Dentinogenic ghost cell tumor

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Dentinogenic ghost cell tumours (DGCT) are benign mixed epithelial and mesenchymal odontogenic tumours with a locally aggressive behaviour.

Terminology

It is also known as the solid, neoplastic'solid' or 'neoplastic form of ‘calcifyingcalcifying odontogenic cyst’, since the 4th WHO classification of head and neck tumours in 2017, both entities are distinguished 1.

Epidemiology

Dentinogenic ghost cell tumoursare very rare tumours with a wide age range and a peak incidence in the fourth and fifth decade. The tumours are more frequent in men 2.

Diagnosis

The diagnosis is established histologically with the presence of >1-2% ghost cells and the presence of dentoid 1,3.

Clinical presentation

Intraosseous dentinogenic ghost cell tumours are usually asymptomatic and incidentally found on imaging studies 2. Extraosseous lesion might present as exophytic sometimes pedunculated soft tissue nodule 2,3.

Complications

Dentinogenic ghost cell tumours might cause cortical resorption and extend into the soft tissues 2.

Pathology

Dentinogenic ghost cell tumours have a characteristic morphology with aan epithelial component looking like ameloblastoma 1.

Location

The tumoursare 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.

Microscopic appearance

Microscopically dentinogenic ghost cell tumours might show the following histological features 1-4:

  • prominent basaloid to stellate reticulum cells lined by peripheral columnar cells
  • areas of calcification

    keartinization

  • keratinization with ghost cells

Radiographic features

Plain radiograph

Dentinogenic ghost cell tumours usually appear radiolucent or mixed radiolucent/radiopaque on orthopantomogram they might be unilocular or multilocular 2.

CT

CT might show an expansile, destructive lesion with calcifications 3,4.

Radiology report

A desciptiondescription of the following features should be included intoin the radiology report:

  • type location and size of the tumour
  • unilocular/multilocular
  • presence of calcifications
  • relation to the adjacent structures and teeth

Treatment and prognosis

Wide local excision has been recommended for of intraosseous lesions and more conservative excision for extraossousextraosseous tumours due to the tumourstumour aggressiveness 1. Tumours can locally recur and therefore longtermlong-term follow-up is recommended 1,2.

History and etymology

The term dentinogenic ghost cell tumour was suggested by Praetorius and colleagues in 1981 4,5.

Differential diagnosis

The differential diagnosis of a dentinogenic ghost cell tumour includes the following 1-4:

