A variant of calcifying epithelial odontogenic tumor with Langerhans cells

A variant of calcifying epithelial odontogenic tumor (CEOT) with Langerhans cells is reported. Compared to a typical CEOT, the tumor islands of this case were thin and composed of a small number of polyhedral epithelial cells. Almost no calcification of homogeneous eosinophilic materials was observed. In addition, clear cells which structurally corresponded to Langerhans cell were intermingled in the epithelial islands. These cells stain positively for S-100 protein, lysozome, MT 1, LN-3 and OKT 6 antibodies, but not for keratin antibody. Electronmicroscopic examination revealed the rod-shaped and racket-shaped structures called Birbeck's granules in the cytoplasm of these clear cells. Our observations indicate a variant case of CEOT with Langerhans cells in tumor nests.     

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.     

骨外型牙源性钙化上皮瘤

骨外型牙源性钙化上皮瘤极为罕见,通过复习1966～2011年英文MEDLINE与1994～2011年中文CHKD中收录的文献,仅有37例骨外型牙源性钙化上皮瘤的个案报告。根据上述资料分析,发现该瘤具有以下临床特点,就诊年龄3～71岁,平均35.8岁;男13例,女19例,男女比例为1∶1.46;肿块直径0.5～4 cm,平均1.76 cm;好发部位主要是牙龈,累及下颌与上颌牙龈的比例为1∶1;组织学特征表现与骨内型牙源性钙化上皮瘤基本相同;复发率为5.4%。因该瘤具有浅表骨侵蚀能力,应行完整的肿瘤切除结合骨面刮治术。     

Ameloblastoma, calcifying epithelial odontogenic tumor, and glandular odontogenic cyst show a distinctive immunophenotype with some myoepithelial antigen expression

Background:  Odontogenic neoplasms have some morphologic overlap with salivary gland neoplasms, many of which show myoepithelial differentiation. In the 1980s, an ultrastructural study identified a population of myoepithelial-like cells in calcifying epithelial odontogenic tumor. Myoepithelial derived tumors have since been shown to have distinct immunohistochemical profiles.
Methods:  We examined a series of odontogenic neoplasms, including 11 ameloblastomas, four calcifying epithelial odontogenic tumors, five glandular odontogenic cysts (GOCs), and five keratocystic odontogenic tumors with a panel of myoepithelial-associated immunohistochemical stains. We also assessed representative control examples of oral mucosa, odontogenic rests, and dentigerous cysts.
Results:  All of the neoplastic and non-neoplastic oral epithelium-derived entities share a p63-positive, high molecular weight cytokeratin (CK5/6)-positive immunophenotype. Calponin reactivity was at least focally present in two of four calcifying epithelial odontogenic tumors, three of five GOCs, and 10 of 11 ameloblastomas; the sole completely non-reactive ameloblastoma represents a lung metastasis. One case of calcifying epithelial odontogenic tumor was focally positive for glial fibrillary acidic protein. However, other more definitive markers of myoepithelial differentiation, including S-100 and smooth muscle actin, were negative. Two of three calcifying epithelial odontogenic tumors and five of five GOCs were also positive for a low molecular weight cytokeratin (CK7).
Conclusions:  Ameloblastomas, GOCs, and calcifying epithelial odontogenic tumors show a distinctive immunophenotype which overlaps with that of myoepithelial-derived salivary gland neoplasms but does not provide definitive support for myoepithelial differentiation.     

钙化上皮瘤及牙源性钙化上皮瘤的临床病理分析

目的：探讨钙化上皮瘤及牙源性钙化上皮瘤的临床表现、诊断及外科处理原则。方法：对13例钙化上皮瘤和4例牙源性钙化上皮瘤患者的临床资料进行回顾性分析。结果：所有患者均经手术治疗。钙化上皮瘤患者平均年龄11．2岁,小于10岁者占69．2％（10例）;位于头颈部8例（61．5％）,四肢4例（30．8％）,躯干1例（7．7％）。牙源性钙化上皮瘤患者平均年龄38．75岁,均位于颌骨。两种肿瘤无明显性别差异,均有复发、恶变的报道。结论：钙化上皮瘤和牙源性钙化上皮瘤在临床表现和病理诊断上具有明显差别,是两种不同的肿瘤;牙源性钙化上皮瘤具有局部浸润性生长,手术切除必须彻底。     

骨外型牙源性钙化上皮瘤1例报告

牙源性钙化上皮瘤是一种良性肿瘤，也称Pindborg瘤，可分为骨内型和骨外型2种，主要发生于颌骨内，偶见于颌骨外。本文报告1例发生于口底的牙源性钙化上皮瘤，其临床症状与口底皮样囊肿相似．术后经组织病理学确诊，主要组织病理学特征是嗜酸性环状钙化团块。因其具有局部侵袭性，为避免复发，提倡完整切除肿物。     

