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Case Report
Extrafollicular Adenomatoid odontogenic tumour presenting as a periapical lesion: Report of a rare entity Manveen Kaur Jawanda a,*, Kumar Rakshak Anand b, Nidhi Mathur c, Priya Gupta a, Shruti Jain a a
Department of Oral and Maxillofacial Pathology, Luxmi Bai Institute of Dental Sciences and Hospital, Patiala, Punjab, India Department of Oral and Maxillofacial Surgery, ITS Dental College and Hospital, Ghaziabad, India c Department of Pathology, Luxmi Bai Institute of Dental Sciences and Hospital, Patiala, Punjab, India b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 20 July 2013 Received in revised form 5 January 2014 Accepted 6 January 2014
Adenomatoid odontogenic tumour (AOT) has been known to the pathologists for the past 100 years as a benign slow growing tumour. Histologically, this tumour has a predominantly solid component and very few case reports have described the cystic nature of AOT. In the present case we review the literature of extrafollicular AOT presenting as a periapical disease and add to the literature a case of cystic extrafollicular AOT in a 17-year-old patient, initially diagnosed as a radicular cyst. Radiographically extrafollicular AOT frequently resembles other odontogenic lesions such as residual, developmental lateral periodontal or radicular cyst, however histopathological findings of this tumour are quite characteristic and present few diagnostic problems. Correlation of clinical and radiological information with histological features is important in the diagnosis of odontogenic cysts and tumours, to decrease the incidence of misdiagnosis for such cases and this in turn will help in preventing recurrences. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Extrafollicular Adenomatoid odontogenic tumour Cystic Adenomatoid odontogenic tumour Extrafollicular cystic variant of Adenomatoid odontogenic tumour Periapical cyst
1. Introduction The odontogenic cysts and tumours are a diverse group of lesions that represent deviation from normal odontogenesis. Adenomatoid odontogenic tumour (AOT) is a rare benign epithelial lesion of odontogenic origin, constituting only 3% of all odontogenic tumours and often diagnosed as odontogenic cyst [1]. The first reported case which meets the diagnostic criterion was reported by Steensland in 1905 as ‘Epithelioma adamantinum’. It was first described by Dreibaldt in 1907 as a Pseudoadenoameloblastoma [2]. It was Stafne in 1948, who considered it as a distinct entity [3]. Philipsen and Birn [4] introduced the term, Adenomatoid odontogenic tumour in 1969 and defined the lesion as, ‘‘A tumour of odontogenic epithelium with duct-like structures and with varying degrees of inductive change in the connective tissue. The tumour may be partly cystic, and in some cases the solid lesion may be present only as masses in the wall of a large cyst.’’ Adenomatoid odontogenic tumour is regarded by some authors as a true benign, non aggressive and non invasive neoplasm and by yet others as a cyst that has a hamartomatous intraluminal proliferation of epithelial cells derived from Hertwig’s epithelial root sheath. This epithelial proliferation at times may fill the lumen to give the impression of a solid tumour [5].
* Corresponding author at: H. No. 9, Doctor’s Colony, Bhadson Road, Patiala 147001, Punjab, India. Tel.: +91 9417211750. E-mail address:
[email protected] (M.K. Jawanda).
AOT is a heterogenous group of lesion existing either as cystic or solid variant. Two third of this tumour is associated with an unerupted tooth, and very rarely the lesion occurs without any impacted tooth. This case report describes characteristic clinical and radiographic features of a rare Extrafollicular AOT mimicking a periapical lesion and presenting as a cyst histologically. 2. Case report A 17-year-old Indian male with a non contributory medical history was referred for diagnosis of a periapical lesion localized between the roots of right maxillary lateral incisor and canine (Fig. 1). The asymptomatic lesion was detected during routine radiographic examination in preparation for orthodontic treatment. The lesion gradually progressed to attain its present size in three months duration with no history of pain, discharge or numbness. Patient gave a vague history of trauma since three years. Clinical examination did not show any coronal or endodontic pathologic condition of teeth 12 and 13, both of which reacted positively to electrometric pulp testing. A Panoramic radiograph revealed a well-defined, oval pear shaped radiolucent lesion approximately 1.0 cm 1.0 cm in size seemingly involving the periapical area of 12 and 13 (Fig. 1). Based on the history and clinical features, provisional diagnosis of periapical cyst and globullomaxillary cyst were given. An exploratory surgical approach was chosen and the mass was enucleated and the cyst separated easily from the adjoining bone.
