Only one other case of desmoplastic ameloblastoma has been reported in the ramus region of mandible of the 90 cases that we have reviewed. Review of. Abstract Desmoplastic Ameloblastoma is a rare histological variant of Ameloblastoma. Approximately cases of desmoplastic Ameloblastoma have been. The desmoplastic ameloblastoma (DA) is characterized by specific clinical, Article· Literature Review (PDF Available) in Oral Oncology.

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Desmoplastic ameloblastoma DA is a relatively rare histological variant of ameloblastoma with specific clinical, radiological, and histological features. This tumor desnoplastic more commonly seen in the anterior region of jaws as a mixed radiopaque-radiolucent lesion resembling benign fibro-osseous lesions. Metaplastic bone formation has been reported in few cases.

This report describes a lesion in the left anterior maxilla of a young female patient. Ameloblastoma is the second most common odontogenic tumor. It arises from odontogenic epithelium and is known for its distinct aggressive clinical behavior and characteristic histologic picture.

Desmoplastic ameloblastoma – A review

A characteristic feature is an almost equal distribution in location between the maxilla and mandible. Radiologically, the desmoplastic variant exhibits atypical and varied radiographic features such as: Localized irregular multilocular radiolucency with indistinct borders, or a mixed radiopaque-radiolucent appearance with ill-defined margins similar to fibro-osseous lesion, or a massive expansile osteolytic lesion with honeycomb, mottled or multilocular appearance.

DA may be defined as a benign but locally invasive variant consisting of proliferating, irregular, often bizarrely shaped islands and cords of odontogenic epithelium of varying sizes embedded in a desmoplastic connective tissue stroma. Formation of metaplastic bone trabeculae osteoplasia rimmed by active osteoblasts has been described in a few cases.

A year-old female reported with a swelling in relation to the upper front teeth. The swelling was slow in growth and present since 4 months. She had also noticed a slight downward movement of the affected teeth and had mild pain. On examination, a bony hard swelling was seen in relation to 21, 22 and 23 on the buccal aspect measuring about 2. Grade I mobility of 21 and 22 was also noticed. Radiographs revealed the presence of irregular radiolucencies with faint radio-opacities in the periapical region of 22 and Lamina dura of the teeth was hazy and teeth were seen to be displaced [ Figure 1 ].

A provisional diagnosis of benign fibro-osseous lesion was made and an incisional biopsy was done. Intra-oral periapical radiograph showing irregular radiolucencies with faint radio-opacities. Both hard-tissue and soft-tissue were received. The specimens showed few irregular epithelial islands in a densely fibrous connective stroma.

The islands consisted of peripheral cuboidal cells and central spindle cells. Some of the islands showed cystic degeneration [ Figure 2 ]. Extensive desmoplasia was seen throughout the stroma compressing the epithelial islands [ Figure 3 ].


Areas of osseous metaplasia with active osteoblasts were also noted [ Figure 4 ]. With the above picture, a diagnosis of DA with osteoplasia was made. Clinically, DA may occur in all age groups with more cases in the 4 th to 5 th decade. There is no gender predilection and is seen equally in males and females. DA is reported to occur in the anterior or premolar region of either maxilla or mandible.

This is in contrast to the conventional ameloblastoma which is found predominantly in the posterior region of mandible. Usually, the patient’s first symptom is a painless swelling. Involved teeth may be displaced with cortical plate expansion. The size of DA is mostly smaller than other types of ameloblastoma.

A case of peripheral DA has also been reported recently. The radiographic features of DA also differ from the conventional ameloblastoma. Again, the borders of DA are usually poorly defined. The unique radiographic picture seen is thought to be due to its infiltrative nature. The presence of osteoplastic bone can also present similarly. Histological features consisted of proliferating, irregularly shaped islands and cords of odontogenic epithelium of varying sizes embedded in a desmoplastic connective tissue stroma.

