Adenomatoid odontogenic tumor in association with dentigerous cyst: An enigma

Sonam Sharma; Amita Sharma

doi:10.4103/jorr.jorr_16_21

CASE REPORT

Year : 2022 | Volume : 14 | Issue : 1 | Page : 44-49

Adenomatoid odontogenic tumor in association with dentigerous cyst: An enigma

Sonam Sharma¹, Amita Sharma²
¹ Department of Pathology, Kalpana Chawla Government Medical College, Karnal, Haryana, India
² Department of Dentistry, SHKM Government Medical College, Mewat, Haryana, India

Date of Submission	01-Mar-2021
Date of Acceptance	10-Nov-2021
Date of Web Publication	04-Jan-2022

Correspondence Address:
Sonam Sharma
Department of Pathology, Kalpana Chawla Government Medical College, Karnal, Haryana
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jorr.jorr_16_21

Abstract

The odontogenic tissue surrounding an impacted tooth is a harbinger of a wide variety of odontogenic cysts and tumors. As these hamartomatous and neoplastic aberrations can occur at any stage of odontogenesis, therefore combined features of odontogenic tumors with epithelial and mesenchymal components can develop within the odontogenic cysts. One of the odontogenic cysts is the dentigerous cyst (DC) which is not only the most common developmental odontogenic cyst arising from the pericoronal tissue but is of great clinical significance owing to its neoplastic potential and association with other rare odontogenic tumors. We herein describe an unusual case of adenomatoid odontogenic tumor which developed secondary to a DC in a 22-year-old female and created a diagnostic conundrum. The approach to such a case, differential diagnosis, and review of the literature is also presented.

Keywords: Adenomatoid odontogenic tumor, benign, dentigerous cyst, lateral incisor, maxilla

How to cite this article:
Sharma S, Sharma A. Adenomatoid odontogenic tumor in association with dentigerous cyst: An enigma. J Oral Res Rev 2022;14:44-9

How to cite this URL:
Sharma S, Sharma A. Adenomatoid odontogenic tumor in association with dentigerous cyst: An enigma. J Oral Res Rev [serial online] 2022 [cited 2022 Jan 19];14:44-9. Available from: https://www.jorr.org/text.asp?2022/14/1/44/334822

Introduction

Adenomatoid odontogenic tumor (AOT) is an uncommon, benign epithelial lesion of odontogenic origin, which accounts for 2.2%–7.1% of all the odontogenic tumors.^[1] Till date, various authors have given multiple terminologies for this neoplasm. It was first described by Steensland et al. in 1905 as “epithelioma adamantinum.”^[2] In the year 1907, Dreibladt et al. used the term “pseudoadenoameloblastoma” for it.^[3] Harbitz et al., in 1915, described its cystic presentation and reported it as “cystic adamantoma.”^[4] However, Staphne in 1948 was the first to recognize this as a distinct pathological entity.^[5] It was until 1969, that Philipsen and Birn proposed the name AOT, which was later widely accepted and adopted by the World Health Organization (WHO) to classify odontogenic tumors in the year 1971.^[6] Nevertheless, recently, it has also been referred to as “two-third tumor” because two-third of the cases occur in the maxilla, two-third involve the young women, two-third are associated with an unerupted tooth, and two-third have been found to arise from the canine area.^[7] Philipsen et al. subdivided AOT into three groups, namely follicular, extrafollicular, and peripheral. They all share a similar histologic characteristic that indicates a common origin as derived from the complex system of dental lamina or its remnant embedded in the gubernacular dentis.^[8] Both the follicular and extrafollicular variants occur intraosseously, accounting for 96% of all AOT and of these, 71% is follicular variant.^[9] The follicular variant is predominantly associated with the crown and often part of the root of an unerupted tooth, most commonly the maxillary canine. Therefore, 77% of follicular variant is often mistaken as dentigerous cyst (DC) based on the clinical and radiographic findings.^[10] On the other hand, the extrafollicular variant has no relation with any particular teeth and often resembles a residual, radicular, globulomaxillary, or a lateral periodontal cyst. The peripheral variant is the least common and occurs extraosseously. It generally appears as a gingival fibroma or an epulis.^[11] Very rarely, AOT can be associated with other odontogenic lesions, such as DC, calcifying odontogenic cyst, odontoma, ameloblastoma and may pose a diagnostic challenge. Paucity of knowledge about this unusual association and debates over its controversial existence prompted us to report a rare case of AOT arising within a DC in a young female who was clinically misdiagnosed as a case of DC.

