The total number of central and peripheral cases reported in the english literature is 10 and although mucosal infiltration is common, peripheral origin of the neoplasm could be verified in only 3 cases. 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. Most pilomatricomas increase slowly in size, some cease to grow to a small size while others grow upto a size of 15 cms. Notch as a possible cell differentiation factor in. Ghost cells gcs are anucleate cells with homogeneous pale eosinophilic cytoplasm and very pale to clear central areas previous nucleus sites. Calcifying cystic odontogenic tumor dentinogenic ghost cell tumor 3. Current concepts and occurrence of epithelial odontogenic tumors. It incorporates the newest techniques for evaluation and treatment, including endoscopic approaches to the skull base, and offers a wealth of evidencebased data and analysis. It is considered to be a solid variant of calcifying odontogenic cyst coc. The dentinogenic ghost cell tumor dgct is a rare benign odontogenic tumor histologically characterized by islands of odontogenic epithelium with the presence of ghost cells and dysplastic dentin at different stages of calcification. Therefore, a comprehensive analysis was performed of the clinical and radiologic features of reported cases of dgct with an emphasis on treatment modalities.
Dentinogenic ghost cell tumor dgct is a rare, odontogenic neoplasm which is considered to be a solid variant of calcifying odontogenic cyst coc with locally aggressive behavior. In 1962, coc was recognized as a distinct clinicopathological entity by gorlin et al. Peripheral dentinogenic ghost cell tumor kamat ss, diwakar. Dentinogenic ghost cell tumors dgct are uncommon neoplasms classified as solid. Foreign body giant cell reaction, which represents a granulomatous response to the shadow cells, can also be identified in regions. Board certified in both oral pathology and oral medicine, dr. At the time of writing 2016, it is still the most widely used classification system although a. 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. Although gcs are present in a variety of odontogenic lesions notably the calcifying cystic odontogenic tumor gcot, their nature. A case of peripheral calcifying odontogenic cyst is presented. Clear cell odontogenic carcinoma ghost cell odontogenic carcinoma odontogenic sarcomas ameloblastic. Calcifying cystic odontogenic tumor ccot demonstrates considerable diversity in histopathology and clinical behavior. Calcification is mostly seen in the ghost cell regions, with the incidence ranging from 69% to 85% 46 figure 2e and f. Coc constitute 12% of all odontogenic tumors and 214% of coc are mainly solid tumors 1.
In this article, we report an extremely rare case of. Dentinogenic ghost cell tumora rare case report with. Dentinogenic ghost cell tumor there is proliferative epithelium and ghost cells interspersed with abundant dentinoid like material, hence the lesion is called dentinogenic ghost cell tumor. Ghost cell odontogenic carcinoma gcoc is the rare malignant counterpart of ccot and it frequently arises from malignant transformation of a recurrent ccot. Adequate surgical intervention is required for aggressive lesions like dentinogenic ghost cell tumor. Odontogenic ghost cell carcinoma johns hopkins university. Dentinogenic ghost cell tumor dgct is a rare tumorous form of calcifying odontogenic cyst and only a small number of cases have been described. Peripheral calcifying cystic odontogenic tumour of the. Copy of dentinogenic ghost cell tumor ready dental. It is more common in males with an age peak at the fourth decade of life 2. A comprehensive atlas and text provides all the assistance you need to accurately identify even the most challenging lesions. Dentinogenic ghost cell tumor dgct has been reported in the literature under different names starting with calcifying ghost cell odontogenic tumor by fejerskov and krogh in 1972. A ghost cell is an enlarged eosinophilic epithelial cell with eosinophilic cytoplasm but without a nucleus. The central intraosseous calcifying odontogenic cyst.
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. Treatment was based on aggressive local resection with adequate safety margins, and monitoring the patient for detection of recurrences. Extraosseous lesions are typically exophytic masses. These authors preferred the term epithelial odontogenic ghost cell tumor to dentinogenic ghost cell tumor, because the latter connotes a mesenchymal tissue origin and the production of true dentin, whereas. Dentinogenic ghost cell tumor of the peripheral variant mimicking. The extraosseous odontogenic tumors may raise diagnostic challenges due to their imitation for reactive gingival lesions. Calcifying cystic odontogenic tumour, dentinogenic ghost cell tumour and ghost cell odontogenic carcinoma. Ghost cell odontogenic carcinoma arising from calcifying. International collaborative study on ghost cell odontogenic tumours.
Dentinogenic ghost cell tumor is a rare odontogenic neoplasm. Dentinogenic ghost cell tumor of the peripheral variant. The dentinogenic ghost cell tumor dgct is an uncommon odontogenic neoplasm, regarded as a solid variant of the calcifying odontogenic cyst coc. The present report describes a case of a 21yearold male with a tumor in. Dentinogenic ghost cell tumors dgcts are uncommon neoplasms classified as a solid variant of the calcifying odontogenic cyst and defined as a locally invasive. The diagnosis and treatment of dentinogenic ghost cell tumor. The dentinogenic ghost cell tumor, journal of oral. Uniqueness and rarity of the lesion is evident from the literature. Sookbin woo draws on her extensive clinical experience to help you achieve diagnostic certainty.
