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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 2  |  Page : 189-191

Aggressive osteolytic lesion of mandible - A case report


1 Department of Oral Medicine and Radiolog, KM Shah Dental College and Hospital, Sumandeep Vidyapeeth, Piparia, Vadodara, Gujarat, India
2 Department of Oral and Maxillofacial Surgery, Manubhai Patel Dental College, Hospital and Oral Research Institute, Vadodara, Gujarat, India
3 Department of Oral Medicine and Radiology, Manubhai Patel Dental College, Hospital and Oral Research Institute, Vadodara, Gujarat, India
4 Department of Oral Pathology and Microbiology, Manubhai Patel Dental College, Hospital and Oral Research Institute, Vadodara, Gujarat, India

Date of Submission30-Nov-2019
Date of Decision22-May-2020
Date of Acceptance23-May-2020
Date of Web Publication27-Jun-2020

Correspondence Address:
Dr. Deepa J Patil
Oral Medicine and Radiology KM Shah Dental College and Hospital, Sumandeep Vidyapeeth, Piparia, Vadodara, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_197_19

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   Abstract 


Odontogenic tumors are rare entities, often derived from epithelial remnants in the gnathic bones following odontogenesis. Ameloblastoma is benign locally invasive accounting for 1% of tumors of jaws. Among the various ameloblastoma, solid multicystic ameloblastoma is believed to be locally aggressive lesion that has the tendency for recurrence. In this report, we present a large aggressive solid multicystic ameloblastoma in a 33-year-old male patient diagnosed by computed tomography and successfully managed by hemimandibulectomy with simultaneous reconstruction using fibula. We have also briefly discussed the molecular pathogenesis and new treatment aspects of ameloblastoma.

Keywords: Ameloblastoma, molecular pathogenesis, reconstruction


How to cite this article:
Patil DJ, Thakur R, Joshi M, Phulare R. Aggressive osteolytic lesion of mandible - A case report. J Indian Acad Oral Med Radiol 2020;32:189-91

How to cite this URL:
Patil DJ, Thakur R, Joshi M, Phulare R. Aggressive osteolytic lesion of mandible - A case report. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2020 Jul 7];32:189-91. Available from: http://www.jiaomr.in/text.asp?2020/32/2/189/288128




   Introduction Top


Ameloblastoma is a benign locally invasive odontogenic tumor, accounting for 1% of jaw tumors and 11% of odontogenic tumors.[1] The importance of ameloblastoma lies in its potential to grow into a larger size with bone deformity. They are typically classified as unicystic, solid/multicystic, peripheral, desmoplastic, and malignant subtypes.[2] A solid or multicystic ameloblastoma is a benign epithelial tumor of odontogenic origin showing a strong tendency to recurrence and local aggression.[1]

Here in, we present a large aggressive lesion involving the left mandible in a 33-year-old male patient who was managed by surgical resection; hemimandibulectomy followed by reconstruction. The paper also discusses the differential diagnosis, molecular pathogenesis, and new treatment aspects of ameloblastoma.


   Case Report Top


A 33-year-old male patient reported with an insidious swelling and pain on the lower left side of the face for 3–4 months. On extra-oral examination, there was a solitary ill-defined diffuse swelling over the left lower third of the face, soft in consistency and non-tender.

Intraorally an ill-defined soft, expansile, solitary swelling [Figure 1] was observed in the left lower posterior buccal vestibule. Aspiration yielded a straw-colored aspirate. In view of the clinical findings, a provisional diagnosis of benign tumor of the left side of lower jaw was made. Ameloblastoma, odontogenic keratocyst (OKC) and odontogenic myxoma were considered in the differential diagnosis. Panoramic radiograph [Figure 2] revealed a large multilocular radiolucency (soap bubble) extending from the lower right central incisor region to the left body, angle, and ramus region with resorption of coronoid process and thinning of the inferior border of mandible. The borders were corticated and scalloped. There was evidence of knife edge shaped external root resorption of 33, 34, 35, 36, 37, and 38. Computed tomography (CT) [Figure 3] scan with contrast revealed a well-defined lytic cystic lesion involving the left body and ramus of the mandible with subtle wall enhancement. 3D reformatted CT [Figure 4] depicting the massive osteolytic lesion with soap bubble multilocular radiolucency encompassing the body and ramus of the left mandible. An incisional biopsy taken from the left mandibular buccal vestibule suggested ameloblastoma on histopathological examination.
Figure 1: Intraoral obliteration of left buccal vestibule

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Figure 2: Panoramic radiograph depicting soap bubble radiolucency of left mandible

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Figure 3: Axial CT demonstrating a multilocular, expansile radiolucent lesion involving the left symphysis, body and ramus of mandible

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Figure 4: 3D CT depicting the multilocular radiolucency with septae extending in to the ramus

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A hemimandibulectomy [Figure 5] was performed along with reconstruction using fibula bone. Macroscopically, specimen revealed solid and large cystic spaces with embedded teeth.
Figure 5: Macroscopic specimen revealing cystic spaces and teeth embedded in the specimen

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The H and E stained sections [Figure 6] revealed a cystic cavity lined by odontogenic epithelium. The basal layer of epithelium was composed of cuboidal cells with prominent and hyperchromatic nuclei resembling ameloblasts and spongiosis. The clinical and histopathological features suggested conventional ameloblastoma with extensive cystic degeneration.
Figure 6: H & E section revealing a cystic cavity lined by odontogenic epithelium at places and a connective tissue capsule

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   Discussion Top


Amelobastoma, as the name implies, are true neoplasms of enamel-organ-type tissue that do not undergo differentiation to the point of enamel formation. It has been postulated that SMAs may also arise because of neoplastic changes in the wall lining of the wall of a non-odontogenic cyst.[2] In our case, we suspected SMA could have originated from a previous OKC because of the anteroposterior extension, but we could not infer it histopathologically.

