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Case Report

Follicular with plexiform ameloblastoma in anterior mandible: Report of case and literature review
Rahul Srivastava a,*, Parvathi Devi b, Bhuvan Jyoti c, Shobhit Pradhan d, Ashutosh Shukla e
Senior Lecturer, Department of Oral Medicine and Radiology, Rama Dental College, Hospital and Research Centre, Kanpur, Uttar Pradesh, India b Professor and Head of the Department, Department of Oral Medicine and Radiology, Rama Dental College, Hospital and Research Centre, Kanpur, Uttar Pradesh, India c Dental Surgeon and Consultant, Oral Medicine and Radiology, Ranchi Institute of Neuro-Psychiatry and Allied Sciences, India d Senior Lecturer, Department of Oral Pathology, HIDS, Paonta Sahib, Himachal Pradesh, India e Student, Department of Oral and Maxillofacial Surgery, Rama Dental College, Hospital and Research Centre, Kanpur, Uttar Pradesh, India
a

article info
Article history: Received 10 November 2011 Accepted 9 July 2012 Keywords: Ameloblastoma Plexiform Follicular

abstract
Ameloblastoma is a benign tumor of odontogenic epithelium which is more commonly seen in the posterior region of mandible and the maxilla. About 66% of ameloblastomas occur in mandible most often in the molar ascending ramus area, while only 10% is seen in mandibular anterior area. Ameloblastomas are slow growing, locally invasive, rarely malignant and in most cases can cause severe abnormalities of the face and jaw. These are seen usually between 40 and 60 years of age. Radiographically it appears as radiolucent lesion usually with well circumscribed borders. Early lesions usually appear unilocular while established ones are generally multilocular. This is a case report of a 40 year old male patient with follicular as well as plexiform type of ameloblastoma who presented with a swelling in lower anterior teeth region, slow growing and crossing the midline which was associated with no pain. 2012 Indian Journal of Dentistry. All rights reserved.

1.

Introduction

Benign mandibular swellings can be due to a wide variety of lesions and can be divided into odontogenic and nonodontogenic origin. Among these are ameloblastoma, radicular cyst, dentigerous cyst, keratocystic odontogenic tumor, central giant cell granuloma, broosseous lesions and osteomas.1 The ameloblastoma is a benign odontogenic tumor of epithelial origin that exhibits a locally aggressive behavior with a high level of recurrence, being believed to theoretically come from dental lamina remains, the enamel organ in

development, epithelial cover of odontogenic cysts or from the cells of the basal layer of the oral mucosa.2 The ameloblastoma is a relatively rare dental tumor described for the rst time by Broca in 1868, and so denominated by Churchill in 1934. According to Larsonn and Almeren, its incidence is 0.6 cases per million, while Shear and Singh found an incidence of 0.31 cases per million in a white population of Witwatersrand in South Africa.3 The etiology of ameloblastoma is still unknown but has been linked to faulty regulation of the genes involved in tooth development.4 Several causative factors have been proposed, including

* Corresponding author. 783/4 W-1, Saket Nagar, Juhi-2, Kanpur, Uttar Pradesh-208014, India. Tel.: 91 9450326179. E-mail address: drrahul_osmf@yahoo.com (R. Srivastava). 0975-962X/$ e see front matter 2012 Indian Journal of Dentistry. All rights reserved. http://dx.doi.org/10.1016/j.ijd.2012.07.001

