International collaborative study on ghost cell odontogenic tumours: Calcifying cystic odontogenic tumour, dentinogenic ghost cell tumour and ghost cell odontogenic carcinoma

Oral Pathology Department, Facultad de Odontología, Universidad Nacional Autónoma de México, México, DF, México.
Journal of Oral Pathology and Medicine (Impact Factor: 1.93). 06/2008; 37(5):302-8. DOI: 10.1111/j.1600-0714.2007.00623.x
Source: PubMed


Calcifying odontogenic cyst was described first by Gorlin et al. in 1962; since then several hundreds of cases had been reported. In 1981, Praetorius et al. proposed a widely used classification. Afterwards, several authors proposed different classifications and discussed its neoplastic potential. The 2005 WHO Classification of Odontogenic Tumours re-named this entity as calcifying cystic odontogenic tumour (CCOT) and defined the clinico-pathological features of the ghost cell odontogenic tumours, the CCOT, the dentinogenic ghost cell tumour (DGCT) and the ghost cell odontogenic carcinoma (GCOC).
The aim of this paper was to review the clinical-pathological features of 122 CCOT, DGCT and GCOC cases retrieved from the files of the oral pathology laboratories from 14 institutions in Mexico, South Africa, Denmark, the USA, Brazil, Guatemala and Peru. It attempts to clarify and to group the clinico-pathological features of the analysed cases and to propose an objective, comprehensive and useful classification under the 2005 WHO classification guidelines.
CCOT cases were divided into four sub-types: (i) simple cystic; (ii) odontoma associated; (iii) ameloblastomatous proliferating; and (iv) CCOT associated with benign odontogenic tumours other than odontomas. DGCT was separated into a central aggressive DGCT and a peripheral non-aggressive counterpart. For GCOC, three variants were identified. The first reported cases of a recurrent peripheral CCOT and a multiple synchronous, CCOT are included.
Our results suggest that ghost cell odontogenic tumours comprise a heterogeneous group of neoplasms which need further studies to define more precisely their biological behaviour.

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Available from: Wilson Alejandro Delgado, Aug 31, 2014
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    • "Although CCOT is designated histologically as a compound lesion composed of COC and tumorous odontogenic epithelium, it can be classified into four sub-types using the following predominant findings, 1) simple cystic (Fig. 2A-C), 2) odontoma associated (Fig. 2D-F), 3) ameloblastomatous proliferating (Fig. 2G-I), or 4) CCOT associated with a benign odontogenic tumor other than odontoma.54,66 "
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    ABSTRACT: Calcifying epithelial odontogenic tumors (CEOTs) and ghost cell odontogenic tumors (GCOTs) are characteristic odontogenic origin epithelial tumors which produce calcifying materials from transformed epithelial tumor cells. CEOT is a benign odontogenic tumor composed of polygonal epithelial tumor cells that show retrogressive calcific changes, amyloid-like deposition, and clear cytoplasm. Differentially, GCOTs are a group of transient tumors characterized by ghost cell presence, which comprise calcifying cystic odontogenic tumor (CCOT), dentinogenic ghost cell tumor (DGCT), and ghost cell odontogenic carcinoma (GCOC), all derived from calcifying odontogenic cysts (COCs). There is considerable confusion about COCs and GCOTs terminology, but these lesions can be classified as COCs or GCOTs, based on their cystic or tumorous natures, respectively. GCOTs include ameloblastomatous tumors derived from dominant odontogenic cysts classified as CCOTs, ghost cell-rich tumors producing dentinoid materials as DGCTs, and the GCOT malignant counterpart, GCOCs. Many authors have reported CEOTs and GCOTs variably express keratins, β-catenin, BCL-2, BSP, RANKL, OPG, Notch1, Jagged1, TGF-β, SMADs, and other proteins. However, these heterogeneous lesions should be differentially diagnosed to allow for accurate tumor progression and prognosis prediction.
    Full-text · Article · Jun 2014 · The Korean Journal of Pathology
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    • "Here, all cases of CCOT were negative for this polypeptide, probably because of the complete absence of squamous differentiation of its epithelium lining. In fact, this tumor is composed of a well-defined basal layer beneath a stellate reticulum-like suprabasal layer, which matches that observed in ameloblastoma and the tooth germ, and even a complete absence of terminal squamous epithelial differentiation [4]. Contradicting this hypothesis, some authors have been suggested squamous differentiation on CCOT pathogenesis because of the detection of Ck13 expression on epithelium lining [15] [17]. "
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    ABSTRACT: Calcifying cystic odontogenic tumors (CCOTs) are benign cystic lesions of odontogenic origin characterized by an ameloblastoma-like epithelium and the presence of a group of cells named ghost cells. The pattern of cytokeratin (Ck) expression on these lesions remains unclear and needs to be clarified. To this end, the expression of Ck6, Ck13, Ck14, Ck18, and Ck19 in the epithelium lining of 7 cases of CCOTs was evaluated by immunohistochemistry. For this, the epithelium lining was divided into 3 distinct regions: basal layer, suprabasal layer, and the compartment composed of ghost cells. In this study, 6 cases (85.7%) were classified as type 1 and 1 (14.3%) as type 4. All cases were negative for Ck13 and Ck18, despite the epithelial layer, as well as in the ghost cells. Ck6 was only positive in the ghost cells. Positivity for Ck14 and Ck19 was found in the basal and suprabasal layers, including the ghost cells. The results showing positivity for Ck14 and Ck19 in all of the analyzed cases reinforce CCOT as being of odontogenic origin, and the restricted expression of Ck6 in the ghost cells may be indicative that these cells suffer an altered differentiation into hair follicles in CCOTs.
    Full-text · Article · Oct 2013 · Annals of diagnostic pathology
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    • "Recurrence in case of ECCOT is rare. Follow-up treatment ranged from 9 months to 60 months, only one case of recurrence could be observed.[10] "
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    ABSTRACT: Odontogenic cysts comprise a diverse group of exceptional lesions derived from epithelial elements of the tooth-forming apparatus. Calcifying cystic odontogenic tumor is a rare odontogenic lesion, which represents about 2% of all odontogenic tumors and cysts. It may occur in a central (intraosseous) or peripheral (extraosseous calcifying cystic odontogenic tumor [ECCOT]) location. ECCOT in contrast to central, tends to affect the older patients, commonly located in the anterior lower jaw, is less aggressive and the recurrence is rare. Here, we report clinico-pathological and radiographic features of ECCOT located in relation to 48 (posterior lower jaw) measuring about 1 cm in the greatest diameter in a 17-year-old male.
    Full-text · Article · Jul 2013
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