The calcifying odontogenic cyst coc was first described in 1962 by gorlin et al. Jan 27, 2014 odontogenic ghost cell carcinoma ogcc is a rare odontogenic tumor characterized by the presence of ghost cells. 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. Threedimensional volumetric analysis of ghost cell odontogenic. Ghost cell odontogenic carcinoma on right mandible and its. Odontogenic ghost cell carcinoma with pulmonary metastasis.
A 70yearold woman presented with a large painful radiolucent mandibular lesion from the right canine to the left angle area through the midline. Clear cell odontogenic carcinoma it is a lowgrade carcinoma that is composed of cells that show uniform nuclei and clear cytoplasm. 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. Until now, only 31 cases of ogccs have been reported in literature. Ghost cell odontogenic carcinoma is an extremely rare odontogenic tumor with unclear etiology.
We have described the clinical and radiographic features, histological. 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 is a rare condition characterized by ameloblasticlike islands of epithelial cells with aberrant keratinitation in the form of ghost cell with varying amounts of dysplastic dentina. It is potentially aggressive and capable of multiple local recurrences and locoregional and distant metastases. Odontogenic cysts can give rise to a variety of neoplasms. Primary intraosseous squamous cell carcinoma derived from keratinizing cystic odontogenic tumor.
It most often affects the posterior mandible and most commonly presents in the third decade of life. Calcifying epithelial odontogenic tumor an overview. Nevoid basal cell carcinoma syndrome gorlin goltz syndrome ccot calcifying cystic odontogenic tumor derived from odontogenic epithelial cell rest. Odontogenic ghost cell carcinoma johns hopkins university. Malignant transformation of calcifying epithelial odontogenic. Due to significant characteristics, they differ from bone tumours seen elsewhere in the skeleton. Squamous cell carcinoma scc in a survey of dogs with oropharyngeal tumors, scc was the second most frequently diagnosed oral tumor 24. Clear cell variant of calcifying epithelial odontogenic tumor. About 70% of patients are women with an age range from 1789 years. Clear cell odontogenic tumora new histologic variant with aggressive potential. It is more common in males with an age peak at the fourth decade of life 2. Mandibular clear cell odontogenic carcinoma world journal. It is a potentially aggressive tumour, capable of recurring frequently, locally invasive, and with distant metastases. Ghost cell odontogenic carcinoma is a very rare malignant neoplasm.
In some cases these small calcifications combine to form large masses 20, 21. Odontogenic tumors questions and study guide quizlet. Keratocystic odontogenic tumor 15 62 10 38 ameloblastoma 9 50 9 50 odontoma 6 46 7 54 odontogenic myxoma 1 50 1 50 calcifying cystic odontogenic tumor 2 100 cementoblastoma 1 100 central odontogenic fibroma 1 100 calcifying epithelial odontogenic tumor 1 100 total 32 51 31 49. Curettage or conservative resection inevitably results in recurrences andor metastasis and more radical resection is warranted in these tumors, especially when they are large and show soft tissue invasion. The clear cell variant of calcifying epithelial odontogenic tumor may contain areas that resemble the clear cell odontogenic carcinoma. Primary intraosseous squamous cell carcinoma arising in. Study 52 odontogenic tumors flashcards from dental s.
Pdf odontogenic tumors are a heterogeneous group of lesions of diverse clinical behavior and. Eogct stands for epithelial odontogenic ghost cell tumor. How is epithelial odontogenic ghost cell tumor abbreviated. This book greatly expands the body of knowledge in the field of odontogenic tumor pathology. Significance of silverstained nucleolar organizer regions in early diagnosis and prognosis of oral squamous cell carcinoma. Maxillary clear cell odontogenic carcinoma a rare entity. This is the case of a middleaged male who presented with a slowgrowing maxillary tumour. Martosfernandez m, alberolaferranti m, huetomadrid ja, bescosatin c. 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. Clear cell odontogenic carcinoma should be considered in the differential diagnosis of jaw tumors with conspicuous clear cell component.
We report a case of a 70 yearold woman with a rapid onset of painful swelling right maxillary tumor. An aggressive intraosseous lesion derived from embryonic connective tissue associated with odontogenesis and primarily consisting of a mucoid ground substance with widely scattered undifferentiated spindled mesenchymal cells. There have been very few reports about gcocs arising from. 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. Dentinogenic ghost cell tumor a neoplastic variety of.
