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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 13  |  Issue : 2  |  Page : 76-79

Desmoplastic ameloblastoma of maxilla - A case report


Department of Oral and Maxillofacial Pathology, GSL Dental College and Hospital, Rajahmundry, Andhra Pradesh, India

Date of Submission14-Apr-2022
Date of Decision05-May-2022
Date of Acceptance06-May-2022
Date of Web Publication20-Jun-2022

Correspondence Address:
Dr. Puja Darshana Mishra
D. No. 5-71/2c, Anjani Street, First Floor, Santosh Nagar, Kakinada - 533 005, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/srmjrds.srmjrds_59_22

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  Abstract 

Desmoplastic ameloblastoma is an unusual type of ameloblastoma which varies substantially from other variants of ameloblastoma. This case report describes a case of desmoplastic ameloblastoma in a 40-year-old male patient who presented with a swelling in the right maxillary anterior region which was painless in nature. The patient was apprehensive about the asymmetry caused by the massive tumor. Initially, conventional computed tomography was done, and the diagnosis was confirmed by histopathology. Surgical resection of the tumor was done, and a prefabricated prosthetic splint was given postoperatively. The surgical site healed completely, and there was no recurrence at 6-month follow up. The present case report emphasizes the specific histological features of the tumor. Due to its invasive nature, early intervention is needed to reduce the disfigurement, functional hindrance, and other complications caused by the tumor.

Keywords: Desmoplastic ameloblastoma, incisional biopsy, massive tumor


How to cite this article:
Mishra PD, Raj Kumar N G, Kumar A S, Karri RL, Ashok S. Desmoplastic ameloblastoma of maxilla - A case report. SRM J Res Dent Sci 2022;13:76-9

How to cite this URL:
Mishra PD, Raj Kumar N G, Kumar A S, Karri RL, Ashok S. Desmoplastic ameloblastoma of maxilla - A case report. SRM J Res Dent Sci [serial online] 2022 [cited 2022 Jul 5];13:76-9. Available from: https://www.srmjrds.in/text.asp?2022/13/2/76/347818


  Introduction Top


Desmoplastic ameloblastoma is a rare variety of ameloblastoma, accounting for only 4%–13% of the total number of ameloblastomas reported in the literature.[1] These tumors are usually detected earlier due to their peculiar anterior location and are smaller in size with an average of 3 cm. 70% of these lesions show buccal expansion.[1] They present with a multilocular appearance and ill-defined border.

In this case report, we present a case of a large desmoplastic ameloblastoma of the anterior maxilla in a 40-year-old male patient. The tumor size was observed to be approximately 4 cm in diameter which is comparatively larger than the reported median size. The present case shows both buccal and palatal expansion. The tumor presented with an ill-defined border, unlike the solid multicystic variant of ameloblastoma, suggesting its locally invasive nature and its aggressiveness.


  Case Report Top


A male patient aged 40 years presented with a chief complaint of swelling in the right upper premolar region for 15 years. The swelling was initially pea sized and gradually progressed to the present size. The patient had visited several clinics before being referred to our institute for further evaluation. The patient had no relevant medical or family history. The patient had habit of cigarette smoking (6 chuttas per day).

Extraorally, a swelling of approximately 4 cm × 4 cm on the right cheek region in oval shape extending superoinferiorly below the right eyeball to the right commissure was seen. Facial asymmetry was evident [Figure 1]. Extraoral findings were confirmed by intraoral examination. The swelling was firm and was tender on palpation. The tooth adjacent to the tumor showed no mobility or displacement.
Figure 1: Extraoral and intraoral picture of the patient showing swelling in the right anterior maxillary region

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Radiographic examination through computed tomography showed an extensive osteolytic lesion extending superomedially into the maxillary sinus. Scalloping of the lateral wall of the maxillary sinus was observed. There was ill-defined intralesional moderately enhancing hypodense soft-tissue component with multiple nonenhancing areas. The present lesion is seen to involve the right half of the palate and right inferior turbinate [Figure 2].
Figure 2: CT scan (a) axial view showing involvement of the right inferior turbinate (circled area) and (b) showing osteolytic lesion extending into the maxillary sinus (circled area). CT: Computed tomography