A specific etiology for ameloblastoma is yet to be elucidated. Human papillomavirus (HPV) infection, non-specific irritation from extractions, trauma, inflammation, and nutritional deficiencies has all anecdotally been proposed as etiologies.[3] Several studies have characterized mutations in the MAPK pathway and the sonic hedgehog (SHH) pathway, in the pathogenesis of ameloblastomas.[4]

Reichart et al. (1995),[2] scrutinized the literature and analyzed 3,677 patients with ameloblastoma. Till date this is the largest retrospective analysis. According to their analysis, ameloblastoma most commonly presents as swelling over the affected region (mandible in 80% of cases) at an average age of 36 years with equal sex distribution. The present patient was a 33-year-old male patient with facial asymmetry, involving the left symphysis, body, and ramus of mandible with horizontally impacted 38, loose teeth and expansion of the buccal and the lingual cortical plate.

For the preoperative assessment and postoperative management, radiographs are imperative. The stereotypical radiographic findings on a panoramic radiograph are a lytic lesion with scalloped margins or a “soap bubble” appearance, which can be associated with resorption of tooth roots and impacted teeth. To plan surgery, helical CT with three-dimensional reformations and holograms should be done. On CT the ameloblastoma appears as uni or multiple cystic areas of low attenuation with enhancing solid areas. The unilocular lesions occur most often in the maxilla. The mixed cystic and solid form often has a honeycomb or soap bubble appearance, a classic finding as seen in our case. Thinning or frank dehiscence of the adjacent cortices with extension into adjacent soft tissues.[5] All these radiographic findings can be corroborated in the present case.

Histopathologically solid/multicystic ameloblastoma (SMA) is characterized by the intramural emergence of multiple cystic spaces. It accounts for 86% of all described ameloblastomas.[6] This form is characterized by aggressive behavior in relation to the surrounding anatomical structures and frequent recurrence in the absence of radical treatment.

Ameloblastoma is known for its high recurrence rate if excision is incomplete. Therefore, the treatment of choice is a surgical excision with wide free margins, 1.5–2 cm beyond the radiological limit.[1] Microvascular surgery with fibula for reconstruction [7] has become the preferred option and was followed in our case.

There is a lot of deliberation in the literature on the role of radiotherapy and chemotherapy as adjuvant therapy. The role of intensity modulated radiotherapy (IMRT) and proton beam therapy as an adjuvant after surgery to control locoregional recurrence has been emphasized in a literature review by Koukourakis et al.[8] Kennedy et al. treated six cases with radiotherapy, and endorsed the use of radiation as an adjunct to reduce the risk of recurrence.[9] Vismodegib, a specific inhibitor of SMO, showed reduction in size of OKC tumor in 4 of 6 patients.[10]


   Conclusion Top


Ameloblastoma is a rare tumor of the mandible with a well-documented propensity for locoregional invasion and risk of recurrence. Trendsetting research in ameloblastic oncogenesis has paved way for two central pathways, MAPK and SHH, as important avenues for clinical trial exploration. This ingenious modality can be helpful in reducing surgical morbidity, which can be substantial in the treatment of ameloblastoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has given his consent for his 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

1.
Becelli R, Carboni A, Cerulli G, Perugini M, Iannetti G. Mandibular ameloblastoma: analysis of surgical treatment carried out in 60 patients between 1977 and 1998. J Craniofac Surg 2002;13:395-400.  Back to cited text no. 1
    
2.
Reichart PA, Philipsen HP, Sonner S. Ameloblastoma: Biological profile of 3677 cases. Eur J Cancer B Oral Oncol 1995;31:86-99.  Back to cited text no. 2
    
3.
Kahn MA. Ameloblastoma in young persons: A clinicopathologic analysis and etiologic investigation. Oral Surg Oral Med Oral Pathol 1989;67:706-15.  Back to cited text no. 3
    
4.
Heikinheimo K, Kurppa KJ, Elenius K. Novel targets for the treatment of ameloblastoma. J Dent Res 2015;94:237-40.  Back to cited text no. 4
    
5.
Ariji Y, Morita M, Katsumata A, Sugita Y, Naitoh M, Goto M, et al. Imaging features contributing to the diagnosis of ameloblastomas and keratocystic odontogenic tumours: Logistic regression analysis. Dentomaxillofac Radiol 2011;40:133-40.  Back to cited text no. 5
    
6.
Peter AR, Philipsen HA. Odontogenic Tumors and Allied Lesions. 1st ed. London: Quintessence; 2004. p. 43-58.  Back to cited text no. 6
    
7.
Disa JJ, Cordeiro PG. Mandible reconstruction with microvascular surgery. Semin Surg Oncol. 2000;19:226-34.  Back to cited text no. 7
    
8.
Koukourakis GV, Miliadou A, Sotiropoulou-Lontou A. Ameloblastoma, a rare benign odontogenic tumour: An interesting tumour review targeting the role of radiation therapy. Clin Transl Oncol 2011;13:793-7.  Back to cited text no. 8
    
9.
Kennedy WR, Werning JW, Kaye FJ, Mendenhall WM. Treatment of ameloblastoma and ameloblastic carcinoma with radiotherapy. Eur Arch Otorhinolaryngol 2016;273:3293-7.  Back to cited text no. 9
    
10.
Ally MS, Tang JY, Joseph T, Thompson B, Lindgren J, Raphael MA, et al. The use of vismodegib to shrink keratocystic odontogenic tumors in patients with basal cell nevus syndrome. JAMA Dermatol 2014;150:542-5.  Back to cited text no. 10
    


    Figures

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



 

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