastoma in the maxilla: A case report and review of literature. Indian J Dent Res [serial online] 2007 [cited 2019 Sep 22];18:214-7. Available from: http://www.ijdr.in/text.asp?2007/18/4/214/35835

   Introduction Top


Ameloblastoma (AM) of the jaw is a neoplasm that accounts for about 1% of all cysts and tumors of the jaws and 18% of the various odontogenic neoplasms. [1]

AM may manifest with a number of histological patterns, including the follicular, plexiform, acanthomatous, keratinizing, granular cell, basal cell and clear cell types. In recent years the histomorphological spectrum of AM has expanded to include a desmoplastic variant. [2] This subtype was first identified by Eversole et al., in 1984; he reported three cases [3] and, to the best of our knowledge, 96 cases have since been reported in the literature.

Because it differs strikingly from the other forms of AM in its morphology, radiographic appearance and anatomical location, it has been suggested that the DA may represent a separate clinicopathologic entity. [1] The DAs are located in the anterior or premolar region of the maxilla or mandible and their radiographic appearance is more typical of a fibroosseous lesion than of an AM. They present radiographically as a unilocular or multilocular radiolucency or as mixed radiolucent / radiopaque areas with either well-defined or poorly defined borders. Histologically, the DA consists of a collagenized (desmoplastic) stroma, in which irregular islands of odontogenic epithelium are scattered.

We report a case of DA in the maxilla and review the literature.


   Case Report Top


A 32-year-old woman presented to the Department of Oral Medicine and Radiology, College of Dental Sciences, Davangere, Karnataka, India, with an asymptomatic, slowly enlarging swelling in her left maxilla of 2 months duration. There was no history of trauma and her past medical history was unremarkable.

Extraoral examination showed a facial swelling and elevation of the left ala of the nose. The swelling was oval and had a smooth surface. The skin over the swelling appeared normal. There was no tenderness to palpation [Figure - 1].

Intraorally, the swelling extended from the left lateral incisor to the left second premolar buccally and palatally and superoinferiorly from the vestibular mucosa to the marginal gingiva of the teeth on buccal side and on the palatal side about 2 cm from the midline to marginal gingiva. The swelling was approximately 2.5 × 3 cm in size, well defined, oval in shape and had a smooth surface. The mucosa over the swelling appeared normal [Figure - 2].

On palpation, the swelling was nontender and the inspection findings were confirmed. The swelling was bony hard in consistency, nonfluctuant, nonreducible, noncompressible and nonpulsatile. It was not fixed to the overlying mucosa and seemed to arise from within the bone. The teeth in the affected area were not sensitive to percussion and no mobility could be demonstrated. Electric pulp vitality testing revealed that all the teeth in the vicinity were vital except the left central incisor.

Radiographic examination of the maxilla revealed a mixed radiolucent / radiopaque lesion with ill-defined sclerotic borders that extended from the distal of the left central incisor to the first premolar. The lesion was approximately 2.5 × 2 cm in size. Expansion of the buccal cortical plate was evident on occlusal radiographs and the lesion contained multiple loculations. The lesion had caused divergence of the roots of the canine and first premolar teeth, with signs of root resorption. The lamina dura at the apical one-third of anteriors and first premolar was absent. The floor of the maxillary sinus appeared radiographically intact [Figure - 3],[Figure - 4].

Aspiration of the lesion was unproductive and a complete hemogram showed values within the normal range. A provisional diagnosis of adenomatoid odontogenic tumor was made and an incisional biopsy was performed to establish a definitive diagnosis. Histologically, the features were consistent with those of DA.

The tumor was treated by partial hemimaxillectomy and a partial denture was provided. The postoperative course was uneventful. The surgical specimen consisted of a portion of the maxilla, extending from distal of the right maxillary canine to distal of the left maxillary second premolar. The specimen was subjected to histopathological examination [Figure - 5].

Microscopic examination revealed small irregular islands and cords of odontogenic epithelium in a dense collagenous stroma. These islands were formed of flattened peripheral cells and central polygonal or spindle-shaped cells, with a few areas of squamous metaplasia. Hyalinization was noted around a few epithelial islands, along with bone formation in few areas. These features were suggestive of DA [Figure - 6].

Examination of the patient after 1 year showed no evidence of recurrence of the lesion.


