Odontogenic ghost cell carcinoma johns hopkins university. Odontogenic ghost cell carcinoma odontogenic ghost cell carcinoma goldenberg, david. T1 orthokeratinized odontogenic cyst with an associated keratocystic odontogenic tumor component and ghost cell keratinization and calcifications in a patient with gardner syndrome. 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. Although gcs are present in a variety of odontogenic lesions notably the calcifying cystic odontogenic tumor gcot, their nature.
Ghost cell odontogenic carcinoma gcoc, a malignant form of ccot, is an exceptionally rare malignant tumor. This book greatly expands the body of knowledge in the field of odontogenic tumor pathology. Ghost cells gcs are anucleate cells with homogeneous pale eosinophilic cytoplasm and very pale to clear central areas previous nucleus sites. It is potentially aggressive and capable of multiple local recurrences and locoregional and distant metastases.
Keratinocystic odontogenic tumor odontogenic keratocyst keratinocystic odontogenic tumor three important things associated with this diagnosis. Nevoid basal cell carcinoma syndrome gorlin goltz syndrome ccot calcifying cystic odontogenic tumor derived from odontogenic epithelial cell rest. Odontogenic cysts can give rise to a variety of neoplasms. Ghost cell odontogenic carcinoma gcoc, a malignant counterpart of calcifying odontogenic cyst coc and dentinogenic ghost cell tumor dgct, is extremely rare. 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. Odontogenic keratocysts make up around 19% of jaw cysts. Notch signaling and ghost cell fate in the calcifying. It is characterized by ameloblasticlike islands of epithelial cells with aberrant keratinization in the form of ghost cells with varying amounts of dysplastic dentin. 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. Odontogenic tumors questions and study guide quizlet. Objectives to report the clinicopathologic findings and followup. Some of the previously reported cases are described as longterm persistent. Calcifying cystic odontogenic tumor ccot is a benign cystic neoplasm of odontogenic origin, characterized by an ameloblastomalike epithelium with ghost cells that may calcify.
A case of exceptionally rare odontogenic malignant tumor, called ghost cell odontogenic carcinoma, is described. An unusual presentation of ghost cell odontogenic carcinoma. Orthokeratinized odontogenic cyst with an associated. Gcoc is defined as a malignant odontogenic epithelial tumor with the features of a calcifying cystic odontogenic tumor, a dentinogenic ghost cell tumor, or both. However, for prognostic reasons, epithelial odontogenic tumors producing calcifications andor ghost cells should be differentially diagnosed based on tumor cellular differentiation. Ghost cell odontogenic carcinoma arising from calcifying. He was asymptomatic until pain symptoms developed prior to initial presentation. Current concepts and occurrence of epithelial odontogenic. Aggressive epithelial odontogenic ghost cell tumor in the.
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 intraosseous squamous cell carcinoma arising in. Calcifying odontogenic cyst gorlin cyst pilomatricoma. 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. This is the case of a middleaged male who presented with a slowgrowing maxillary tumour. The calcifying ghost cell odontogenic cyst cgcoc was first described by gorlin et al. Ghost cell odontogenic carcinoma is an extremely rare odontogenic tumor with unclear etiology. The present report describes 2 cases of primary intraosseous squamous cell carcinoma. The most notable features of this pathologic entity are histopathological features which include a cystic lining demonstrating characteristic ghost epithelial cells with a propensity to calcify.
New tumour entities in the 4th edition of the world health. The odontogenic ghost cell carcinoma ogcc is the malignant counterpart of a calcifying cystic odontogenic tumor and dentinogenic ghost cell tumor. The patient was treated surgically, and one of the surgical margins was involved, the patient received adjuvant radiotherapy for local control. The tumor cells infiltrate into the adjacent fibrovascular tissue. Dentigerous cysts are one of the most common odontogenic cysts of the oral cavity. Ghost cell odontogenic carcinoma gcoc is a rare malignant variant of odontogenic tumor with ghost cells. We report a case of a 70 yearold woman with a rapid onset of painful swelling right maxillary tumor. Ghost cell odontogenic carcinoma gcoc is the malignant counterpart of calcifying cystic odontogenic tumour and dentinogenic ghost cell tumour. Pdf odontogenic ghost cell carcinoma ogcc is a rare neoplastic variant of calcifying odontogenic cyst, with aggressive clinical characteristics. 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. We have described the clinical and radiographic features, histological.
