Simultaneous adenomatoid odontogenic and keratocystic odontogenic tumours in a patient with Gorlin‐Goltz syndrome

Gorlin and Goltz described a syndrome in which multiple basal cell carcinomas, odontogenic keratocysts and bifid ribs occurred in combination. The jaw keratocysts are a consistent feature of ‘Gorlin‐Goltz’ or naevoid basal cell carcinoma syndrome. Central nervous system and ocular involvement occurred together with the fairly typical facial features of frontal bossing and hypertelorism. This case report documents the pathology associated with an impacted maxillary canine tooth in a boy with Gorlin‐Goltz syndrome. The patient presented for investigation of the failure of eruption of the right permanent maxillary canine tooth. Radiographic investigation showed the presence of a well circumscribed radiolucency located around the crown of an impacted right maxillary canine tooth. The patient's medical history revealed a medulloblastoma that was treated 13 years ago. The right maxillary canine tooth and associated peri‐coronal tissue were removed under general anaesthetic. A diagnosis of a keratocystic odontogenic tumour with an associated adenomatoid odontogenic tumour was made. The common differential diagnoses for a peri‐coronal radiolucency in the maxilla that need to be considered by dentists include a dentigerous cyst, follicular keratocystic odontogenic tumour and adenomatoid odontogenic tumour. A rare case of both keratocystic odontogenic tumour and associated follicular adenomatoid odontogenic tumour is described in a patient with naevoid basal cell carcinoma syndrome.     

Small Central Odontogenic Fibroma Mimicking Hyperplastic Dental Follicle and Dentigerous Cyst

Central odontogenic fibroma has been defined as a benign odontogenic tumor, representing the intraosseous counterpart of a peripheral odontogenic fibroma. The odontogenic fibroma is a rare tumor. Differential diagnosis of radiolucent lesions in the molar-premolar region of mandible which involve impacted tooth may include central odontogenic fibroma, hyperplastic dental follicle, dentigerous cyst, unicystic ameloblastoma, and keratocystic odontogenic tumor. We describe an example of a small central odontogenic fibroma mimicking hyperplastic dental follicle and dentigerous cyst, resulting in uneruption of a primary tooth.     

Epulides in the dog: a review

This article is based on a review of the literature and the study of pathology sections obtained from various veterinary pathology laboratories. Epulis is a non-specific, clinical designation for a localized, exophytic growth on the gingiva. Four reactive epulides occur in human beings, namely focal fibrous hyperplasia (fibrous epulis). pyogenic granuloma. peripheral giant cell granuloma (giant cell epulis. and peripheral ossifying fibroma (calcifying fibrous epulisl). The first three also occur in dogs but only focal fibrous hyperplasia appears to be common. The peripheral ossifying fibroma has not yet been reported in dogs. Odontogenic tumors occurring on the gingiva (i.e., as epulides) are referred to as peripheral odontogenic tumors. Three types have been reported in dogs. One, the common fibromatous epulis. is equivalent to the rare peripheral odontogenic fibroma in human beings. Another, the acanthomatous epulis. appears to be a form of ameloblastoma but differs from the peripheral ameloblastoma in human beings in that it invades bone; its biological behavior is therefore that of the human intraosseous ameloblastoma. The third, a rare lesion, has been referred to in the veterinary literature as a calcifying epithelial odontogenic tumor, although it is not the canine counterpart of the human CEOT The term, amyloid-producing odontogenic tumor , has been suggested as being appropriate for this lesion.     

周边型牙源性纤维瘤35例临床病理研究

目的 :总结周边型牙源性纤维瘤的临床病理特点。方法 :按WHO(1992 )牙源性肿瘤分类标准对 35例周边型牙源性纤维瘤的临床病理资料进行回顾性分析。结果 :35例中 ,男性 14例 ,女性 2 1例 ;平均年龄 31岁 ;上颌牙龈 14例 ,下颌牙龈 2 0例。肿块为局部软组织突出表现。X线片和手术中见五例牙槽骨有吸收改变。三例术后复发。组织学上 ,肿瘤由致密结缔组织组成 ,含数量不等的成纤维细胞 ,一例伴有颗粒细胞。肿瘤边界尚清 ,缺少明显包膜。结论 :周边型牙源性纤维瘤临床表现与龈瘤不能区分 ,组织学主要为WHO型牙源性纤维瘤 ,手术切除不彻底易复发。     

Central odontogenic fibroma current concepts

The author reviews current knowledge concerning the central odontogenic fibroma, which at present is incompletely understood, and reaches the following conclusions. 1) The separation of this lesion into simple and WHO types remains valid because they exhibit different histologic features. However, more care should be taken in rendering the diagnosis of the WHO type than in the past; unlike the simple type, it is a fibroblastic lesion. 2) Complex central odontogenic fibroma is a more appropriate term than the WHO type because the WHO does not use the latter term in its 1992 manual. 3) The microscopic distinction of simple odontogenic fibroma from desmoplastic fibroma remains difficult in some cases. 4) The granular cell odontogenic tumor, which has sometimes been referred to as a type of odontogenic fibroma, is a separate entity, although some simple odontogenic fibromas exhibit scattered granular cells. 5) The separation of lesions that have been reported recently as odontogenic fibromas with giant cell reactions from central giant cell granulomas that exhibit foci of odontogenic epithelium requires further study.     