The epithelial cells at the periphery of the islands are cuboidal and occasionally show hyperchromatic nuclei. Columnar cells demonstrating reversed nuclear polarity are rarely conspicuous although some islands may show ameloblast-like cells peripherally.

DA exhibiting granular cell transformation in some of the tumor cells has been reported. Extensive stromal desmoplasia is a striking finding with a tendency to squeeze or compress the odontogenic islands at the periphery. The mechanism of desmoplasia is not understood even after two decades.

Various immunohistochemical studies have reported DA ammeloblastoma cells as showing variable expression of S protein and desmin, high expression of caspase-3 and Fas, decreased expression of cytokeratin a,eloblastoma and high expression of p The extracellular matrix has an important role in the behavior of neoplastic cells and immunohistochemical studies on the expression of tenascin and fibronectin proteins and Type I collagen in hybrid lesions have also desmoplwstic reported. This indicates the greater aggressive potential of conventional follicular ameloblastoma as compared to DA as tenascin has been observed in unstable environments like neoplasia.

Type I collagen showed intense staining in a fibrillar pattern in DA areas reflecting its high grade in the matrix. Prominent osteoplasia within the tumor tissue seen in our case has been reported only in desmoplastif other cases of DA so far. Remnants of non-neoplastic bone often seem to remain in the tumor tissue.

Studies have been done to find whether the bone found is new bone or mature bone. Fibrous bone can be recognized using Masson-trichrome staining. Hence it can be suggested that if the desmoplasia is caused by tumor cell stimulation of stromal fibroblasts, then that stimuli could also affect another cell type, i. The biologic behavior of the lesion is still debatable. Conflicting reports are available regarding the recurrence because of paucity of adequate samples.


Furthermore, the lesions in maxilla are close to many vital structures and the thin cortical bone reivew a weak barrier and the tumor usually infiltrates into marrow spaces. The radiological and histological findings of poor encapsulation and ill-defined borders warrants a long-term follow-up.

A radical approach to its treatment is recommended as in a case of conventional ameloblastoma. National Center for Biotechnology InformationU.

J Oral Maxillofac Pathol. Author information Copyright and License information Disclaimer. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. This article has been cited by other articles in PMC. Abstract Desmoplastic ameloblastoma DA is a relatively rare histological variant of ameloblastoma with specific clinical, radiological, and histological features. Open in a separate window.

Footnotes Source of Support: Odontogenic Tumors and Allied Lesions. Benign Neoplasms and tumor-like lesions arising from the odontogenic apparatus showing odontogenic epithelium with mature fibrous stroma, without ectomesenchyme; p. Ameloblastomas with pronounced desmoplasia. J Oral Maxillofac Surg. Biological profile based on cases from the literature and own files.

Desmoplastic ameloblastoma-a report of two clinical cases. Braz J Oral Sci. Effiom OA, Odukoya Desmoppastic.

Analysis of 17 Nigerian cases. A histopathologic study of ameloblastomas with special reference to the desmoplastic variant. Report of a rare case. Radiologic features of desmoplastic variant of ameloblastoma. Clinical and histopathological analyses of desmoplastic ameloblastoma.

A case report and literature review. Desmoplastic ameloblastoma featuring basal cell ameloblastoma: A unique case of desmoplastic ameloblastoma of the mandible: Report of a case and brief ammeloblastoma of the English language literature. Desmoplastic ameloblastoma in Indians: Report of five cases and review of literature. Indian J Dent Res. Desmoplastic ameloblastoma a hybrid variant: Report of a case and review of the literature. Immunoexpression of transforming growth factor beta in desmoplastic desmopplastic.

Pathology of the desmoplastic ameloblastoma. J Oral Pathol Med.

Desmoplastic ameloblastoma with osteoplasia: Review of literature with a case report

Recurrent desmoplastic ameloblastoma of the maxilla: J Can Dent Assoc. Articles from Journal of Oral and Maxillofacial Pathology: Support Center Support Center.

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