Case Report

A 22-year-old female reported to the outpatient department of dentistry with a chief complaint of slowly progressive painless swelling over left front side of upper jaw causing disfigurement of the face, for the past 2 years. Her medical and family history was noncontributory. Her general physical and systemic examination revealed no abnormality. On extraoral examination, a diffuse swelling measuring approximately 2.5 cm × 2.5 cm extending from left infraorbital region superiorly to the left corner of the mouth, obliterating the nasolabial fold was seen. On palpation, it was firm in consistency, nontender and the overlying skin was normal. Intraoral examination showed a soft fluctuant swelling with normal mucosa, extending from the left maxillary central incisor to the second premolar region, obliterating the labial vestibule. The left deciduous lateral incisor was retained while the left permanent lateral incisor was missing. Radiographic findings with the help of intraoral and extraoral X-rays (orthopantomogram) confirmed a well-defined, unilocular radiolucency extending from the left maxillary central incisor to the second premolar region with an impacted permanent lateral incisor [Figure 1]. Fine needle aspiration cytology of the swelling was performed which yielded straw colored fluid. On the basis of clinical, radiological, and cytological findings, the lesion was provisionally diagnosed as a DC. Her routine hematological, microbiological, and biochemical investigations were within normal limits. Under local anesthesia, the retained left deciduous lateral incisor was extracted and surgical enucleation of the lesion was done. The resected specimen was sent for histopathological evaluation. Her postoperative recovery was uneventful.

Figure 1: Preoperative orthopantomogram exhibiting radiolucency in relation to an unerupted left lateral incisor

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On histopathological examination, grossly, a gray brown, globular soft tissue piece measuring 2 cm × 2 cm with an impacted tooth was seen [Figure 2]a. The cut section revealed a cystic cavity with few solid areas and a fully formed crown of the tooth embedded within it and the stunted angulated root lying outside [Figure 2]b. Microscopic sections showed a cyst wall lined by few layers of nonkeratinized epithelium without rete ridges, resembling reduced enamel epithelium. The epithelium at places exhibited whirling and cell rich nodules, duct and rosette-like pattern with proliferating interlacing cords. The duct-like structures were composed of a central space surrounded by cuboidal to columnar cells forming small nests. The nuclei of these cells showed reverse polarization. Some of these central spaces were filled with eosinophilic material. Areas of calcification were also evident within the epithelium. The underlying connective tissue showed loosely arranged collagen fibers, fibroblasts, thin congested blood vessels, myxoid areas, and few epithelial nodules [Figure 3], [Figure 4], [Figure 5]. Based on these histomorphological features, a final diagnosis of AOT arising within the DC was made. The patient is still under regular follow-up and has shown no signs of recurrence over the past 1-year postoperative period.

Figure 2: (a) Enucleated specimen showing soft tissue extending beyond the cementoenamel junction of an impacted tooth (b) Both cystic space and solid tissue along with an impacted tooth on cut section

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Figure 3: Photomicrograph showing dentigerous cyst lining and AOT origin (H and E, ×40)

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Figure 4: (a) Nonkeratinized cystic lining epithelium with fibrocollagenous tissue wall, characteristic of dentigerous cyst (H and E, ×100), (b) Solid masses of cells in areas of connective tissue capsule (H and E, ×100), (c) Odontogenic epithelial cells arranged in form of islands and duct-like pattern in scanty connective tissue stroma (H and E, ×100), (d) Nodules of variable sizes with calcified material (H and E, ×100)

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Figure 5: (a) Solid proliferation of polygonal and spindle-shaped cells with rosette-like structures and a central space consisting of eosinophilic hyaline aggregates with calcification in fibrous stroma (H and E, ×200), (b) Higher magnification of nodules (H and E, ×400), (c) Eosinophilic hyaline droplet material (H and E, ×400), (d) Spindle-shaped cells in between the odontogenic epithelial cell islands (H and E, ×400)

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Discussion

AOT is one of the most intriguing odontogenic lesions, which has been regarded as a hamartoma, a benign neoplasm, a developmental anomaly, or a cyst.^[12] Various clinical, radiological, and pathological features of this rare entity warrant an attention. The age range for its occurrence varies from 3 to 82 years, with a very tall peak in the second decade of life and over 50% AOT usually involve the adolescents between the ages of 13 and 19 years. It shows female preponderance with female: male ratio being 2:1, nevertheless, an even higher female predominance of approximately 3:1 is seen in certain Asian population.^[13] The size of AOT ranges from 1.5 to 6.0 cm and it has a striking tendency to arise in the anterior maxilla, nevertheless, other areas of the jaws can also be involved, such as the sinus, the posterior maxillary, and anterior mandibular regions. It is frequently associated with an impacted canine followed by premolars, incisors, and molars.^{[9],[14],[15]} The present case had similar clinical findings, as the patient was a female in the second decade with a 2.5 cm × 2.5 cm lesion in the anterior maxilla associated with an impacted lateral incisor.

