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. Odontogenic ghost cell carcinomas ogcc are rare and only a dozen or so have been reported in the literature. Complete resolution of a calcifying cystic odontogenic. It develops from remnants of the odontogenic epithelium or from an odontogenic cyst or tumor.
Clinical and radiological features of odontogenic ghost. Pathogenesis and nomenclature of odontogenic carcinomas. Aggressive malignant epithelial odontogenic ghost cell tumor aggressive malignant epithelial odontogenic ghost cell tumor castle, james t arendt, douglas m. Calcifying cystic odontogenic tumor ccot demonstrates considerable diversity in histopathology and clinical behavior. Clinical and radiological features of 22 ogccs 4 new and 18 from the literature were analysed. A very rare malignant variety of odontogenic ghost cell tumor has been reported as odontogenic ghost cell carcinoma. In contrast, pioc exhibits sheets or islands of malignant epithelial cells, with minimal or absent clear cell component. Peripheral dentinogenic ghost cell tumor is a rare tumor with only 24 cases previously described in the english literature. The majority of cases have been reported to occur in the anterior part of the jaws. On ct and mr studies, the tumor was seen as a large, heterogeneous softtissue mass that caused marked destruction of the mandible and invaded the mouth floor and tongue base. Odontogenic ghost cell carcinoma johns hopkins university. We report the first pediatric metastatic ogcc case treated with multimodal. We investigated the immunohistochemical characteristics of cases of odontogenic carcinoma, including 3 cases of ameloblastic carcinoma, 9 cases of primary intraosseous squamous cell carcinoma pioscc, and 1 case of ghost cell odontogenic carcinoma gcoc, to elucidate the origin and determine diagnostic markers of odontogenic carcinoma.
Jan 17, 2018 january 17, 2018 errata for icdo3 sitetype validation list the icdo3 sitetype validation program was modified to only allow for the sitehistologybehavior combinations listed in this publication. Among them, pigmented calcifying cystic odontogenic tumor, formerly known as calcifying odontogenic cyst, is the most common lesion with 20 reported cases. A case of pigmented calcifying cystic odontogenic tumor associated with odontoma occurring at the mandibular canine. 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.
D, frcpath assistant prof of general pathology senior lecturer of oral pathology and oral histology slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Ghost cell odontogenic carcinoma gcoc is the malignant counterpart of calcifying cystic odontogenic tumour and dentinogenic ghost cell tumour. It is potentially aggressive and capable of multiple local recurrences and locoregional and distant metastases. It is more common in males with an age peak at the fourth decade of life 2. Here, we report the complete resolution of a calcifying cystic odontogenic tumor ccot in the right mandible after marsupialization in an 8yearold girl with a mixed dentition. These findings confirmed that genetic alterations not only provide valuable information on oncogenesis but also contribute. Ghost cell odontogenic carcinoma is an extremely rare odontogenic tumor with unclear etiology. Ghost cell odontogenic carcinoma gcoc is the rare malignant counterpart of ccot and it frequently arises from malignant transformation of a recurrent ccot. Clear cell odontogenic tumor is a rare neoplasm of the jaws that histologically may be confused with metastatic renal cell carcinoma.
Odontogenic tumors are lesions that are derived from remnants of the components of the developing tooth germ. Ghost cell odontogenic carcinoma gcoc is a rare malignant odontogenic epithelial tumor with features of benign calcifying odontogenic cysts. The extraosseous odontogenic tumors may raise diagnostic challenges due to their imitation for reactive gingival lesions. Classified as an odontogenic carcinoma, which includes.
This is the case of a middleaged male who presented with a slowgrowing maxillary tumour. Clinical and radiological features of odontogenic ghost cell. Although cysts are common in the jaws, most are radicular cysts of inflammatory origin or simple dentigerous cysts. A pigmented calcifying cystic odontogenic tumor associated. New tumour entities in the 4th edition of the world health. Predictive factors of potential malignant transformation. We describe a case of gcoc which was considerably derived from a previously resected calcifying cystic odontogenic tumor ccot. Odontogenic ghost cell carcinoma ogcc, a malignant counterpart of the calcifying odontogenic cyst coc, is exceedingly rare. 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. Sometimes, there are no visible clinical features and it may cause delay in an accurate diagnosis. Odontogenic sarcomas amelobastic fibrosarcoma amelobastic fibrodentinosarcoma ameloblastic fibroodontosarcoma 6. Herein, we report two cases of gcoc and describe their clinical features, histological characteristics, and treatment. To analyse systematically the clinical and radiological features of odontogenic ghost cell carcinoma ogcc.
