The majority of cases have been reported to occur in the anterior part of the jaws. Pdgct occurs in older adults most commonly in the anterior mandibular region. Case report mandibular ghost cell odontogenic carcinoma. An international collaborative study group on ghost cell odontogenic tumors gathered and discussed all ghost cell lesions 19. Pdf calcifying odontogenic cyst, dentinogenic ghost cell tumor. Dentinogenic ghost cell tumor odontogenic ectomesenchymal odontogenic. Although gcs are present in a variety of odontogenic lesions notably the calcifying cystic odontogenic tumor gcot, their nature and. Immunohistochemical characteristics of odontogenic. Odontogenic ghost cell carcinoma ogcc is a rare odontogenic tumor characterized by the presence of ghost cells. Ghost cell odontogenic carcinoma gcoc is a rare malignant odontogenic tumor with aggressive growth characteristics.
Odontogenic ghost cell carcinoma ogcc, a malignant counterpart of the calcifying odontogenic cyst coc, is exceedingly rare. They may arise from the epithelial part of the tooth germ, the mesenchymal part or from both. The ghost cell odontogenic carcinoma gcoc is a rare neoplasm that represents the malignant end of the spectrum of ghost cell odontogenic tumors. Ghost cell odontogenic carcinoma gcoc was first described in detail as a malignant focus within a calcifying odontogenic cyst coc by ikemura et al. Ethics documents guidelines for the manuscript publishing process. Previous descriptions of this tumor were based on identification of malignant histologic characteristics such as infiltration, cellular pleomorphism, numerous mitoses, and necrosis concurrent with classical benign coc or its solid benign. Ki67 and p53 expression in ghost cell odontogenic carcinoma.
Previous descriptions of this tumor were based on identification of malignant histologic characteristics such as infiltration, cellular pleomorphism, numerous mitoses, and necrosis concurrent with classical benign coc or its solid benign variant, the. Pdf international collaborative study on ghost cell. Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring in the anterior region of the mandible in 5th7th decades and shows a female preponderance. A recurrent case of odontogenic ghost cell tumour of the mandible. It is potentially aggressive, capable of frequent recurrences and locoregional and distant metastases. He was asymptomatic until pain symptoms developed prior to initial presentation. Thirty cases of calcifying odontogenic cyst coc and 6 cases of dentinogenic ghost cell tumor dgct were selected for histopathological and immunohistochemical analysis.
Previous descriptions of this tumor were based on identification of malignant histologic characteristics such as infiltration, cellular pleomorphism, numerous mitoses, and necrosis concurrent with classical benign coc or its solid benign variant, the odontogenic ghost cell tumor. The tumor cells infiltrate into the adjacent fibrovascular tissue. It is more common in males with an age peak at the fourth decade of life 2. Ghost cell odontogenic carcinoma gcoc is the malignant counterpart of calcifying cystic odontogenic tumour and dentinogenic ghost cell tumour. Ghost cell odontogenic carcinoma gcoc is an extremely rare tumor of ghost cells. A noncystic solid variant is well known the odontogenic dentinogenic ghost cell tumor and a rare malignant variant has been reported odontogenic ghost cell carcinoma. Peripheral dentinogenic ghost cell tumor is a rare tumor with only 24 cases previously described in the english literature. Ghost cell odontogenic carcinoma, malignant odontogenic tumor. Ghost cells gcs are anucleate cells with homogeneous pale eosinophilic cytoplasm and very pale to clear central areas previous nucleus sites.
Chapter 21 odontogenic and non odontogenic tumors of the jaws introduction. Odontogenic carcinoma arising from benign odontogenic cysts. Notch signaling and ghost cell fate in the calcifying cystig. Ghost cell odontogenic carcinoma gcoc is an exceptionally rare and malignant odontogenic epithelial tumor, and has similar features to ccot. We have described the clinical and radiographic features, histological. Chapter 21 odontogenic and nonodontogenic tumors of the jaws introduction. It is potentially aggressive and capable of multiple local recurrences and locoregional and distant metastases. This article is from korean journal of pathology, volume 48. Ghost cell odontogenic carcinoma arising from calcifying. Clinical and radiological features of 22 ogccs 4 new and 18 from the literature were analysed. This case presents an additional case, a 47yearold chinese man presented with a slowgrowing mandibular lesion with history of ameloblastoma. At the time of writing 2016, it is still the most widely used classification system although a new revision is due to come up in 2017.