牙源性钙化上皮瘤2例

牙源性钙化上皮瘤（CEOT）是一种具有局部浸润性生长特点的良性牙源性上皮性肿瘤,可分为骨内型（中央型）、骨外型（外周型）两种,后者罕见。本文报道2例牙源性钙化上皮瘤,并结合文献探讨其临床、影像表现特点。     

Histopathologic study of epithelial components in the connective tissue wall of unilocular type of calcifying odontogenic cyst

Histopathologic study of satellite cysts and odontogenic epithelial islands in connective tissue wall of unilocular type of calcifying odontogenic cyst (COC) was made. The material was 13 cases consisting of 3 simple unicystic COCs, 9 odontome producing COCs and 1 ameloblastomatous proliferating COC. Satellite cysts were found in 6 cases, and were histologically classified into following types: simple cystic, odontome producing and ameloblastomatous. Histologic types of satellite cysts did not coincide with those of main cystic lesions in some cases. Odontogenic epithelial islands with or without proliferating feature were found in 9 cases, and were found in all cases with satellite cysts. Melanin and melanocytes were seen in an ameloblastomatous satellite cysts of 1 of 3 pigmented COCs.     

Ameloblastoma ex calcifying odontogenic cyst (dentinogenic ghost cell tumor)

Calcifying odontogenic cyst (COC) has shown to be of extensive diversity in its clinical and histopathological features, as well as in its biological behavior. In this report, a rare case is described of ameloblastoma ex COC (dentinogenic ghost cell tumor) and the relevant literature is briefly reviewed.     

Peripheral calcifying odontogenic cyst

BACKGROUND: Calcifying odontogenic cyst (COC) is a rare lesion representing about 1% of jaw cysts. It may occur in a central (intraosseous) or peripheral (extraosseous) location. METHOD: A case of peripheral COC located on the gingiva, appearing as a painless, circumscribed, pink nodule has been reported. RESULTS: Peripheral, in contrast to central, COC tends to affect older patients. Peripheral COC is a less aggressive lesion than the central counterpart, and a simple excision biopsy is curative. CONCLUSION: The histological finding of a keratinized epithelium rich in ghost cells has helped in making the diagnosis.     

Ameloblastomatous calcifying odontogenic cyst: a rare histologic variant

Calcifying odontogenic cyst (COC) is an uncommon developmental odontogenic cyst first described by Gorlin in 1962. It is considered as extremely rare and accounts for only 1% of jaw cysts reported. Because of its diverse histopathology, there has always been confusion about its nature as a cyst, neoplasm or hamartoma. Several subclassifications have been proposed. Here, we present a case of calcifying odontogenic cyst with ameloblastic proliferation - an extremely rare histologic variant. The classical histologic features of the lining epithelium in the form of cords and presence of characteristic ghost cells were seen along with ameloblastomatous proliferations. Ameloblastomatous COC microscopically resembles unicystic ameloblastoma except for the ghost cells and calcifications within the proliferative epithelium. The nature of the COC is controversial. The case is presented here for its rarity, and difference between ameloblastomatous COC and ameloblastoma ex COC has been emphasized.     

牙源性钙化上皮瘤中朗格汉斯细胞的研究

目的：报告两例罕见的含朗格汉斯细胞的牙源性钙化上皮瘤，中心型及周边型各一例。方法：两例肿瘤行常规HE染色及PAS、刚果红组织化学染色；CD1a、CD68、HLA—DR、S-100蛋白免疫组织化学染色；并对1例新鲜肿瘤行透射电镜观察。结果：肿瘤以小条索／巢状结构为主，可见较多透明细胞，嗜酸性均质状物质极少发生钙化。4种标记朗格汉斯细胞的抗体在瘤细胞中均有阳性表达，透射电镜发现有些透明细胞的胞浆有朗格汉斯细胞特征性的Birbeck颗粒，细胞核分叶卷曲。结论：电镜及免疫组化研究结果表明这两例肿瘤为含朗格汉斯细胞的牙源性钙化上皮瘤。     

Clear cell variant of calcigying epithelia odontogenic tumor (CEOT) in the maxilla: report of a cse with immunohistochemical and ultrastructural investigations

A rare case of clear cell variant of calcifying epithelial odontogenic tumor is presented with immunohistochemical and ultrastructural investigations. A 14-year-old Japanese girl was admitted with a complaint of swelling in the right posterior maxilla. Radiological examination showed a well-circumscribed radiolucent lesion located close to the impacted third molar. After only a partial tumor excision, the tumor recurred 13 years later. It appeared radiologically as an irregular radiodensity, and a subtotal maxillectomy was performed. Histological examination showed sheets and/or strands composed almost entirely of clear vacuolated epithelial cells in a stroma containing intercellular amyloid-like material and calcification. Histochemical and ultrastructural analysis detected cytoplasmic glycogen granules in the clear cells, and positive immunoreactivities for cytokeratins 8, 13 and 19; filaggrin and anti-ameloblastoma antibodies suggested an odontogenic epithelial origin.     