Coc constitute 12% of all odontogenic tumors and 214% of coc are mainly solid tumors. Tumors derived from odontogenic mesenchyme andor odontogenic ectomesenchyme with or without included odontogenic epithelium. Peripheral dentinogenic ghost cell tumor is a rare tumor with only 24 cases previously described in the english literature. The peripheral variant of this clinical rarity appears as a wellcircumscribed mass mimicking a nonspecific gingival enlargement. We present a new case of pdgct located in the maxillary premolar gingiva of a. Clinical pathologic correlations, 7th edition presents diseases and conditions by appearance and presentation, so that you can easily identify and plan treatment for common oral and paraoral problems. Ghost cell odontogenic carcinoma is an extremely rare odontogenic tumor with unclear etiology. The terminology proposed for this lesion is dentinogenic ghost cell tumor. Predictive factors of potential malignant transformation. Dental panoramic pantomogram showing a multilocular radiolucency that straddles the midline of the.
Odontogenic ghost cell carcinoma ogcc, a malignant counterpart of the calcifying odontogenic cyst coc, is exceedingly rare. Clinicopathologic analysis and syndecan1 and ki67 expression in calcifying cystic odontogenic tumors, dentinogenic ghost cell tumor, and ghost cell odontogenic carcinoma. Wnt signaling as a possible promoting factor of cell. Current concepts and occurrence of epithelial odontogenic. Dentinogenic ghostcell tumor dgct is a rare, odontogenic neoplasm which is considered to be a solid variant of the calcifying odontogenic cyst coc with locally. We present a case of dentinogenic ghost cell tumor. Pdf dentinogenic ghost cell tumors dgcts are uncommon neoplasms classified as a solid variant of the. Hence, patients should be treated with more radical approach and should be kept on long term. Betacatenin is expressed aberrantly in tumors expressing shadow cells. Covering tumors of the nose, sinuses, and nasopharynx for the first time in a single volume, this book is essential for all specialists.
Immunohistochemistry bcl 2 and mib1 positivity for cells of odontogenic epithelium the ghost cells and dentinoid. In this paper, we present a case of ccot and discuss its distinct histopathologic features in recurrence. Dentinogenic ghost cell tumor dgct is a very rare odontogenic lesion, with most knowledge based on single case reports. We report a case of aggressive epithelial odontogenic ghost cell tumor arising from the mandible in a 32yearold man. The tumor consisted predominantly of ghost cells with occasional areas of dentinoid in close proximity to masses of ghost cells. Dentinogenic ghost cell tumor dgct is a rare, odontogenic neoplasm which is considered to be a solid variant of the calcifying odontogenic cyst coc with locally. Notch signaling and ghost cell fate in the calcifying. Peripheral dentinogenic ghost cell tumor, journal of oral. Ghost cell odontogenic carcinoma it is also called as malignant epithelial odontogenic tumor. Odontogenic fibroma odontogenic myxoma cementoblastoma the combination of two odontogenic tumors is a rarely reported finding5. The 2005 who histological classification of odontogenic tumors lays out a classification system for neoplasms and other tumors related to the odontogenic apparatus. It is a locally invasive neoplasm that is characterized by ameloblastomalike epithelial islands, ghost cells and dentinoid. Dentinogenic ghost cell tumor dgct, also known as odontogenic ghost cell tumor, is considered as a rare neoplastic variant of calcifying odontogenic cyst coc. The different imaging and histological studies confirmed the diagnosis of a dentinogenic ghost cell tumor.
The dentinogenic ghost cell tumor comes under the spectrum of ghost cell lesions. This is a retrospective study of patients with dgct who underwent one or multiple surgical interventions during the 10year period from 1997 to 2007. Dentinogenic ghost cell tumor comprises less than 0. This tumor makes up for only 2%14% of all cocs and less than 0. Showing areas of ghost cells, keratin material, giant cell reaction and inflammatory infiltrate. Dentinogenic ghost cell tumor dgct is an uncommon locally invasive odontogenic tumor regarded by many as a variant of calcifying odontogenic cyst. The purpose of this study is to assess the diagnosis, treatment and prognosis of the dentinogenic ghost cell tumor dgct. Hassanein am, glanz sm, kessler hp, eskin ta, liu c. Dentinogenic ghost cell tumor a neoplastic variety of. Notch signaling is an evolutionarily conserved mechanism that enables adjacent cells to adopt different fates. The tumor displayed a variety of densities and signal intensities on ct and mr. Pilomatricoma, craniopharyngioma, and calcifying odontogenic cyst.
Volume 37, issue 4, octoberdecember 2015, pages 243246. Dentinogenic ghost cell tumor odontogenic ectomesenchymal odontogenic. Showing whorls of keratin in the center of the islands. Dgct is a locally invasive odontogenic neoplasm characterised by. Tumors of the nose, sinuses and nasopharynx valerie j. The majority of cases have been reported to occur in the anterior part. It is characterized by ameloblastomatous odontogenic epithelium, presence of ghost cells and dentinoid material. Pdf dentinogenic ghost cell tumor dgct is a rare, odontogenic neoplasm which is considered to be a solid variant of calcifying odontogenic cyst. 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.
Dentinogenic ghost cell tumor dgct is a rare epithelial odontogenic neoplasm, representing 1. It shows slight male predilection with a tendency to occur in the anterior segment of the jaws canine to first molar, with equal frequency in maxilla and mandible. 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. An unusual case of dentinogenic ghost cell tumor, a peculiar variant of calcifying odontogenic cyst, is reported. Investigation of the betacatenin gene in a case of dentinogenic ghost cell tumor. Only 214% of coc are solid tumors and are considered to be dgcts. The dentinogenic ghost cell tumor seems to be more aggressive than ccot. Ccot can occur peripherally or centrally, although only % of ccots are extraosseous. Pdgct occurs in older adults most commonly in the anterior mandibular region. Dentinogenic ghost cell tumor commonly occurs in elderly, with an average age for the presentation being 50 years range.
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