Ghost cell odontogenic carcinoma gcoc is a rare malignant odontogenic epithelial tumor with features of calcifying odontogenic cysts that can. To analyse systematically the clinical and radiological features of odontogenic ghost cell carcinoma ogcc. Pdf odontogenic ghost cell carcinoma ogcc is a rare neoplastic variant of calcifying odontogenic cyst, with aggressive clinical characteristics. Morgan odontogenic tumors comprise a range of disorders of growth, from malignant and benign. Carcinoma arising in the bone is an extremely rare condition. Odontogenic ghost cell carcinoma odontogenic ghost cell carcinoma goldenberg, david. The most notable features of this pathologic entity are histopathological features which include a cystic lining demonstrating characteristic. According to who 2005, ghost cell odontogenic carcinoma is a malignant epithelial tumor with features of calcifying cystic odontogenic tumor or dentinogenic ghost cell tumor classified as an odontogenic carcinoma, which includes. Calcifying ghost cell odontogenic tumor cgcot was first described by gorlin et al. Ghost cell odontogenic carcinoma gcoc is a malignant odontogenic epithelial tumor with features of calcifying cystic odontogenic tumor ccot andor dentinogenic ghost cell tumor dgct. An external file that holds a picture, illustration, etc. A 45 year old woman presented with a radiolucent left mandibular swelling associated with. In the whoiarc classification of head and neck pathology, this clinical entity had been known for years as the odontogenic. May 01, 2008 read international collaborative study on ghost cell odontogenic tumours.
We report the first pediatric metastatic ogcc case treated with multimodal therapy. The majority of cases represent well differentiated keratinizing carcinoma 6,28. The extraosseous odontogenic tumors may raise diagnostic challenges due to their imitation for reactive gingival lesions. Odontogenic ghost cell carcinoma johns hopkins university. Ghost cell odontogenic carcinoma, aggressive epithelial ghost cell odontogenic tumor, dentinogenic ghost cell ameloblastoma, carcinoma arising in a coc, and malignant calcifying ghost cell odontogenic tumor 6. Gorlin nevoid basal cell carcinoma syndrome basal cell carcinomas in nonsun exposed areas. Primary intraosseous squamous cell carcinoma arising from. Ghost cell odontogenic carcinoma on right mandible and its. Multiple basal cell carcinomas multiple jaw cysts odontogenic keratocysts numerous bone abnormalities including bi. Other tumours, such as calcifying epithelial odontogenic tumour pindborg tumour and ghost cell lesions, may comprise only about 1% of the total and are therefore very rarely seen. A,axial unenhanced ct scan shows a large, poorly marginated, lobulated softtissue mass causing irregular destruction of the body of the mandible, predominantly in the right.
The calcifying ghost cell odontogenic cyst cgcoc was first described by gorlin et al. International collaborative study on ghost cell odontogenic. International collaborative study on ghost cell odontogenic tumours. According to the latest world health organization classification in 2005, malignant odontogenic epithelium tumors consist of metastatic ameloblastoma, ameloblastic carcinoma, primary intraosseous carcinoma, ghost cell odontogenic carcinoma gcoc, and clear cell odontogenic carcinoma. Odontogenic keratocysts make up around 19% of jaw cysts. There were 17 77% males and 5 23% females maletofemale ratio of 3. The chart showing pdf series, word series, html series, scan qr codes. Previous descriptions of this tumor were based on identification of malignant histologic characteristics such as infiltration, cellular pleomorphism, numerous mitoses, and necrosis concurrent with classical benign coc. Ameloblastic carcinoma, calcifying odontogenic cyst, ghost cell carcinoma, keratinizing epithelial odontogenic cyst, maxillary tumor, odontogenic carcinoma. Pdf dentinogenic ghost cell tumor dgct is a rare, odontogenic neoplasm which is considered to be a solid variant of calcifying. Aggressive epithelial odontogenic ghost cell tumor in the. The 2005 who histological classification of odontogenic tumors lays out a classification system for neoplasms and other tumors related to the odontogenic apparatus.
In a recent study in an iranian population, the researchers by searching 14,106 patient files during a period of 44 years found only 37 cases of ghost cell odontogenic tumors. Primary intraosseous squamous cell carcinoma arising in. The present report describes 2 cases of primary intraosseous squamous cell carcinoma. The 2005 who classification of odontogenic tumours re. This study aimed to revisit benign odontogenic ghost cell lesions bogcl by hematoxylin and eosin staining and immunohistochemistry. Odontogenic cysts can give rise to a variety of neoplasms. Calcifying ghost cell odontogenic cyst is comparatively rare in occurrence, constituting about 0. Until now, only 31 cases of ogccs have been reported in literature. Ghost cell odontogenic carcinoma with suspected cholesterol. We describe a case of gcoc which was considerably derived from a previously resected calcifying cystic odontogenic tumor ccot. Benign odontogenic ghost cell lesions revisited and new. Gcoc is defined as a malignant odontogenic epithelial tumor with the.
Odontogenic ghost cell carcinomas ogcc are rare and only a dozen or so have been reported in the literature. Ghost cell odontogenic carcinoma is an extremely rare odontogenic tumor with unclear etiology. A primary intraosseous squamous cell carcinoma pioscc is defined as a squamous cell carcinoma arising within the jaw bones, which has no initial connection with the oral mucous membrane and develops from remnants of odontogenic epithelium deriving embryologically from ledge or. According to who 2005, ghost cell odontogenic carcinoma is a malignant epithelial tumor with features of calcifying cystic odontogenic tumor or dentinogenic ghost cell tumor. An odontogenic keratocyst is a rare and benign but locally aggressive developmental cyst. Squamous cell carcinoma arising from an odontogenic. The ghost cell odontogenic neoplasms are rare jaw tumors. This is the case of a middleaged male who presented with a slowgrowing maxillary tumour. Odontogenic and nonodontogenic tumors of the jaws are a relatively rare and heterogeneous group of benign and malignant neoplasms, hamartomas, and other bonerelated lesions that demonstrate great variability in etiology, biologic behavior, and clinical significance. Calcifying odontogenic cyst gorlin cyst pilomatricoma. In this chapter, only those composed of both odontogenic epithelium and mesenchyme will be dealt with, separate chapters being devoted to the.