Odontogenic fibroma like lesions: Clinico-pathological considerations

Radiographically enlarged dental follicle is observed in many cases associated with delayed tooth eruption. Pericoronal radiolucencies are seen in neoplasms (odontogenic fibroma), developmental anomalies (regional odontodysplasia), hamartomatous lesions (odontogenic epithelial hamartoma), opercula of third molars, in follicles associated with unerupted third molars, regional odontodysplasia, in dental follicles around impacted teeth of enamel dysplasia with hypodontia syndrome and amelogenesis imperfecta cases with multiple impactions. Interestingly, operculum and the follicle of these lesions histopathologically are identical to odontogenic fibroma (WHO) type. However, the lack of universally accepted clinic-pathological features for such lesions may hinder their recognition. We report a case of regional odontodysplasia wherein (in which) the impacted canine is surrounded by radiolucency measuring about 1.5–2 cm, histopathologic examination of the excised opercula revealed features reminiscent of central odontogenic fibroma (WHO type) with an abundance of odontogenic epithelium and calcifications. The aim of this paper is to discuss various lesions exhibiting histopathological features similar to odontogenic fibroma, as awareness of the clinicopathological features of such lesions is very important to plan proper treatment.     

Adenomatoid odontogenic tumor: biologic profile based on 499 cases

Topographically, the AOT occurs in peripheral and central variants, the latter further in follicular (with embedded tooth) and extrafollicular (no embedded tooth) types. The AOT is slow growing with few or no symptoms. Tumor growth may cause displacement of teeth rather than root resorption. The follicular AOT mimics a follicular cyst, the extrafollicular a residual or "globulo-maxillary" cyst and the peripheral a gingival fibroma. All variants of AOT show identical histologic features. The central variants account for 97.2%, 73.0% of which are follicular. The follicular variant (M:F ratio 1 to 1.9) is three times as frequent as the extrafollicular. The follicular variant is diagnosed earlier in life (mean age 17 yr) than the extrafollicular (mean age 24 yr). 53.1% of all variants occur within the teens (13-19 yr). Follicular AOT is associated with one embedded tooth in 93.2%. Maxillary permanent canines account for 41.7% and all four canines for 60.1% of AOT-associated embedded teeth. Ranking four among the odontogenic tumors the AOT is not a particularly rare tumor. Conservative surgical excision is the treatment of choice. Documented recurrences have not been reported.     

Peripheral odontogenic fibroma. Report of 5 cases

The peripheral odontogenic fibroma (WHO type) is a relatively rare, benign, unencapsulated, exophytic gingival mass of fibrous connective tissue. Odontogenic epithelium is found within the gingival mass, but usually appears to play a minor role when compared to the fibrous component. According to the present concept, cases reported in the literature under the terms "odontogenic gingival epithelial harmartoma" "hamartoma of the dental lamina" and "peripheral ameloblastic fibrodentinoma" are actually examples of peripheral odontogenic fibroma. Review of the literature revealed only 30 acceptable cases that fit the present concept of peripheral odontogenic fibroma. Because of the paucity of reported cases, the histomorphological spectrum and the clinical features of this lesion have not yet been fully established. This article presents five new cases of peripheral odontogenic fibroma. The connective tissue ranged from markedly cellular to relatively acellular well collagenized. Islands and strands of epithelium were present in all five cases: in four they were scanty and in one abundant. A matrix of mineralized material was present in four cases. The peripheral odontogenic fibroma must be differentiated histologically from peripheral ossifying fibroma, which is a reactive lesion, and from the peripheral ameloblastoma and the calcifying epithelial odontogenic tumour.     