Pdf a lesion categorized between ghost cell odontogenic. Predictive factors of potential malignant transformation in. We report the first pediatric metastatic ogcc case treated with multimodal therapy. Genetic and histopathological analysis of a case of. International collaborative study on ghost cell odontogenic tumours. Immunohistochemical characteristics of odontogenic. 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. 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.
Ghost cell odontogenic carcinoma arising from calcifying. Aggressive malignant epithelial odontogenic ghost cell. Ghost cell odontogenic carcinoma gcoc was first described in detail as a malignant focus within a calcifying odontogenic cyst coc by ikemura et al. 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. The 2005 who classification of odontogenic tumours re. He was asymptomatic until pain symptoms developed prior to. 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. Ghost cell odontogenic carcinoma 934 clear cell odontogenic carcinoma 823 serrated adenocarcinoma melanotic schwannoma 83393 follicular thyroid carcinoma ftc, encapsulated angioinvasive adenocarcinoma in situ, nonmucinous 82563 c319 accessory sinus, nos c600c602,c608c609 penis c632 scrotum. Classified into benign mixed epithelial and mesenchymal odontogenic tumour in the 4th edition of the world health organization who classification of head and neck tumours in 2017. The calcifying cystic odontogenic tumor or calcifying odontogenic cyst is a benign cystic neoplasm of odontogenic origin that is characterized by an ameloblastomalike epithelium and ghost cells. A,axial unenhanced ct scan shows a large, poorly marginated, lobulated softtissue mass causing irregular destruction of the body of.
Morgan odontogenic tumors comprise a range of disorders of growth, from malignant and benign neoplasms, to malformations of dental tissues of selflimited growth. Ghost cell odontogenic carcinoma gcoc is an exceptionally rare and malignant odontogenic tumor with aggressive growth characteristics. Ghost cell odontogenic carcinoma is malignant counterpart of calcifying odontogenic cyst, which is rare to occur. Ghost cell odontogenic carcinoma gcoc is an exceedingly rare malignant tumor on the spectrum of already uncommon odontogenic or dentinogenic tumors. Mcqs in prosthodontics major and minor connectors removable. In addition, the tumor affected the tooth, suggesting that the disease may originate from an odontogenic tumor, progressing. Others are less frequently encountered and may present diagnostic difficulties because of their varied features.
The odontogenic ghost cell carcinoma ogcc is the malignant counterpart of a calcifying cystic odontogenic tumor and dentinogenic ghost cell tumor. Malignant lesions in the anterior maxilla pdf free download. Malignant tumours of the maxilla recurrent mass in the anterior maxilla malignant tumors of the superior maxilla malignant fibrous histiocytoma of the maxilla primary malignant fibrous histiocytoma of the maxilla. Aggressive epithelial odontogenic ghost cell tumor in the. Jan 21, 2008 the 2005 who classification of odontogenic tumours re.
At the time of writing 2016, it is still the most widely used classification system although a. 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. Primordial odontogenic tumor pot was first reported by mosqueda taylor et al in 2014. Ghost cell odontogenic carcinoma gcoc, a malignant counterpart of calcifying odontogenic cyst coc and dentinogenic ghost cell tumor dgct, is extremely rare. Odontogenic ghost cell carcinoma odontogenic ghost cell carcinoma goldenberg, david. Odontogenic carcinoma with dentinoid in longterm followup. Ghost cell odontogenic carcinoma is a rare condition characterized by ameloblasticlike islands of epi thelial cells with aberrant keratinitation in the form of ghost cell with varying amounts of dysplastic dentina.