1871-4048/$ – see front matter ß 2014 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.pedex.2014.01.001
Please cite this article in press as: M.K. Jawanda, et al., Extrafollicular Adenomatoid odontogenic tumour presenting as a periapical lesion: Report of a rare entity, Int. J. Pediatr. Otorhinolaryngol. Extra (2014), http://dx.doi.org/10.1016/j.pedex.2014.01.001
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Fig. 1. Panoramic radiograph along with clinical picture of the patient.
Fig. 2. Low-power view of the cystic space and solid area (AOT) (haematoxylin and eosin, 4). (B) Corresponding to the macroscopic observation (A) of the cut section of the specimen.
The gross soft tissue specimen received was greyish brown in colour and cut surface showed a cystic space. Two distinct regions could be identified within the cut sections of the specimen, cystic space and solid tumour areas (Fig. 2A). The cystic region was brownish in colour and contained a little brownish fluid. The solid tumour part had an oval circinate appearance and resembled Tapioca. Corresponding with the gross observations, two parts (Cystic space and solid tumour nodules) could be identified microscopically (Fig. 2B). Cystic area was composed of dense fibrous tissue lined by one to three layers of cuboidal cells. Furthermore, this lining of the cyst was in continuity with the intraluminal and intramural proliferations exhibiting sheets of tumour cells (Fig. 3A). The sheets of tumour proliferation were chiefly composed of spindle shaped and polygonal cells with areas of duct like
structures lined by single layer of tall columnar ameloblast like cells, with the nuclei polarized away from the luminal surface (Fig. 3B). A thin eosinophilic rim of varying thickness was seen in the lumen of several ducts like structures. Few areas of the cystic lining showed an irregular arcading pattern of stratified squamous epithelium which resembled a radicular cyst (Fig. 3A). Spherical calcific deposits were noted intraepithelially in cystic lining (Fig. 4). Areas of large amount of eosinophilic matrix material were seen in the stroma, consistent with dentinoid, among the sheets of epithelial cells (Fig. 3A). Based on these features, the lesion was diagnosed as Adenomatoid odontogenic tumour, concurrent with Extrafollicular cystic variant of Adenomatoid odontogenic tumour. The postoperative course was uneventful. One year of follow up has not shown any evidence of recurrence.
Please cite this article in press as: M.K. Jawanda, et al., Extrafollicular Adenomatoid odontogenic tumour presenting as a periapical lesion: Report of a rare entity, Int. J. Pediatr. Otorhinolaryngol. Extra (2014), http://dx.doi.org/10.1016/j.pedex.2014.01.001
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Fig. 3. (A) One to three cell thick cystic epithelium proliferating in a lace like pattern along with cystic epithelium showing luminal and intramural proliferation of sheets of tumour cells. Subepithelial hyalinization resembling dentinoid among sheets of epithelial cells seen (haematoxylin and eosin stain, 10). (B) Magnified view showing ducts lined by tall columnar cells with nuclei polarized away from the lumen (haematoxylin–eosin stain, 40). (C) High power showing typical pattern of cuboidal or columnar epithelial cells forming nests of rosette like structures (haematoxylin–eosin stain, 40).
Fig. 4. Tumour nodules showing foci of aggregates of eosinophilic hyaline droplet material and calcifications (periodic acid-Schiff stain (A), 40, Masson trichrome stain (B), 40).