   Discussion Top


AMs are benign, locally aggressive, polymorphic neoplasms that consist of proliferating odontogenic epithelium. [4],[5]

Leon Barnes has categorized AMs into four types on the basis of the behavioral pattern, anatomical location, histological features and radiographic appearance; these are the solid (multicystic), unicystic, desmoplastic and peripheral varieties. The first three are intraosseous (central), while the last is extraosseous (mucosal).

Since its initial description in 1984 by Eversole, 96 cases of DA have been reported in the literature [Table - 1]. [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18] It constitutes 0.9% to 12.1% of all AMs. [6] The mean age at initial presentation is 42.3 years (range 17-70 years) and men and women are almost equally affected. Age and gender predilection for DA is similar to that for the other AMs.

A striking difference between DA and AM is in the anatomic distribution and radiographic and histologic features.

DA affects the maxilla and the mandible equally, whereas AM shows a marked predilection for the mandible in a ratio of 5:1 (mandible: maxilla). DA shows a marked predilection for the anterior region of the mandible and maxilla, whereas AM is more common in the posterior region of the mandible. [6]

The radiographic features of DA differ in almost all cases from that of AM. Although the radiographic features of AM are not pathognomic, they are classically described as a unilocular or multilocular radiolucency with well-defined borders. In the maxilla, most AMs produce a unilocular radiolucency, [5] whereas DAs have a mixed radiolucent / radiopaque appearance in 64% of cases and are radiolucent in 36% of cases. Among these 27% of cases are multilocular and 9% are unilocular.

DAs are described as poorly defined in 88% of cases. This contrasts with the well-defined, often sclerotic, borders seen in AMs. The case reported had a mixed radiolucent / radiopaque appearance with partially sclerotic borders.

Tooth displacement is a common feature in DAs in almost 92% of the cases and root resorption is seen in 33% of cases. Our case showed tooth displacement as well as root resorption. [5]

CT, in high resolution, has been shown to be effective in defining the tumor boundaries and extension, if any, into the adjacent structures. Magnetic resonance imaging may be equally effective for this. However, an advantage of CT over MRI is its ability to distinguish DA from other fibrosseous lesions by the detection of the thick, bony trabeculae situated peripherally between the tumor elements. [4],[5],[7],[9],[10],[18]

Histologically, DAs show stromal desmoplasia, in the form of moderately cellular, fibrous connective tissue with abundant collagen, which is a consistent and distinguishing feature. The epithelial component consists of small, ovoid or follicle-shaped islands. The peripheral layer of cells is usually cuboidal and occasionally hyperchromatic. The palisaded columnar cells demonstrating the reversed nuclear polarity so typical of multicystic and unicystic AM are rarely conspicuous. In addition, a central zone resembling stellate reticulum is encountered only occasionally. Instead, this area appears hypercellular and is composed of spindle-shaped or polygonal epithelial cells. Occasional squamous metaplasia and foci of keratinization may be seen centrally. New bone formation, which probably contributes to the radiopacity, may be prominent in some areas. [1] Occasional DAs may exhibit interspersed zones of classic follicular or plexiform AMs; these have been designated as 'hybrid lesions'. [1],[2],[7],[12]

Preliminary results of a comparative immunohistochemical investigation of DA and typical follicular AM have shown an intense staining for collagen type VI adjacent to the tumor islands in the former, but not in the latter. This observation indicates an active de novo synthesis of extracellular matrix proteins. In other words, the tumor stroma in the DA is not scar tissue but, rather, a newly produced connective tissue. [7]

Studies have also shown increased production of the cytokine known as transforming growth factor β in association with this lesion, suggesting that this may be responsible for the desmoplasia.[1],[3] Oxytalan fibers have been demonstrated in the stroma of DA. These findings suggest that desmoplastic ameloblastoma arise from the epithelial rests of Malassez in the periodontal membrane of the related tooth. [14],[15]

In spite of the apparent and gross circumscription of the desmoplastic lesions, there has been no evidence of a capsule in the cases reported. Tumor elements were present at the periphery of these lesions, suggesting that they have a propensity to recur with a frequency equal to that of other types of AMs. [2] Therefore, it is recommended that DAs should always be treated by complete surgical resection of their margins.


   Conclusion Top


Based on the available information on DAs, the clinician should keep in mind some salient features while diagnosing and treating patients with these neoplasms.

As the majority of DAs present with radiographic features resembling that of fibroosseous lesions, a definitive diagnosis should always be based on the histology and not the radiographic and clinical findings. [11],[16]

In spite of the apparent and gross circumscription of the desmoplastic lesions, they have the potential for recurrence because they fail to produce a capsule; it is therefore recommended that DAs should always be treated by complete surgical resection.