Other less common odontogenic malignant tumors include ghost cell odontogenic carcinoma and primary intraosseous squamous cell carcinomas 1, 16. In this report, we present a case of nevoid basal cell carcinoma syndrome in a 26yearold male patient. 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. Clear cell odontogenic carcinoma ccoc is a rare odontogenic tumor with predominance in the posterior mandible, usually occurring in the fifth to seventh decades of life, and being found more commonly in females. The islands look identical to ameloblastoma, but that stroma is mature and dense whereas this ct is loose and resembles primitive dental papilla. It describes the epidemiologic, radiologic, and histopathologic features of presently known neoplasms and hamartomatous or tumorlike lesions arising from the toothforming structures, as well as a number of distinctive jaw lesions that must be distinguished from odontogenic tumors. Review article pathogenesis and nomenclature of odontogenic. Nonodontogenic tumours of the jaw originating from bone and its mesenchymal tissue represent a large heterogeneous group of diverse diseases. Ghost cell odontogenic carcinoma gcoc is a rare malignant odontogenic tumor, only 37 cases of gcoc have been reported in the. Pathogenesis and nomenclature of odontogenic carcinomas. Ghost cell odontogenic carcinoma arising from calcifying. Dec 12, 2006 clear cell odontogenic carcinoma should be considered in the differential diagnosis of jaw tumors with conspicuous clear cell component.
Clear cell odontogenic carcinoma mimicking a cystic lesion. Multiple organ systems may be affected in this syndrome including abnormalities of the skin, skeletal system, genitourinary system and central nevus system. Calcifying epithelial odontogenic tumor ceot is a rare, benign, locally aggressive odontogenic epithelial tumor that affects the jaws. Odontogenic ghost cell carcinoma ogcc is a rare odontogenic tumor characterized by the presence of ghost cells. 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. He was asymptomatic until pain symptoms developed prior to. Clear cell odontogenic carcinomaa rare case report. There are previous reports of ameloblastic carcinoma misdiagnosed as ameloblastoma and treated more conservatively, leading to. Ki67 and p53 expression in ghost cell odontogenic carcinoma.
Ghost cell odontogenic carcinoma gcoc is the malignant counterpart of calcifying cystic odontogenic tumour and dentinogenic ghost cell tumour. Odontogenic tumors at university of colorado health sciences. A diagnosis of clear cell odontogenic carcinoma ccocameloblastomatous variant was made after. An odontogenic keratocyst is a rare and benign but locally aggressive developmental cyst. Multiple basal cell carcinomas multiple jaw cysts odontogenic keratocysts numerous bone abnormalities including bi. Clear cell odontogenic carcinoma ccoc is a rare intraosseous carcinoma of the jaw. Gorlin nevoid basal cell carcinoma syndrome basal cell carcinomas in nonsun exposed areas. The software made it possible to combine a particular slice of the ct scan and the 3 d. Odontogenic ghost cell carcinoma ogcc, a malignant counterpart of the calcifying odontogenic cyst coc, is exceedingly rare. Odontogenic tumors comprise a complex group of lesions with varied histopathological and clinical features. Some are composed only of odontogenic epithelium, while many are mixed i. He was asymptomatic until pain symptoms developed prior to initial presentation.
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. It has sheets and strands of odontogenic epithelial cells. Calcifying ghost cell odontogenic cyst is comparatively rare in occurrence, constituting about 0. The 2005 who histological classification of odontogenic tumors lays out a classification system for neoplasms and other tumors related to the odontogenic apparatus.
Eogct epithelial odontogenic ghost cell tumor acronymfinder. Clear cell odontogenic tumor is a rare neoplasm of the jaws that histologically may be confused with metastatic renal cell carcinoma. Coc, also referred to as calcifying ghost cell odontogenic cyst cgcoc, is a heterogeneous lesion existing either as cystic or solid variant. Ghost cell odontogenic carcinoma gcoc is a rare malignant odontogenic epithelial tumor with features of benign calcifying odontogenic cysts. The present report describes 2 cases of primary intraosseous squamous cell carcinoma. Malignant ectomesenchymal odontogenic neoplasms odontogenic sarcomas 253. Odontogenic tumors and allied lesions edition 1 by peter a. Nevoid basal cell carcinoma syndrome bcns is an autosomal dominant inherited disorder. Clear cell odontogenic carcinoma ccoc is a rare odontogenic tumour with female predilection occurring in the anterior region of the mandible with peak age incidence of 5th and 7th decade of life. Calcifying cystic odontogenic tumor ccot is defined as an odontogenic cyst like benign neoplasm that characteristically contains several. Case report mandibular ghost cell odontogenic carcinoma.
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. Odontogenic ghost cell carcinoma ogcc is a rare neoplastic variant of calcifying odontogenic cyst, with aggressive clinical characteristics. Dentigerous cysts are one of the most common odontogenic cysts of the oral cavity. Ameloblastic carcinoma, calcifying odontogenic cyst, ghost cell carcinoma, keratinizing epithelial odontogenic cyst, maxillary tumor, odontogenic carcinoma. Some tumors are true neoplasms, while some are hamartomas developmental malformations. Ghost cell odontogenic carcinoma gcoc was first described in detail as a malignant focus within a calcifying odontogenic cyst coc by ikemura et al.925 1285 242 1467 401 322 539 108 631 1614 665 429 105 1353 629 16 1326 1274 1239 900 274 739 941 832 778 98 860 1165 115