3. Discussion AOT is a very infrequent lesion as compared to other pathologic processes of the oral & maxillofacial region, as some studies have shown that these represent only 0.1% of tumours and cysts of jaws and 3% of all odontogenic tumours [3]. AOT is an epithelial tumour with an inductive effect on odontogenic ectomesenchyme. An extensive review by Philipsen et al. [6] based on 499 cases from various international case studies describes three variants of AOT
(Table 1). The follicular type is more common as compared to extrafollicular variant. Philipsen et al. [7] postulated that follicular type of AOT developed from rests of cells within the dental lamina, and therefore as a result surrounds the tooth. Furthermore, the development of extrafollicular AOT may proceed when the epithelial rests, which give rise to the tumour, are located at the periphery of the path of the tooth eruption. The tooth will, therefore, not be impeded in its eruption, by the developing tumour, and the AOT will not be associated with the tooth. The
Please cite this article in press as: M.K. Jawanda, et al., Extrafollicular Adenomatoid odontogenic tumour presenting as a periapical lesion: Report of a rare entity, Int. J. Pediatr. Otorhinolaryngol. Extra (2014), http://dx.doi.org/10.1016/j.pedex.2014.01.001
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Table 1 Radiographic appearance of Adenomatoid odontogenic tumour variants. Adenomatoid odontogenic tumor Intraosseous variant (97% of all AOTs) Follicular type (73%) Tumour is located around the crown and often including part of root of an unerupted tooth – Envelopmental D/D: Dentigerous cyst
Extraosseous variant (3% of all AOTs) Peripheral or epulis type Extrafollicular type (24%) Tumour not associated with a unerupted tooth With slight erosion of bone crest (a) Without relation to tooth structure neither erupted or unerupted. (b) Inter-radicular: Adjacent roots diverge apically due to tumour expansion. D/D: Gobullomaxillary cyst (c) Superimposed at root apex. D/D: Periapical lesion (d) Superimposed at mid-root level. D/D: lateral periodontal cyst
Table 2 Reported cases of extrafollicular AOT mimicking radicular cyst.
1. 2. 3. 4. 5. 6. 7. 8. 9.
Authors
Gender
Age
Site
Bernier and Tiecke, 1956 [8] Abrams, Melrose, and Howell, 1968 [9] Awazawa, 1972 [10] Giansanti, Someren and Waldron, 1970 [11] Philipsen et al., 1992 [7] Curran, Miller and Murrah, 1997 [12] Philipsen et al., 2002 [13] Kumar et al., 2010 [14] Our case
F M F F F F F F M
26 16 23 27 17 23 15 17 17
Lower Upper Lower Lower Upper Lower Upper Lower Upper
Race left canine right central incisor right lateral incisor, canine and first premolar right second and third molar left canine left lateral incisor and canine right cental and lateral incisor left lateral incisor, canine and Ist & IInd Premolar lateral incisor and canine
White White Asian White Asian African-American Asian Asian Asian
Table 3 Unusual findings seen in our case compared to typical features of an Adenomatoid odontogenic tumour (AOT). Typical features of most AOTs
Our case
Follicular variant more common as compared to extrafollicular variant (73% of all intraosseous AOT’s) Female predilection (F:M = 2:1) Unilocular radiolucent area with radiopaque specks Histopathological features presents as a solid tumour mass of odontogenic epithelium with duct-like structures and with varying degrees of inductive change in the connective tissue.
Extrafollicular variant (24% of all intraosseous AOT’s)
lesion then presents radiographically as a residual, developmental lateral periodontal or radicular cyst, depending on the location of the rests. In the reported case, we presume that the neoplastic epithelium must have arisen from the remnants of odontogenic epithelium, which were present within the bone. A systematic search of the English-language medical literature revealed only a total of 8 cases, of the extrafollicular variant of the AOT mimicking a periapical lesion. The clinical characteristics of these 8 cases and the current case are summarized in Table 2 [7– 14]. Our case report illustrates characteristic clinical & radiographic features of the extrafollicular variant of the AOT mimicking a periapical lesion. There were, in fact, a number of clinicoradiologic indicators that could have suggested that this case in a 17-year-old male was a possible extrafollicular AOT mimicking radicular cyst, although this subvariant is indeed a rarity. The age distribution of patients with AOT shows a peak in the second decade [6], the female sex is almost twice as often affected as the male [2]. The incisor/canine region of the upper jaw is often the site of an extrafollicular AOT [2], in which the tumour produces a slowly enlarging swelling. A distinct radiopaque border of unilocular radiolucency is typical of radiographic appearance of AOT [2], but in our case the periodontal ligament and lamina dura were not found to be intact around involved teeth, an important finding that should make a periapical radiolucent lesion such as periapical cyst and granuloma, more likely [7]. In addition to these, our case
Male Unilocular radiolucent area without radiopaque specks Histopathological examination displayed cystic configuration with a central cavity lined, in part, by stratified squamous epithelium. Focally, the cyst lining was interrupted by intraluminal and intramural proliferations of AOT nodules (Fig. 3A).