Odontogenic tumors and allied lesions edition 1 by peter. So far 30 cases of ghost cell odontogenic carcinoma. Incisional biopsy and microscopic examination revealed an aggressive epithelial odontogenic tumor with histologic features suggesting malignant transformation from an odontogenic cyst. Odontogenic ghost cell carcinoma ogcc is a rare neoplastic variant of calcifying odontogenic cyst, with. We have described the clinical and radiographic features, histological. Radiographic appearance and histopathological features however, there are. In this paper, we present a case of ccot and discuss its distinct histopathologic features in recurrence. A ghost cell is an enlarged eosinophilic epithelial cell with eosinophilic cytoplasm but without a nucleus. Pediatric metastatic odontogenic ghost cell carcinoma. Ki67 and p53 expression in ghost cell odontogenic carcinoma. The classification of odontogenic cysts has been widely debated and there has been much debate and controversy about the true nature of some of the lesions. Clinical, radiographic, and histopathological findings showed a simple cystic variant of ccot in the region of the deciduous second molar, with dislocation of the permanent second premolar tooth germ. Odontogenic ghost cell carcinoma displays in addition to a malignant epithelial component the classic features of calcifying odontogenic cyst or tumor, including the pre. Pigmented intraosseous odontogenic lesions are rare with only 47 reported cases in the english literature.
Odontogenic ghost cell carcinoma goldenberg 2004 head. He was asymptomatic until pain symptoms developed prior to initial presentation. Current concepts and occurrence of epithelial odontogenic. Dentinogenic ghost cell tumora rare case report with.
Aggressive malignant epithelial odontogenic ghost cell tumor a cystic odontogenic neoplasm as well as various forms of odontogenic carcinoma was suspected. International collaborative study on ghost cell odontogenic. 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. Adenoid ameloblastoma also contains ghost cells and dentinoid similar to ameloblastomatous coc but has a pseudocribriform morphology. Clear cell odontogenic carcinoma with ghost cells and inductive dentin formationreport of a. Dentinogenic ghost cell tumor a neoplastic variety of. We also reported a patient with ghost cell odontogenic carcinoma with a mutation in the ctnnb1 gene, suggesting that ctnnb1 gene mutations are one of the common features of lesions accompanied by ghost cell keratinization. Pdgct occurs in older adults most commonly in the anterior mandibular region. Odontogenic keratocyst iii odontogenic keratocyst is noted for its.
We report herein a case of a ghost cell odontogenic carcinoma arising in the mandible of a caucasian male 86 years of age. Peripheral calcifying cystic odontogenic tumour of the. Dentinogenic ghost cell tumors dgct are very rare tumors considered as solid variants of calcifying epithelial odontogenic cysts ceoc. We report a case of aggressive epithelial odontogenic ghost cell tumor arising from the mandible in a 32yearold man. A case occurring in posterior molar region of the mandible in a 75yearold edentulous woman is reported. Item does not contain fulltextghost cell odontogenic carcinoma gcoc is the malignant counterpart of calcifying cystic odontogenic tumour and dentinogenic ghost cell tumour. Gorlin nevoid basal cell carcinoma syndrome basal cell carcinomas in nonsun exposed areas. Pdf ghost cell odontogenic carcinoma of the maxilla.
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. C440c449 88253 myofibroblastic sarcoma c470c476,c478c479 88253 myofibroblastic sarcoma 95423 epithelioid malignant peripheral nerve sheath tumor c480c482,c488. The 2005 who histological classification of odontogenic tumors lays out a classification system for neoplasms and other tumors related to the odontogenic apparatus. Primary intraosseous squamous cell carcinoma pioscc is a rare epithelial odontogenic carcinoma in the jaw. Dentinogenic ghost cell tumor a neoplastic variety of calcifying odontogenic cyst. Odontogenic carcinoma has the dentinoid material but very few ghost cells and shows infiltration to adjacent stroma. 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. Clear cell odontogenic carcinoma ccoc, clear cell odontogenic tumor previously called clear cell ameloblastoma considered benign by who of 1992, but due to its high potential for regional spread and distant metastases, it was reclassified as malignant in 2005.