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nonspecic irritating factors such as extraction, caries, trauma, infection, inammation, or tooth eruption; nutritional decit disorders; and viral pathogenesis.5 It generally occurs in bone, and it has been postulated that the epithelium of origin is derived from one of the following sources: cell rests of the enamel organ, epithelium of odontogenic cysts, disturbances of the developing enamel organ, basal cells of the surface epithelium or heterotropic epithelium in other parts of the body. The theory of an odontogenic origin for the ameloblastoma is supported clinically by the tumors common occurrence in the tooth bearing area and is further reinforced by the nding of Spouge that one in every three such tumors are mural proliferations in intimate association with the reduced enamel forming epithelium of dentigerous cysts.6 Ameloblastomas can occur at any location in the mandible or maxilla, but the regions of the inferior molars and mandibular ramus are the most prevalent anatomical locations (80%). When the tumor occurs in the maxilla, the posterior region is the most affected, and the evolution and invasion of the tumor may compromise the maxillary sinus and the orbit.7 Ameloblastomas of the mandible occur 12 years earlier than those of the maxilla. Ameloblastomas occur most frequently in the molar region of the mandible. In Blacks, ameloblastomas occur more frequently in the anterior region of the jaws.8 The lesion presents prevalence between the third and the fourth decade of life, equally affecting both genders and having no predilection for race. Radiographically, the neoplasia can be presented as a unilocular or multilocular radiolucent image in the shape of soap bubbles or honeycomb. The absorption of the adjacent teeth roots is not found to be uncommon and the presence of a tooth inside, usually the inferior third molar can be associated to the tumoral mass.2 The chief histopathological variants of ameloblastoma are the follicular and plexiform types, followed by the acanthomatous and granular cell types. Uncommon variants include desmoplastic, basal cell, clear cell ameloblastoma, keratoameloblastoma and papilliferous ameloblastoma.9

Fig. 1 e Patient showing extraoral swelling in chin region.

2.

Case report

A 40 years old male patient reported to the department of oral medicine and radiology with chief complaint of swelling in the lower front teeth region since 6 months. Patient gave the history of swelling in the anterior mandibular region which gradually increased in size to attain the present size which was asymptomatic. On examination patient was moderately built and nourished and his vital signs were within normal limit. Extraoral examination revealed a diffuse swelling present over the chin region measuring approximately 5 7 cms in size, extending superioinferiorly from lower lip up to inferior border of the mandible and anteroposteriorly from right to left corner of the mouth. Overlying skin of the swelling was normal (Fig. 1). Intraorally, solitary well dened swelling in anterior part of mandible extending from 35 to 45, crossing the midline with vestibular obliteration. Overlying mucosa was normal in color, smooth and shiny. There was generalized edema of gingival with bleeding on probing and deposits of stains and calculus (Fig. 2). Grade I mobility was present in 31,32,41,42 and teeth

from 35 to 45 were found to be nonvital. Swelling was rm in consistency and non tender on palpation. Based on history and clinical examination, a provisional diagnosis of ameloblastoma was given. Fine needle aspiration of uid was done and send for biochemical examination which revealed protein content 6.7 gm% (Fig. 3). Cross sectional mandibular occlusal projection revealed expansion of buccal and lingual cortical plate along with multilocular radiolucency extending from 35 to 46 separated by thick septae (Fig. 4). Panoramic radiograph showed a large well dened multilocular radiolucency in the mandible extending from 36 to 46. The radiolucency was corticated and separated by thick septae with scalloped borders. Root resorption in relation to 34, 35 was seen and there was widening of periodontal ligament space and loss of lamina dura (Fig. 5). Axial CT images demonstrated well dened multilocular expansile lesion with destructive changes and cortical thinning (Fig. 6). Incisional biopsy of the lesion was performed and microscopic examination revealed long

Fig. 2 e Intraoral examination showing swelling in anterior part of mandible.

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Fig. 5 e Panoramic radiograph.

Fig. 3 e Aspirated uid.

anastomosing cords or larger sheets of epithelium which were bounded by columnar or cuboidal ameloblast like cells surrounding more loosely arranged epithelial cells. Supporting stroma tends to be loosely arranged and vascular. It was also composed of many small discrete island of tumor composed of peripheral layer of cuboidal or columnar cells whose nuclei were generally well polarized. This closely resembles ameloblasts or preameloblasts. Central parts of follicles were showing stellate reticulum like cells. Cyst formation is relatively common in this type (Figs. 7 and 8). Histopathological ndings were suggestive of both plexiform and follicular ameloblastoma. A nal diagnosis of plexiform and follicular ameloblastoma was made with respect to the above ndings.