history of trauma favours it to be more likely, a radicular cyst. The histological features of a classical AOT are characteristic and present a few diagnostic problems. The clinical appearance and radiological features of the present case gave an impression that it was a radicular cyst, however, its histopathological features proved otherwise (Table 3). AOT’s are usually solid but are occasionally cystic. Cystic presentation of AOT has been reported way back in 1915 by Harbitz, who reported the lesion as ‘‘Cystic Adamantoma’’ [15]. Gadewar and Srikant [16] reviewed the literature for AOT’s arising in an odontogenic cyst and provided an insight to the varied demographic profile, clinical behaviour and prognosis of cystic variant of follicular AOT, but very few case reports have described the cystic variant of extrafollicular AOT. As noted from the macroscopic and microscopic observations, the tumour portion in the current case is continuous with the cystic lining and protrudes into the lumen it created. The cystic component of AOT has been variedly termed as dentigerous cyst [17,18], calcifying odontogenic cyst [19,20] or unicystic ameloblastoma [21] but the classification of the cystic lining into a known odontogenic cyst or to categorize the cyst as a component of AOT per se is open to conjecture. It is a known fact, that, tumours without blood supply are unable to grow more than about one millimetre across. So, as tumour grows, the cells in the middle of the tumour mass start degenerating. So, it is reasonable to assume that extrafollicular AOT, in initial stage are
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solid and as tumour grows, cystic degeneration results into a cystic component. So, whether the cystic variant of extrafollicular AOT is a true cystic lesion or is a result of cystic degeneration is open for debate. Nodule formation in a cyst lining is well known in odontogenic cysts (e.g. lateral periodontal cysts, glandular odontogenic cysts). This feature was also observed in case reports by Zhang et al. [20], Nonaka et al. [18], and Jivan et al. [21]. The presence of AOT in nodules in cyst lining of our present case, gives a histological proof that, solid extrafollicular AOT has transformed into a cystic lesion. Curran et al. [12] described an extrafollicular AOT that presented with periapical disease and had histological features that reveal a cystic configuration with a central cavity lined by stratified squamous epithelium that is interrupted by the growth of the AOT. The presence of two distinct epithelial odontogenic patterns [12] [our present case], stratified squamous epithelium mimicking a radicular cyst and odontogenic epithelium composed of AOT nodules, in a single lesion is probably not a collision of cyst and a tumour, but instead are expression of the histodifferentiation potential of the odontogenic epithelium from which these lesions are derived. This assumption is supported by the finding of both histological types in the epithelial lining of the same cystic cavity. We assume that AOT always had a cystic component along with the solid tumour but only very few case reports have described the cystic lining and we agree with Marx and Stern [22] who stated that the lesion presents as a cyst with intraluminal proliferation which fills the cystic space giving a solid appearance and proposed the term ‘‘Adenomatoid odontogenic cyst.’’ Given that the number of reported cases till date is so small that we are unable to speculate whether AOT is a cyst or a tumour. This single case report cannot answer this question; however further investigation into this possibility would take place as more cases of cystic AOT occur. 4. Conclusion The clinical significance of this case is that though radiological observation appeared to be simple, unambiguous and obvious, however the histopathological observation showed that it was a rare odontogenic lesion. There is an important need to report similar and other such cases, as we feel that many cases are surgically managed but unfortunately not reported. All such cases should be reported, so that we can increase not only our local literature bank, but also play a positive contribution to further clarify the biologic behaviour of this particular subvariant, which is very rare indeed, being the least reported presentation of the extrafollicular variant.
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