The distinct feature of this lesion is a cystic lining demonstrating ghost epithelial cells with a. Ghost cell odontogenic carcinoma gcoc is an exceptionally rare and malignant odontogenic tumor with aggressive growth characteristics. Odontogenic ghost cell carcinoma with pulmonary metastasis. Clear cell odontogenic carcinoma ghost cell odontogenic carcinoma odontogenic sarcomas ameloblastic. Six months follow up period shows no signs of recurrence. A case occurring in posterior molar region of the mandible in a 75yearold edentulous woman is reported.
This tumor appears to be more common in asians based on the cases that have been reported and is more common in the maxilla than the mandible. Odontogenic and non odontogenic tumors of the jaws are a relatively rare and heterogeneous group of benign and malignant neoplasms, hamartomas, and other bonerelated lesions that demonstrate great variability in etiology, biologic behavior, and clinical significance. Odontogenic tumors encompass a group of lesions that share a common origin from odontogenic tissues. Dentinogenic ghost cell tumors dgct are very rare tumors considered as solid variants of calcifying epithelial odontogenic cysts ceoc. It is an exceedingly rare, highly aggressive, rapidly growing, and infiltrative tumor with increased risk of local recurrence and distant metastasis. Although touted as a benign lesion in the initial stages, once the aggressive nature of the lesion became evident, it was reclassified as a malignant tumor by the world health.
The odontogenic ghost cell carcinoma ogcc is the malignant counterpart of a calcifying cystic odontogenic tumor and dentinogenic ghost cell tumor. Clinical and radiological features of odontogenic ghost. Jan 21, 2008 the 2005 who classification of odontogenic tumours re. Clear cell odontogenic carcinoma is a rare jaw lesion with predominantly clear cell characteristics. Pdf background the aim of this study was to integrate the available data published on calcifying odontogenic cyst coc, dentinogenic ghost cell tumor. Among them, the peripheral dentinogenic ghost cell tumor pdgct is an extremely rare benign odontogenic neoplasm with up to 30 cases published in the literature. A histopathologic examination shows epithelial cell nests in tumor tissue. Classified as an odontogenic carcinoma, which includes. Martosfernandez m, alberolaferranti m, huetomadrid ja, bescosatin c. Background clear cell odontogenic carcinoma ccoc, which has been described within the past 2 decades, is a rare odontogenic tumor that tends to occur in the mandible of older adults, with a predilection for women. In the current who system, ghost cell tumors have been classified into three major categories on the basis of the cystic, solid, or malignant nature of the lesion. Read international collaborative study on ghost cell odontogenic tumours. Consequently, the name clear cell odontogenic carcinoma was thought to be more appropriate in view of the malignant potential manifested by this neoplastic lesion 2022.
Primary malignant tumors of the jaws can arise from odontogenic epithelial remnants within the alveolar segment or from the transformation or degeneration of benign lesions. Odontogenic ghost cell carcinoma ogcc is an extremely rare tumor, wherein the optimal management remains uncertain. They are locally invasive neoplasms and their main characteristic features are ameloblastoma like odontogenic epithelial proliferation, an aberrant keratinization in the form of ghost cells and dysplastic dentin. It most often affects the posterior mandible and most commonly presents in the third decade of life. A ghost cell is an enlarged eosinophilic epithelial cell with eosinophilic cytoplasm but without a nucleus. Current concepts and occurrence of epithelial odontogenic. Dentigerous cysts are one of the most common odontogenic cysts of the oral cavity. Nov 10, 2011 notch signaling is an evolutionarily conserved mechanism that enables adjacent cells to adopt different fates. Pioc must be considered in the differential diagnosis of malignant tumors of odontogenic epithelium, including ameloblastic carcinoma, intraosseous mucoepidermoid carcinoma, clear cell odontogenic carcinoma, and a ma. Calcifying ghost cell odontogenic tumor cgcot with.
B tumor cells are admixed with anucleate ghost cells. Pathogenesis and nomenclature of odontogenic carcinomas. Ghost cell odontogenic carcinoma gcoc is a rare malignant. Although known by many names, the basic histologic identifying feature includes the identification of the ghost cells and the malignant changes associated with the cells. 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.
Ledesmamontes c, gorlin rj, shear m, prae torius f, mosquedataylor a, altini m, et al. Dentinogenic ghost cell tumora rare case report with. Ghost cell odontogenic carcinoma gcoc is a rare malignant odontogenic tumor, only 37 cases of gcoc have been reported in the englishlanguage literature to date. Ghost cell odontogenic carcinoma it is also called as malignant epithelial odontogenic tumor.