3.

Differential diagnosis

The rst in the clinical differential diagnosis was Odontogenic Keratocyst (OKC) commonly occurs in mandibular posterior region, develop during 2nd and 3rd decade of life, it has tendency to grow in anterior posterior direction with in medullary cavity without causing obvious bone expansion, aspiration may reveal thick, yellow and cheesy material. The next entity was considered central giant cell granuloma typically occurs anterior to mandibular rst molar and often cross the midline affects mostly adolescents and young adults. The next entity that was considered was Calcifying epithelial

odontogenic tumor (CEOT) as this too has a predilection for the age range of 8e92 years with average age of 42 years and for the posterior areas of the mandible (premolar molar area). (Table 1) As radiological differential diagnosis, rst entity was considered Odontogenic keratocyst (OKC), contains curved septa but usually grow with in medullary cavity without causing obvious bone expansion and rarely causing root resorption. Next, Central giant cell granuloma (CGCG) was considered that presents with multilocular radiolucency and often shows the resorption of the root surfaces of the adjacent teeth having more granular and ill dened septa. The next entity was considered Odontogenic Myxoma. In the early stage it has an osteoporotic appearance, consisting of multilocular radiolucency with well-developed locules. In the second stage of break-out or in the destructive phase, it is characterized by loss of locules with signicant expansion. These lesions may cross the midline and may cause root resorption and tooth displacement.10e13 (Table 2) The microscopic differential diagnosis may include ameloblastic broma and squamous odontogenic tumor. Microscopic appearance ameloblastic broma shows cords and thin strands of odontogenic epithelium resembling the dental lamina, cap and bell stages of early odontogenesis. Fibroblasts and embryonic connective tissue form the background. Squamous Odontogenic tumor consists of elongated and round islands of normal looking stratied squamous epithelium against a cellular brous connective tissue background. Epithelial islands may vary in size which has a basal cell layer of inactive appearing cuboidal cells. Some islands are composed of matured intermediate cells with prominent desmosomal bridges. Many epithelial islands have central areas of microcyst formation.14,15

4.

Discussion

Fig. 4 e Mandibular occlusal projection.

Ameloblastoma is a tumor originated from the epithelium involved with the formation of teeth. It has aggressive behavior and recurrent course, but it is rarely metastatic. Ameloblastoma represents 1% of all tumors and cysts that involve maxillomandibular area and about 10% of odontogenic tumors. Ameloblastoma can be classied in three types, considering clinical and radiographic features: solid or multicystic (86% of cases), unicystic (13% of cases) and peripherical (1% of cases).16 Based mainly on the clinical behavior

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Fig. 6 e Axial CT images demonstrated well dened multilocular expansile lesion with destructive changes.

and prognosis, three types of ameloblastoma can presently be distinguished: the conventional or classical, intraosseous, solid or multicystic ameloblastoma, the unicystic ameloblastoma, and the peripheral ameloblastoma.5 Ackerman et al, in their study of unicystic ameloblastomas, dened three subgroups. Group I (42%) consisted of a unilocular cyst with a nondescript but variable epithelial lining. Inactive odontogenic cell rests might be present in the brous wall, but there was no inltration by neoplastic epithelium. Group II lesions (9%) featured intraluminal plexiform proliferation but no inltration of the cyst wall. In Group III lesions (49%), plexiform or follicular d type ameloblastoma, sometimes in continuity with the cyst lining, inltrated the wall. Group III lesions need to be treated more aggressively similar to solid/multicystic Ameloblastoma.17 Sirichitra V and Dhiravarangkura P in 1984 studied 147 cases of intrabony ameloblastoma and found that there was no direct correlation between the histologic patterns and the radiographic appearances of the tumors but there is a slight indication that both monocystic and polycystic types tend to have the plexiform pattern, while the soap-bubble type tends to have the follicular pattern.18 Ameloblastoma in the mandible can progress to great size and cause facial asymmetry, displacement of teeth, loose teeth, malocclusion, and pathologic fractures. Tumor size may ranges from 1 to 16 cm at presentation which result

