Dentinogenic ghost cell tumor
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Dentinogenic ghost cell tumors (DGCT) are benign mixed epithelial and mesenchymal odontogenic tumors with locally aggressive behavior.
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It is also known as the 'solid' or 'neoplastic form of calcifying odontogenic cyst’, since the 4th WHO classification of head and neck tumors in 2017, both entities are distinguished 1.
Dentinogenic ghost cell tumors are very rare tumors with a wide age range and a peak incidence in the fourth and fifth decade. The tumors are more frequent in men 2.
The diagnosis is established histologically with the presence of >1-2% ghost cells and the presence of dentoid 1,3.
Intraosseous dentinogenic ghost cell tumors are usually asymptomatic and incidentally found on imaging studies 2. Extraosseous lesion might present as exophytic sometimes pedunculated soft tissue nodule 2,3.
Dentinogenic ghost cell tumors might cause cortical resorption and extend into the soft tissues 2.
Dentinogenic ghost cell tumors have a characteristic morphology with an epithelial component looking like ameloblastoma 1.
The tumors are more frequently found in an intraosseous location in the posterior maxilla and posterior mandible. If found in an extraosseous position it is seen typically in the anterior part of the jawbone 2.
Microscopically dentinogenic ghost cell tumors might show the following histological features 1-4:
- prominent basaloid to stellate reticulum cells lined by peripheral columnar cells
- areas of calcification
- keratinization with ghost cells
Dentinogenic ghost cell tumors usually appear radiolucent or mixed radiolucent/radiopaque on orthopantomogram they might be unilocular or multilocular 2.
CT might show an expansile, destructive lesion with calcifications 3,4.
A description of the following features should be included in the radiology report:
- type location and size of the tumor
- presence of calcifications
- relation to the adjacent structures and teeth
Treatment and prognosis
Wide local excision has been recommended for intraosseous lesions and more conservative excision for extraosseous tumors due to the tumor aggressiveness 1. Tumors can locally recur and therefore long-term follow-up is recommended 1,2.
History and etymology
The term dentinogenic ghost cell tumor was suggested by Praetorius and colleagues in 1981 4,5.
The differential diagnosis of a dentinogenic ghost cell tumor includes the following 1-4:
- 1. Siozopoulou V & Vanhoenacker F. World Health Organization Classification of Odontogenic Tumors and Imaging Approach of Jaw Lesions. Semin Musculoskelet Radiol. 2020;24(05):535-48. doi:10.1055/s-0040-1710357 - Pubmed
- 2. Wright J & Soluk Tekkeşin M. Odontogenic Tumors. Where Are We in 2017? J Istanbul Univ Fac Dent. 2017;51(3 Suppl 1):S10-30. doi:10.17096/jiufd.52886 - Pubmed
- 3. Biggs T, Hayes S, Harries P, Salib R. Maxillary Dentinogenic Ghost Cell Tumour. Annals. 2013;95(3):e9-e11. doi:10.1308/003588413x13511609957010 - Pubmed
- 4. Bafna S, Joy T, Tupkari J, Landge J. Dentinogenic Ghost Cell Tumor. J Oral Maxillofac Pathol. 2016;20(1):163. doi:10.4103/0973-029x.180985 - Pubmed
- 5. Prætorius F, Hjørting-Hansen E, Gorlin R, Vickers R. Calcifying Odontogenic Cyst. Acta Odontol Scand. 1981;39(4):227-40. doi:10.3109/00016358109162284 - Pubmed