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Both hard tissue and soft tissues were received for histopathological analysis [Figure 3]. Histopathological analysis revealed the presence of fibrous connective tissue stroma exhibiting dense collagen bundles. The odontogenic epithelium was compressed due to extensive desmoplasia in the stroma, and few inflammatory cells were noticed. The compressed odontogenic epithelium was stretched, giving it a “Kite tail like appearance” [Figure 4] and [Figure 5] in the microscope. Surgical resection of tumor was done and a prefabricated prosthetic splint was given immediately. The patient was followed up at 1-month, 3-month, and 6-month intervals. Follow-up at 6-month intervals showed no recurrence.
Figure 3: (a) Shows the incisional biopsy specimen received for histopathological analysis. (b) shows resected surgical tissue

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Figure 4: Photomicrograph of incisional biopsy: (a and b) Shows dense collagen bundles and compressed odontogenic epithelium (black arrows) under ×4 and ×10 magnification, respectively (c) Shows “kite tail like appearance” of the odontogenic epithelium (red arrow) under ×40 magnification

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Figure 5: Photomicrograph of the resected specimen (a) Dense collagen bundles and compressed odontogenic islands under ×10 magnification (b) Green arrow shows odontogenic epithelium, and blue arrow shows thick collagen bundles indicating desmoplasia under ×40 magnification

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  Discussion Top


Ameloblastoma is a benign tumor of jaws which is odontogenic in origin. They occur clinically in three different forms – unicystic, conventional, and extraosseous forms. Radiographically, conventional and unilocular patterns are noted.[2] Follicular, acanthomatous, plexiform, granular type, basal cellular, keratoameloblastoma, clear cell ameloblastoma, and desmoplastic and papilliferous keratoameloblastoma are the histological variants of the tumor.[3] Desmoplastic variant is quite rare among the mentioned variants and presents with characteristic radiologic and histologic features. It was first described by Eversole in the year 1984 as “ameloblastoma with pronounced desmoplasia.” Histologically, when this variant shows a combination of plexiform or follicular ameloblastoma, it is called as “hybrid” lesion.[4] Mobility and displacement of adjacent teeth is a common feature in these lesions.

Desmoplastic ameloblastoma occurs in various age groups but predominantly between 30 and 40 years of age.[5] The mean age of occurrence of this tumor is 41.9 years, which is nearly the same age of the presented case. Based on the data collected, it can be concluded that this tumor is common among the Asian population compared to other geographical areas.[1] Like conventional variants, it has no gender preponderance. In contrast to the conventional forms, which occur in the posterior, the tumor is generally seen in the anterior regions.[5]

The radiological analysis of the regular multicystic ameloblastoma presents as a localized lesion with a honeycomb pattern with a relatively well-defined border, whereas the desmoplastic variant shows a mixed radiologic appearance and ill-defined periphery. The mixed radiologic appearance of the lesion can be attributed to the synthesis of the extracellular protein in the lesion, which might serve as a focal point for the calcification seen in this variant.[6] However, this mixed appearance is also believed to be due to osseous metaplasia within the lesion. In the present scenario, computed tomographic images showed a multilocular lesion with multiple nonenhancing areas. The ill-defined border can also be correlated histopathologically to be a bone remodeling in response to the expansion of the bone, as observed in the above-presented case.[7]

In computed tomography, the lesion shows an apparent expansion in the buccal side with partial cortical erosion, while a classical ameloblastoma may exhibit buccolingual expansion with perforation. There are three radiological presentations of desmoplastic ameloblastoma so far described until now. They are osteofibrosis type which has a mixed radiologic appearance, a radiolucent type, and a compound type with a mixed appearance, but radiolucent areas dominate.[7] Due to its large size and peculiar mixed radiologic appearance, it mimics osteoblastoma, osteosarcoma, desmoplastic fibroma, ossifying fibroma, and primary intraosseous squamous cell carcinoma, all of which can be differentiated only upon microscopic evaluation.