from expansion of bone and invasion into soft tissue. Typically ameloblastoma present as painless slow growing mass.1 The tumor is usually asymptomatic, grows slowly and small lesions can be detected only by routine radiographic examinations. Recently, the World Health Organization (WHO) considered the desmoplasic ameloblastoma not only a histological variant, but a clinic variant of this tumor itself.2 There are a lot of histological subtypes of ameloblastoma: plexiform, follicular, unicystic, basal cells, granulous cells, clear cells, acantomatous, vascular and desmoplastic. The most common histological patterns are plexiform and follicular. There are no data that prove the relation of the subtypes with clinical course.16 On histology, the follicular variant exhibits islands and sheets of stellate-shaped cells with central cyst formation. The plexiform subtype consists of anastomozing cords of stellate-shaped tumor cells with two to three layers of ameloblast-like cells peripherally. Various less frequent histologic subtypes are also described but these (as well as the follicular and plexiform subtypes) have little clinical signicance.4 The majority of patients with ameloblastoma are asymptomatic and the symptoms appear with the tumoral expansion.16 Follicular ameloblastoma presents as a painless swelling or slow expansion of the jaws, and it is described as multilocular expansile radiolucency that occurs

Fig. 7 e Follicular type.

Fig. 8 e Plexiform type.

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Table 1 e Clinical differential diagnosis. Features

Incidence Age Sex M: male F: female Sign and symptoms Site

Ameloblastoma
11% of odontogenic tumors 3rd to 4th decade of life M>F 1.1:1 Painless swelling and slow growth Mandible posterior ramus

OKC
7e11% of odontogenic cysts 2nd to 3rd decade of life M>F 1.7:1 Painless swelling Mandible molar ramus

CGCG
<7% benign lesions of jaw <20 years F>M

CEOT
0.4e3% of odontogenic tumors 8e92 years 36.9 years M>F 6:5 Painless swelling and slow growth Mandible: Maxilla 2:1 Premolar molar region

Aggressive: pain, swelling, rapid growth. Mand > Max. Anterior to 1st molar May cross midline

most frequently in mandibular molar/ramus area.5 Several extensive surveys and reviews of ameloblastoma have been published, the most notable being those of Robinson in 1937 and Small and Waldron in 1955. Analysis of more than 1000 cases by the latter authors revealed that the ameloblastoma most commonly occurs in the 20e49 years age, with average age at rst diagnosis being about 39 years.17 Kim S.G et al compared the clinical, radiologic, and histopathologic features of 71 intraosseous ameloblastomas. Data with respect to the patients ages, sex, tumor locations, and surgical treatment history, as well as the radiographic ndings and number of recurrences, were analyzed. They found 39 (54.9%) of the 71 subjects were males, and 32 (45.1%) were females. 62 (87.3%) of the 71 ameloblastomas were located in the mandible. Swelling was the most common symptom and was experienced by 27 (38.0%) patients. Radiographically, 42 (59.2%) of the 71 tumors were unilocular with a welldemarcated border. Of the remaining 29 cases, 14 were multilocular, 2 were of soapbubble shape, and 13 were unknown in appearance. The most common histologic pattern was plexiform, rather than follicular or acanthomatous.19 In the case reported here, the patient was male and was in fourth decade of his life. Clinically, it manifests in anterior part of mandible initially as a painless small swelling which gradually increased in size. Histopathological features revealed both follicular as well as plexiform ameloblastoma which adds to the unique case till reported in literature. Ameloblastoma was known for its high recurrence rate if excision was incomplete. Therefore the treatment of choice is surgical excision with wide free margins.1