Odontogenic myxoma: An updated analysis of 1,692 cases reported in the literature

The aim of the present study was to integrate the available data published on odontogenic myxoma (OM) into a comprehensive analysis of its clinical/radiological features. Electronic search undertaken in January/2018, looking for publications reporting cases of OM. A total of 377 publications were included. We identified 1,692 lesions, and 695 were used for the analysis of recurrence. There is a predominance of OMs in females and in mandibles. OMs usually present with bone expansion, asymptomatic cortical perforation, and a multilocular appearance. Lesion location (maxilla/mandible), bone expansion, cortical bone perforation, locular radiological appearance, tooth resorption, odontogenic epithelial rests, or angular septa are not associated with recurrence. While curettage (31.3%) showed the highest recurrence rate, marginal resection (1.3%) and segmental resection (3.1%) showed the lowest values. Enucleation + peripheral osteotomy (6.7%) showed better results than enucleation (13.1%) or enucleation + curettage (12.7%). In comparison with unilocular lesions, multilocular ones were significantly more prevalent in mandibles, more often presented expansion and cortical bone perforation, had larger mean size, and were more often treated by segmental resection. Conservative surgical procedures are associated with higher probability of recurrence of OM. Taking into consideration the recurrence rate and morbidity associated with different surgical treatments, tumor enucleation followed by peripheral osteotomy should be considered as the first therapeutic choice.     

Central calcifying epithelial odontogenic tumour in the posterior maxilla: a case report

The calcifying epithelial odontogenic tumour (CEOT), or Pindborg tumour, is a rare, benign odontogenic tumour. CEOT is usually asymptomatic and an incidental radiological finding, often presenting as a mandibular radiolucency with flecks of calcific material. We report an unusual case of CEOT in the left posterior maxilla of a 46‐year‐old male that was associated with an unerupted tooth. The tumour in this case caused non‐specific sinus symptoms and appeared radiographically similar to an odontoma or ossifying fibroma due to its dense calcific contents. Diagnosis was confirmed histologically following surgical removal of the lesion, which showed classic CEOT histomorphology. We report this case to highlight the unusual clinico‐radiologic presentation and illustrate the diagnostic difficulties that can occur with radiolucent and/or radiopaque lesions in the jaws.     

Adenomatoid odontogenic tumour: A case report

Adenomatoid odontogenic tumour (AOT) is a rare benign odontogenic tumour characterized by a progressively slow growing pattern and symptomless behavior. The differential diagnosis between AOT and other odontogenic tumours, such as ameloblastoma, should be well conducted in order to avoid extensive ablative surgery. This report presents an unusual case of an 11-year-old male patient who referred to the oral surgeon due to a significant painless gingival swelling in the anterior mandible. A panoramic X-ray revealed a round radiolucid image of an intraosseous lesion with well defined boards and related to the left lateral incisor and left canine. The Computerized Tomography was performed and the sagittal sections revealed a tooth image in contact with the inferior board of the tumour. Additionally, the coronal sections showed the presence of a tooth inside the lesion. Several calcifying nodules could be distinguished within the cystic area. The clinical diagnostic hypothesis was of calcifying epithelium odontogenic tumour but the histological sections were consistent with AOT. The tumour was enucleated under local anesthesia. After one year follow-up there were no signs of reoccurrence. With respect to the distinguishing tumour enlargement and localization in the lower jaw, the reported case is an uncommon example of AOT.     

Clinicopathological investigation of odontogenic fibroma in tuberous sclerosis complex

Tuberous sclerosis complex (TSC) is an autosomal dominant inherited disease characterized by systemic hamartoma and diverse systemic features. TSC1 and TSC2 are the causative genes, and mental retardation, epileptic seizures, and facial angiofibroma develop in many patients with the disease. The case of a patient with TSC who developed a central odontogenic fibroma of the mandible is reported here. The patient was a 21-year-old woman who was referred with a swelling of the labial gingiva in the region of the right lower lateral incisor and canine. Dental radiography revealed a multilocular radiolucent region with a clear boundary. The right lower lateral incisor and canine were continuous with the lesion and thus were excised en bloc. The lesion was encapsulated and easily dissected. The diagnosis on immunohistological staining was odontogenic fibroma without an epithelial component. TSC1/2 gene mutation causes abnormal activation of mammalian target of rapamycin (mTOR) downstream of the PI3K–AKT pathway. The odontogenic fibroma in this patient was positive for mTOR, suggesting that the development of the odontogenic fibroma was the result of abnormal activation of mTOR, as in angiofibroma. The clinical course of this patient is presented and the developmental mechanism of central odontogenic fibroma is discussed.     

The peripheral odontogenic fibroma: an attempt at clarification

Two different lesions of the gingiva that have been referred to previously as peripheral odontogenic fibromas are discussed. The first of these is the rare extraosseous counterpart of the central odontogenic fibroma (WHO type)1 and is therefore referred to in this article as the peripheral odontogenic fibroma (WHO type). It is probably treated adequately by simple excision, but a study of its biologic behavior is lacking. The second lesion is reactive, is common, and has a marked tendency to recur. It has been known by numerous synonyms, including calcifying fibrous epulis and peripheral ossifying fibroma, as well as peripheral odontogenic fibroma. The term peripheral ossifying fibroma should be retained for this lesion to avoid confusion with the peripheral odontogenic fibroma (WHO type).     