Few lesions like ameloblastic fibroma and squamous odontogenic tumor may mimic the lesion histologically. Occasionally, Vickers–Gorlin criteria can be seen, which helps in diagnosis.[7] Sometimes, these odontogenic islands show hypercellular central areas composed of spindle-shaped polygonal epithelial cells and cuboidal peripheral cells. Histologically, the stellate reticulum-like cells are often scant, unlike conventional solid ameloblastoma, and the odontogenic epithelium is fragmented by the dense stroma. Desmoplastic ameloblastoma shows reduced expression of CK19 and increased expression of p63. Both basal and suprabasal cells show positivity for CK5 whereas only some basal and suprabasal cells show positivity for CK14. Basement membrane shows strong positivity for laminin V and type IV collagen.[9]

Compared to the regular central variant, the stroma of the desmoplastic form shows an intensely positive reaction to collagen Type VI, thus ruling out scar tissue. A study conducted showed the presence of oxytalan fibers in stroma which suggested the origin of tumor to be from the periodontal ligament of the adjacent tooth.[4] Pertaining to its unique nature and growing knowledge about the tumor, it was categorized as a different subtype of ameloblastoma in the World Health Organization classification of odontogenic tumors in 2005. Sivapathasundharam et al. suggested that the change in stromal connective tissue of the tumor can be attributed to the maturation of the solid multicystic variants which can be explained by the occurrence of hybrid tumors.[4] The location of the tumor can also be explained by the fact that the lesions in the anterior region mature earlier, explaining the mixed appearance and the unique histologic features of the lesion. The treatment modality depends on the location and size of the lesion, the lesser compact nature of the maxilla, and the presence of the maxillary sinus would cause rapid infiltration of the tumor mass, thus requiring aggressive therapy.[10] The surgical intervention can be through radical surgery (surgical resection) or enucleation.


  Conclusion Top


The present case report emphasizes the distinct features of the tumor, which supports the classification of tumor as a distinct subtype of ameloblastoma. Due to its invasive nature, early intervention is needed to reduce the disfigurement, functional hindrance, and other complications caused by the tumor. Further studies are needed to understand the complete nature of the tumor.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Sun ZJ, Wu YR, Cheng N, Zwahlen RA, Zhao YF. Desmoplastic ameloblastoma – A review. Oral Oncol 2009;45:752-9.  Back to cited text no. 1
    
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Sheikh S, Pallagatti S, Singla I, Kalucha A. Desmoplastic ameloblastoma: A case report. J Dent Res Dent Clin Dent Prospects 2011;5:27-32.  Back to cited text no. 2
    
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Gade L, Patankar S, Khot K, Korde S, Alex S. Desmoplastic ameloblastoma of maxilla – A case report. J Clin Exp Dent 2010;2:204-6.  Back to cited text no. 3
    
4.
Sivapathasundharam B, Einstein A, Syed RI. Desmoplastic ameloblastoma in Indians: Report of five cases and review of literature. Indian J Dent Res 2007;18:218-21.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Savithri V, Janardhanan M, Suresh R, Kumar RV. Desmoplastic ameloblastoma with osteoplasia: Review of literature with a case report. J Oral Maxillofac Pathol 2013;17:298-301.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Effiom OA, Odukoya O. Desmoplastic ameloblastoma: Analysis of 17 Nigerian cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;111:e27-31.  Back to cited text no. 6
    
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Amitha JL, Shakthi D, Karen B, Srikanth N, Mohan B, Dharnappa P, et al. Desmoplastic ameloblastoma: A report of three cases from a single institute with review of literature. Eur J Mol Clin Med 2021;8:381-9.  Back to cited text no. 7
    
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Majumdar S, Uppala D, Kotina S, Veera SK, Boddepalli R. Desmoplastic ameloblastoma. Int J Appl Basic Med Res 2014;4:S53-5.  Back to cited text no. 8
    
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Bologna-Molina R, Mosqueda-Taylor A, de Almeida-Oslei P, Toral-Rizo V, Martínez-Mata G. Peripheral desmoplastic ameloblastoma: Histopathological and immunohistochemical profile of a case. Med Oral Patol Oral Cir Bucal 2010;15:e846-9.  Back to cited text no. 9
    
10.
Sharma A, Ingole S, Deshpande M, Meshram D. Retrospective analysis of Desmoplastic Ameloblastoma: Clinical review. Med Oral Patol Oral Cir Bucal 2021;26:e246-55.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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Abstract
Introduction
Case Report
Discussion
Conclusion
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