   Acknowledgements Top


The authors express their gratitude to the Departments of Oral Surgery and Oral Pathology, College of Dental Sciences, Davangere for the help rendered.

 
   References Top

1.Barnes L. Surgical pathology of the head and neck. 2 nd ed. Vol.3, Marcel Deckker Inc: New York; 2001.   Back to cited text no. 1      
2.Waldron CA, el-Mofty SK. A histopathologic study of 116 amelobalstomas with special reference to the desmoplastic variant. Oral Surg Oral Med Oral Pathol 1987;63:441-51.   Back to cited text no. 2      
3.Eversole LR, Leider AS, Hansen LS. Ameloblastomas with pronounced desmoplasia. J Oral Maxillofac Surg 1984;42:735-40.   Back to cited text no. 3      
4.Thompson IO, Van Rensburg LJ, Phillips VM. Desmoplastic ameloblastoma: Correlative histopathology, radiology and CT-MR imaging. J Oral Pathol Med 1996;25:405-10.   Back to cited text no. 4      
5.Kaffe I, Buchner A, Taicher S. Radiologic features of desmoplastic variant of ameloblastoma. Oral Surg Oral Med Oral Pathol 1993;76:525-9.   Back to cited text no. 5      
6.Beckley ML, Farhood V, Helfend LK, Alijanian A. Desmoplastic ameloblastomas of he mandible: A case report and review of literature. J Oral Maxillofac Surg 2002;60:194-8.   Back to cited text no. 6      
7.Philipsen HP, Ormiston IW, Reichart PA. The desmo- and osteoplastic ameloblastoma. Histologic variant or clinicopatholgic entity? Case reports. Int J Oral Maxillofac Surg 1992;21:352-7.   Back to cited text no. 7      
8.Sakashita H, Miyata M, Okabe K, Kurumaya H. Desmoplastic ameloblastoma in the maxilla: Case report. J Oral Maxillofac Surg 1998;56:783-6.   Back to cited text no. 8      
9.Yoshimura Y, Saito H. Desmoplastic variation of ameloblastoma: Report of a case and review of the literature. J Oral Maxillofac Surg 1990;48:1231-5.   Back to cited text no. 9      
10.Iida S, Kogo M, Kishino M, Matsuya T. Desmoplastic ameloblastoma with large cystic change in the maxillary sinus. Report of a case. J Oral Maxillofac Surg 2002;60:1195-8.   Back to cited text no. 10      
11.Louis PJ, Fugler RC, August M. Mixed radiolucent / Radiopaque lesion of the maxilla. J Oral Maxillofac Surg 2000;58:86-90.   Back to cited text no. 11      
12.Wakoh M, Harada T, Inoue T. Follicular / desmoplastic hybrid ameloblastoma with radiographic features of concomitant fiber osseous and solitary cystic lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:774-80.   Back to cited text no. 12      
13.Neville BW, Damm DD, Allen CM, Bouquot. Oral and maxillofacial pathology. 2 nd ed. Saunders: Philadelphia, PA; 2002.   Back to cited text no. 13      
14.Kawai T, Kishino M, Hiranuma H, Sasai T, Ishida T. A unique case of desmoplastic ameloblastoma of the mandible: Report of a case and brief review of the English language literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:258-63.   Back to cited text no. 14      
15.Kishino M, Murakami S, Fukuda Y, Ishida T. Pathology of the desmoplastic ameloblastoma. J Oral Pathol Med 2001;30:35-40.   Back to cited text no. 15      
16.Ng KH, Siar CH. Desmoplastic variant of ameloblastoma in Malaysians. Br J Oral Maxillofac Surg 1993;31:299-303.   Back to cited text no. 16      
17.Maneul S, Simon D, Rajendran R, Raghava Naik B. Desmoplastic ameloblastoma: A case report. J Oral Maxillofac Surg 2002;60:1186-8.   Back to cited text no. 17      
18.Keszler A, Paparella ML, Dominguez FV. Desmoplastic and non-desmoplastic ameloblastoma: A comparative clinico pathologic analysis. Oral Dis 1996;2:228-31.  Back to cited text no. 18      

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Correspondence Address:
M C Neetha
Department of Oral Medicine and Radiology, College of Dental Sciences, Davangere - 577 004
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9290.35835

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]
 
 
    Tables

  [Table - 1]

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