Bachmann and Linfesty (2009) reported a solid/multicystic type ameloblatoma that showed a variety of histologic types, with plexiform and follicular predominating and was treated with a partial resection of the mandible.20 The traditional approach for a mandibulectomy is through a lipsplitting incision and though it has the disadvantage of postoperative morbidity; it gives a better exposure for complete tumour removal. Shirani et al (2007) in a series of 7 patients introduced a new technique of removal of large ameloblastoma with immediate reconstruction by using only an intraoral incision. It has the advantages of removing and repositioning of the mandible intraorally and therefore allows removal of the lesion and reconstruction procedure to be done simultaneously. Facial scar and damage to the marginal mandibular nerve that innervate the lips can also be avoided via this technique. Eppley (2002) in his review of 60 mandibular ameloblastoma cases have shown that there was no recurrence of those cases treated via en bloc resection as compared to enucleation and curettage in which the recurrence rate was as high as 25%e50%. Reconstruction of large mandibular defects represents a challenge to head and neck reconstructive surgeons. There are different methods of mandibular reconstruction for large defect. Among all, microvascular surgery has become the preferred option. Four donor sites i.e., bula, iliac crest, radial forearm, and scapula have become the primary sources of vascularized bone and soft tissue for the oral reconstruction. Among all these, bula has multiple advantages including bone length and thickness, donor site location permitting ap harvest simultaneously with tumor resection because both teams are at different end of the table, and

Table 2 e Radiological differential diagnosis. Features

Periphery and shape Borders/margins Cortical expansion Internal structure

Ameloblastoma
Smooth, oval Well dene and often curved Both buccal and lingual Multilocular/unilocular Honeycomb/soap-bubble 38% Extensive

OKC
Smooth, round or oval Well corticated unless sec. infected, scalloped Minimal expansion, rare Radiolucent: common, Multilocular: curved internal septa Rare

CGCG
Irregular/double boundary Well dened smooth and scalloped Uneven expansion both buccal and lingual perforation Multilocular, thin more granular and ill dene septae e Profound, irregular outline

Odontogenic myxoma
Irregular Mand: well corticated Max: poorly dened Both buccal and lingual Multilocular well develop locules Tennis racket, step ladder e Rare

Associated with impacted tooth Root resorption

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minimal donor site morbidity and therefore should be considered as a choice in reconstruction. Another method of reconstruction is internal distraction osteogenesis as has been ndor popularized by McCarthy et al (1992).1 Clokie and Sa (2008) described latest technique using bioimplant containing BMP-7, they included 10 patients with major mandibular defects following resection of biopsy-proven ameloblastoma lesions or osteomyelitis of the mandibular body or ramus. The resection defects were spanned with rigid reconstruction plates to hold the remaining mandibular segments in the correct position. The defects were lled with a bioimplant containing bone morphogenetic protein-7 (BMP-7) in a demineralized bone matrix (DBM) suspended in a reverse-phase medium to effect sustained BMP delivery. Radiographic evidence of mandibular bone formation was found in all cases and at the end of 1 year, functional and esthetic reconstruction of the mandible was complete.21 However, ameloblastomas that appear as unilocular lesions radiographically may be treated conservatively (i.e., with enucleation or curettage or both) whenever all areas of the cystic lumen can be controlled intraoperatively. Supraperiosteal resection of the bone is necessary when extensive thinning or perforation of the cortical plates is noted. Chemotherapy and radiation seem to be contraindicated. Postoperative follow-up is important in the management of ameloblastoma.19 Present case was treated with En bloc resection followed by reconstruction of deformity with allograft under general anesthesia.

5.

Conclusion

Ameloblastomas are an enigmatic group of oral tumors. Ameloblastomas not only occur in the posterior region, but can also occur in anterior region of the jaw. Since variants of ameloblastoma differ in biologic behavior, the histopathological examination is essential along with instrumental examination which is of signicance to the clinician for effective treatment plan as well as to prevent recurrence.

Conicts of interest
All authors have none to declare.

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