The pathogenesis behind AOT still remains a subject of speculations. Some authors have mentioned that its origin is from the odontogenic cysts especially DC and have even suggested the term “hybrid variant” for AOT arising from these cysts.^[16],[17] On the other hand, few researchers have believed that it could be derived from fully formed enamel organ, dental lamina system complex, and/or its epithelial remnants. AOT grows into a nearby or next to a dental follicle while forming a cystic space as proposed by the “envelopmental” theory.^[18],[19] The present case fulfilled both the envelopmental pathogenesis and the “hybrid variant” concept. However, whether the origin of follicular variant of AOT occurs before or after the cystic expansion is still debatable. If the tumor grows after cystic expansion, then this confirms its origin from a DC, however, if it occurs before cystic expansion, then the tumor tissue fills the follicular space and the AOT presents as a solid tumor. However, it is reasonable to assume that, given enough time, even those originating from a cyst may grow and fill the lumen completely. Nevertheless, it cannot be ruled out that the DC with an impacted tooth developed first followed by development of AOT in the cyst wall.^[17]

According to the pertinent world literature, there are only a handful of AOT cases which have been reported in association with DC till date [Table 1].^{[20],[21],[22],[23],[24],[25],[26],[27],[28],[29],[30],[31],[32],[33],[34],[35],[36],[37],[38],[39],[40],[41],[42],[43],[44],[10],[14],[15],[16],[17]} On summarization of these documented cases, it was observed that the majority of them were young patients of Indian/Asian descent, therefore; a genetic basis of this association cannot be ruled out and requires further exploration.

Table 1: Literature review of adenomatoid odontogenic tumor cases associated with dentigerous cyst (current case included)

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In such an unusual setting, it is emphasized to make a final diagnosis only after correlating the clinical presentation of the patient with the radiological, cytological, and histopathological findings of the resected specimen as one might find it extremely difficult to separate these two entities, based on only one diagnostic modality. It has been observed that the radiological features of AOT are nonspecific and it shares the characteristics with not only DC but also other odontogenic lesions, such as calcifying odontogenic cyst, unicystic ameloblastoma, and keratocystic odontogenic tumor. However, there are certain clues on radiology which can help in differentiating AOT from DC, which most frequently occurs as a unilocular radiolucency of variable size with well-defined borders and enclosing only the coronal portion of the impacted tooth, whereas AOT shows radiolucency usually surrounding both the coronal and radicular aspects of the involved tooth. However, in cases where AOT grows from DC, like in the present case, the radiographs are inconclusive. However, the irregularity in the wall of cyst and small calcified foci may indicate the development of AOT.^[17],[45] On aspiration, straw-colored fluid helps in differentiating DC from the solid tumor clinically and macroscopically follicular type of AOT sometimes extends apically beyond the cementoenamel junction (CEJ), while DC is attached to the tooth at the cervical region.^[19],[32] On cut section of the DC specimen a cystic space is revealed whereas that of an AOT shows a solid mass or a partly cystic space.^[17] Microscopically, the DC displays a thin fibrous cyst wall with a myxomatous appearance. The epithelial lining consists of 2–4 layers of flat or cuboidal cells, which in fact is the reduced enamel epithelium and is characteristically nonkeratinized. Nests, islands, or strands of odontogenic epithelium are often seen in the fibrous capsule. Localized proliferation of epithelial lining may occur in response to inflammation. Hyaline (Rushton) bodies may be found in the epithelium, especially in cysts exhibiting inflammation. Sometimes mucous secreting cells and rarely ciliated cells form a part of the epithelial lining, and occasionally sebaceous cells and lymphoid follicles with germinal centers are seen in the connective tissue.^[45] For AOT, varied histoarchitectural patterns have been documented and the WHO has defined AOT as “a tumor of odontogenic epithelium with duct-like structures and with varying degrees of inductive change in the connective tissue. The tumor may be partly cystic, and in some cases, the solid lesion may be present only as masses in the wall of a large cyst”.^[12],[27] In the present case too, aspiration revealed straw-colored fluid which initially triggered the diagnosis of DC. However, on gross examination, the soft tissue extended beyond the CEJ and the cut section showed both the solid and cystic areas which made us to consider an AOT. Histologically, the tumor consisted of a fibrous wall lined by squamous epithelium with few cell layers, as well as nodules of polyhedral cells amid small tubules of short columnar cells containing eosinophilic and mineralized material. These findings were consistent with other studies which demonstrate that AOT may originate from the cystic epithelium and protrude toward the lumen.^[28],[33]

The treatment of choice in such a case is conservative surgical enucleation or curettage, as both the AOT and DC are benign, encapsulated lesions and have a very less recurrence rate. More invasive treatment, such as partial block resection, should be indicated only in exceptional cases of large tumors or when there is a risk of bone fracture.^[28] Marsupialization of AOT lesions associated with DC is another option, which permits preservation of the teeth involved, followed by orthodontic repositioning.^[38] The prognosis of such cases is quite favorable, however, a long-term regular follow-up is mandatory as it is both interesting and difficult to comment upon the aggressive behavior of the AOT, although the oncogenic potential of DC is well-documented in the literature.^[45]

Conclusion

AOT is rare odontogenic neoplasm which is generally misdiagnosed, especially when it arises from or associated with a DC. Hence, it should be included as one of the important differentials, especially among the maxillary cystic lesions with an impacted tooth. Preoperatively, a combination of detailed clinical history, intraoral examination, cytological, radiological assessment aid in its diagnosis, but it is the meticulous histopathological evaluation which is confirmative. Nevertheless, more insight is required to understand the genesis and behavior of this “hybrid variant” of AOT in near future.

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.

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