Cystic variant of calcifying epithelial odontogenic tumor

The calcifying epithelial odontogenic tumor is a rare benign odontogenic neoplasm of the jaw. Clinically, calcifying epithelial odontogenic tumor manifests as an intraosseous lesion (central type) in the majority of cases (95%). Extraosseous or peripheral lesions account for less than 5% of cases. Calcifying epithelial odontogenic tumor can be associated with an impacted tooth and give a radiographic simulation of dentigerous cyst. Most calcifying epithelial odontogenic tumors are solid in nature, histopathologically, and might have few cyst-like spaces within them. However, a true cystic calcifying epithelial odontogenic tumor is a rare possibility. We describe a case of a true cystic variant of calcifying epithelial odontogenic tumor in a 30-year-old male, which to our knowledge, is only the second reported case.     

成釉细胞纤维牙瘤的临床特点及治疗

成釉细胞纤维牙瘤是一种少见的牙源性肿瘤,多发生于20岁以下,男性多于女性,上下颌骨均可发生,多发生于下颌骨,生长缓慢,无自觉症状,常表现为颌面部的肿胀、牙齿迟萌。X线片常见单房改变,表现为边界清楚的囊性透射影,不易与牙源性肿瘤鉴别。组织学表现,软组织成分为牙源性上皮和胚胎性的结缔组织,类似成釉细胞纤维瘤的形态,硬组织成分为牙本质、釉质样组织,类似牙瘤。恶变可能性低,治疗方式为手术摘除,一般不易复发。该文结合我科收治的成釉细胞纤维牙瘤病例,探讨成釉细胞纤维牙瘤的临床特点及治疗与预后。     

Odontogenic keratocyst in maxillary sinus with invasive behaviour

Odontogenic keratocyst is a cystic lesion characterized by a high rate of recurrence. This report describes a rare case of ciliated epithelium-lined odontogenic keratocyst in the maxilla of a 27-year-old female. Panoramic radiography showed a lytic lesion on the right maxilla associated with an impacted molar tooth. Computerized tomography image revealed the involvement of the lesion with the right maxillary sinus, destroying the sinus floor. Histopathologically, the typical keratinized epithelial-lined cyst of odontogenic keratocyst abruptly changed into a ciliated epithelium, suggesting the fusion of both these epithelia rather a metaplastic transformation. The biological behaviour of odontogenic keratocysts is discussed.     

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提要：外周性牙源性肿瘤又称骨外型牙源性肿瘤或软组织牙源性肿瘤,主要发生在牙龈,包括牙源性真性肿瘤及错构瘤。外周性牙源性肿瘤约占所有牙源性肿瘤的4%左右,英文文献表明其中外周性牙源性纤维瘤最多见,其次是外周性成釉细胞瘤及外周性牙源性钙化囊性瘤。外周性牙源性肿瘤临床上容易与牙龈发生的炎症性或反应性病变相混淆,明确诊断依赖组织病理学检查。外周性牙源性肿瘤不包括骨内型牙源性肿瘤穿破骨皮质侵犯牙龈。外周性牙源性肿瘤预后普遍好于相应的骨内型肿瘤,但切除不彻底仍可复发,建议长期随访。     

The central odontogenic fibroma. Clinical and morphologic studies.

The clinical, pathologic, and ultrastructural features and pertinent case history of an 11-year-old boy with a rare benign odontogenic neoplasm, the central odontogenic fibroma, are presented. A review of the literature reveals seven other lesions with clinical and pathologic findings similar to those of this case. This study supports the concept that the central odontogenic fibroma is a distinct odontogenic neoplasm of bone which occurs most commonly in the mandible as a multilocular, radiolucent, and slowly growing expansile lesion with no tendency to recur after surgical enucleation. The ultrastructural findings indicate that the central odontogenic fibroma and the odontogenic myxoma share many common morphologic features and have an apparently similar histogenesis.     

Diffuse peripheral odontogenic fibroma: report of 3 cases

Since peripheral odontogenic fibroma (POF) is characteristically described as a solitary lesion and no diffuse POF had been reported in the literature, our cases should be considered as extremely unusual. Three diffuse cases of POF are described of which one case was seen in association with ocular and skin lesions. The question arises whether POF should be considered as a true odontogenic tumor rather than a diffuse hamartomatous lesion caused by uncontrolled induction of the gingiva. It is also possible that such lesions could be part of a yet undescribed syndrome.     

Benign cementoblastoma – A rare odontogenic tumour

Benign cementoblastoma is a rare odontogenic tumour derived from odontogenic ectomesenchyme of cementoblast origin that forms cementum layer on the roots of a tooth. Benign cementoblastomas have a distinct clinical and radiographic appearance. It is important to consider them as one among the differential diagnosis in bony swellings of mandible. A case report of a 55 yr old female patient diagnosed as a case of benign cementoblastoma is presented.