Odontogenic ghost cell carcinoma with pulmonary metastasis. 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. Pathogenesis and nomenclature of odontogenic carcinomas. An odontogenic keratocyst is a rare and benign but locally aggressive developmental cyst. Odontogenic ghost cell carcinoma ogcc is an exceptionally rare malignant odontogenic epithelial tumor. Ghost cell odontogenic carcinoma it is also called as malignant epithelial odontogenic tumor. He was asymptomatic until pain symptoms developed prior to. In the whoiarc classification of head and neck pathology, this clinical entity had been known for years as the odontogenic keratocyst. Odontogenic keratocyst because of its behavior, many oral pathologists now consider the okc an odontogenic tumor that has a cystic form 2005 classi. The tumor was considered to be derived from calcifying odontogenic cyst, which had been resected 5 years before. Dentinogenic ghost cell tumor a neoplastic variety of. The 2005 who histological classification of odontogenic tumors lays out a classification system for neoplasms and other tumors related to the odontogenic apparatus. Calcifying ghost cell odontogenic cyst is comparatively rare in occurrence, constituting about 0.
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. At the time of writing 2016, it is still the most widely used classification system although a. 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. A ghost cell is an enlarged eosinophilic epithelial cell with eosinophilic cytoplasm but without a nucleus. Case report mandibular ghost cell odontogenic carcinoma. Ghost cell odontogenic carcinoma arising in calcifying. References this article related to pathology is a stub. 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. The chart showing pdf series, word series, html series, scan qr codes. Ledesmamontes c, gorlin rj, shear m, praetorius f, et al. Ghost cell odontogenic carcinoma gcoc is a malignant odontogenic epithelial tumor with features of calcifying cystic odontogenic tumor.
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. This article is from korean journal of pathology, volume 48. Odontogenic ghost cell carcinomas ogcc are rare and only a dozen or so have been reported in the literature. Eogct stands for epithelial odontogenic ghost cell tumor. Six months follow up period shows no signs of recurrence. 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. Morgan odontogenic tumors comprise a range of disorders of growth, from malignant and benign. This definition appears very rarely and is found in the following acronym finder categories. Calcifying cystic odontogenic tumor associated with an. 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. Dentinogenic ghost cell tumor a neoplastic variety of calcifying odontogenic cyst. Ghost cell odontogenic carcinoma on right mandible and its.
Ghost cell odontogenic carcinoma, malignant odontogenic tumor. It most often affects the posterior mandible and most commonly presents in the third decade of life. B tumor cells are admixed with anucleate ghost cells. Ghost cell odontogenic carcinoma is a rare malignant tumor. Clear cell odontogenic carcinoma ghost cell odontogenic carcinoma odontogenic sarcomas ameloblastic. As far as possible, descriptions and illustrations in. To our best knowledge, there have been only 27 cases reported so far. Classified as an odontogenic carcinoma, which includes. International collaborative study on ghost cell odontogenic tumours. Odontogenic ghost cell carcinoma ogcc, a malignant counterpart of the calcifying odontogenic cyst coc, is exceedingly rare. Notch signaling is an evolutionarily conserved mechanism that enables adjacent cells to adopt different fates. Ghost cell odontogenic carcinoma with suspected cholesterol. 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.
Chapter 21 odontogenic and nonodontogenic tumors of the jaws introduction. A histopathologic examination shows epithelial cell nests in tumor tissue. Ghost cell odontogenic carcinoma arising in the background of a. In the present resected specimen, cellular atypia, mitotic activity, ki67 labeling index, and p53 positivity were all increased in comparison with the initially. The final chapter discusses how to distinguish between odontogenic tumours and developing odontogenic tissues, an area fraught with difficulties and with severe consequences for the patient if this distinction is not drawn appropriately.