Initiation of ectopic epithelial calcification in a calcifying odontogenic cyst

Ultrastructural observation was performed on a calcifying odontogenic cyst (COC) associated with an odontoma and arising in the right mandibular region of an 8-year-old Japanese boy. Four types of cells were identified in the epithelial layer of the COC. The basal cells were low columnar in shape and contained some intracellular organelles. They were attached to the neighboring cells with a few desmosomes and resembled inner enamel epithelium of the normal enamel organ. The stellate reticulum-like cells, polygonal in shape, possessed desmosomes and many cytoplasmic projections. Some intracellular organelles and a few bundles of tonofilaments were observed in the cytoplasm. The light oval cells that were pale staining with toluidine blue contained dilated membranous organelles and many relatively evenly distributed tonofilaments. These cells were usually scattered in the vicinity of the focal accumulations of ghost cells, and the cell membrane was discontinuous in parts. The ghost cells contained many bundles of tonofilaments that were 60-240 nm in diameter and arranged in various directions. No intact intracellular organelles were noted in the cytoplasm. They were attached to the neighboring ghost cells with some desmosomes and their cell membrane was discontinuous in parts. A variety of vesicles, 90-450 nm in diameter, were scattered among the tonofilament bundles. Some of these contained needle-like crystals that were considered to be initial calcification sites in ghost cells. These vesicles presented morphological similarities to matrix vesicles, and it is therefore suggested that matrix vesicle-like structures are deeply involved with initiation of calcification of ghost cells in COC.     

So-called calcifying odontogenic cyst: review and discussion on the terminology and classification

Toida M: So-called calcifying odontogenic cyst: review and discussion on the terminology and classification. J Oral Pathol Med 1998; 27: 49–52. © Munks-gaard, 1998.
The so-called calcifying odontogenic cyst (COC) shows extensive diversity in its clinico-histopathological appearances and biological behaviour. Because of this diversity, there has been confusion and disagreement on the terminology and classification of this lesion. The attempts at classification of COC may be divided into two concepts. The first concept is the "monistic" one that all COCs are neoplastic in nature, even though the majority are cystic in architecture and appear to be non-neoplastic. The second is the "dualistic" concept that COC contains two entities: a cyst and a neoplasm. Although the World Health Organization (WHO) classified COC as an odontogenic tumour in 1992 based on the former concept, current thinking favors strongly the latter one. In this article, several previous classifications of COC in the literature are discussed and a new simple classification scheme based on the "dualistic" concept is proposed.     

Cystic variant of calcifying epithelial odontogenic tumor

Calcifying epithelial odontogenic tumor (CEOT) is a benign, locally aggressive odontogenic neoplasm characterized by sheets and nests of epithelial cells with deeply eosinophilic or occasionally clear cytoplasm, calcifications, and eosinophilic amorphous material that stains positive for amyloid. Although many cases of CEOT are associated with impacted teeth and occasionally appear radiographically as dentigerous cysts, a true cystic variant has not been previously reported. We report a 15-year-old white male with a large cystic maxillary lesion that filled most of the left maxillary sinus. It deformed the medial wall, the inferior orbital floor, and caused narrowing of the left inferior meatus. Histologically, the cystic lining showed characteristics of CEOT. An intraluminal component that featured histologic characteristics of CEOT was identified during surgery. The lesion was enucleated and the postsurgical course of the patient was uneventful. Because follow-up has been for less than 1 year, a meaningful long-term prognosis cannot be determined at present. However, the patient has not reported any symptoms or signs of recurrence during the follow-up period.     

Calcifying epithelial odontogenic tumor of the maxilla with ulcerative stomatitis: a case report

Calcifying epithelial odontogenic tumor (CEOT) is a rare benign odontogenic tumor, known as Pindborg tumor. Although ulcer formation was reported in one previously involving the peripheral maxilla, such change of the overlying mucosa has been reported in intraosseous CEOT. We report maxillary CEOT in a patient who complained of spontaneous pain due to extensive ulcer formation of the oral mucosa.