  • -<p><strong>Dentinogenic ghost cell tumours (DGCT) </strong>are benign mixed epithelial and mesenchymal odontogenic tumours with a locally aggressive behaviour.</p><h4>Terminology</h4><p>It is also known as the solid, neoplastic form of ‘calcifying odontogenic cyst’, since the 4th WHO classification of head and neck tumours in 2017 both entities are distinguished <sup>1</sup>.</p><h4>Epidemiology</h4><p>Dentinogenic ghost cell tumours<strong> </strong>are very rare tumours with a wide age range and a peak incidence in the fourth and fifth decade. The tumours are more frequent in men <sup>2</sup>.</p><h4>Diagnosis</h4><p>The diagnosis is established histologically with the presence of &gt;1-2% ghost cells and the presence of dentoid <sup>1,3</sup>.</p><h4>Clinical presentation</h4><p>Intraosseous dentinogenic ghost cell tumours are usually asymptomatic and incidentally found on imaging studies <sup>2</sup>. Extraosseous lesion might present as exophytic sometimes pedunculated soft tissue nodule <sup>2,3</sup>.</p><h5>Complications</h5><p>Dentinogenic ghost cell tumours might cause cortical resorption and extend into the soft tissues <sup>2</sup>.</p><h4>Pathology</h4><p>Dentinogenic ghost cell tumours have a characteristic morphology with a epithelial component looking like ameloblastoma <sup>1</sup>.</p><h5>Location</h5><p>The tumours<strong> </strong>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 <sup>2</sup>.</p><h5>Microscopic appearance</h5><p> </p><p>Microscopically dentinogenic ghost cell tumours might show the following histological features <sup>1-4</sup>:</p><p>prominent basaloid to stellate reticulum cells lined by peripheral columnar cells</p><p>areas of calcification</p><p>keartinization with ghost cells</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Dentinogenic ghost cell tumours usually appear radiolucent or mixed radiolucent/radiopaque on orthopantomogram they might be unilocular or multilocular <sup>2</sup>.</p><h5>CT</h5><p>CT might show an expansile, destructive lesion with calcifications <sup>3,4</sup>.</p><h4>Radiology report</h4><p>A desciption of the following features should be included into the radiology report:</p><p>type location and size of the tumour</p><p>unilocular/multilocular</p><p>presence of calcifications</p><p>relation to the adjacent structures and teeth</p><h4>Treatment and prognosis</h4><p>Wide local excision has been recommended for of intraosseous lesions and more conservative excision for extraossous tumours due to the tumours aggressiveness <sup>1</sup>. Tumours can locally recur and therefore longterm follow-up is recommended <sup>1,2</sup>.</p><p> </p><h4>History and etymology</h4><p>The term dentinogenic ghost cell tumour was suggested by Praetorius and colleagues in 1981 <sup>4,5</sup>.</p><h4>Differential diagnosis</h4><p>The differential diagnosis of a dentinogenic ghost cell tumour includes the following <sup>1-4</sup>:</p><p>ameloblastoma</p><p>central giant cell granuloma</p><p>central ossifying fibroma</p>
  • +<p><strong>Dentinogenic ghost cell tumours (DGCT) </strong>are benign mixed epithelial and mesenchymal odontogenic tumours with locally aggressive behaviour.</p><h4>Terminology</h4><p>It is also known as the 'solid' or 'neoplastic form of calcifying odontogenic cyst’, since the 4th <a href="/articles/who-classification-of-head-and-neck-tumors">WHO classification of head and neck tumours</a> in 2017, both entities are distinguished <sup>1</sup>.</p><h4>Epidemiology</h4><p>Dentinogenic ghost cell tumours<strong> </strong>are very rare tumours with a wide age range and a peak incidence in the fourth and fifth decade. The tumours are more frequent in men <sup>2</sup>.</p><h4>Diagnosis</h4><p>The diagnosis is established histologically with the presence of &gt;1-2% ghost cells and the presence of dentoid <sup>1,3</sup>.</p><h4>Clinical presentation</h4><p>Intraosseous dentinogenic ghost cell tumours are usually asymptomatic and incidentally found on imaging studies <sup>2</sup>. Extraosseous lesion might present as exophytic sometimes pedunculated soft tissue nodule <sup>2,3</sup>.</p><h5>Complications</h5><p>Dentinogenic ghost cell tumours might cause cortical resorption and extend into the soft tissues <sup>2</sup>.</p><h4>Pathology</h4><p>Dentinogenic ghost cell tumours have a characteristic morphology with an epithelial component looking like <a href="/articles/ameloblastoma">ameloblastoma</a> <sup>1</sup>.</p><h5>Location</h5><p>The tumours<strong> </strong>are more frequently found in an intraosseous location in the posterior <a href="/articles/maxilla">maxilla</a> and posterior <a href="/articles/mandible">mandible</a>. If found in an extraosseous position it is seen typically in the anterior part of the jawbone <sup>2</sup>.</p><h5>Microscopic appearance</h5><p>Microscopically dentinogenic ghost cell tumours might show the following histological features <sup>1-4</sup>:</p><ul>
  • +<li>prominent basaloid to stellate reticulum cells lined by peripheral columnar cells</li>
  • +<li>areas of calcification</li>
  • +<li>keratinization with ghost cells</li>
  • +</ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Dentinogenic ghost cell tumours usually appear radiolucent or mixed radiolucent/radiopaque on orthopantomogram they might be unilocular or multilocular <sup>2</sup>.</p><h5>CT</h5><p>CT might show an expansile, destructive lesion with calcifications <sup>3,4</sup>.</p><h4>Radiology report</h4><p>A description of the following features should be included in the radiology report:</p><ul>
  • +<li>type location and size of the tumour</li>
  • +<li>unilocular/multilocular</li>
  • +<li>presence of calcifications</li>
  • +<li>relation to the adjacent structures and teeth</li>
  • +</ul><h4>Treatment and prognosis</h4><p>Wide local excision has been recommended for intraosseous lesions and more conservative excision for extraosseous tumours due to the tumour aggressiveness <sup>1</sup>. Tumours can locally recur and therefore long-term follow-up is recommended <sup>1,2</sup>.</p><h4>History and etymology</h4><p>The term dentinogenic ghost cell tumour was suggested by Praetorius and colleagues in 1981 <sup>4,5</sup>.</p><h4>Differential diagnosis</h4><p>The differential diagnosis of a dentinogenic ghost cell tumour includes the following <sup>1-4</sup>:</p><ul>
  • +<li><a href="/articles/ameloblastoma">ameloblastoma</a></li>
  • +<li><a href="/articles/calcifying-odontogenic-cyst">calcifying odontogenic cyst</a></li>
  • +<li><a href="/articles/central-giant-cell-lesions-granuloma">central giant cell granuloma</a></li>
  • +<li><a href="/articles/cemento-ossifying-fibroma">central ossifying fibroma</a></li>
  • +</ul>

References changed:

  • 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. <a href="https://doi.org/10.1055/s-0040-1710357">doi:10.1055/s-0040-1710357</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/33036041">Pubmed</a>
  • 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. <a href="https://doi.org/10.17096/jiufd.52886">doi:10.17096/jiufd.52886</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29354306">Pubmed</a>
  • 3. Biggs T, Hayes S, Harries P, Salib R. Maxillary Dentinogenic Ghost Cell Tumour. Annals. 2013;95(3):e9-e11. <a href="https://doi.org/10.1308/003588413x13511609957010">doi:10.1308/003588413x13511609957010</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23827283">Pubmed</a>
  • 4. Bafna S, Joy T, Tupkari J, Landge J. Dentinogenic Ghost Cell Tumor. J Oral Maxillofac Pathol. 2016;20(1):163. <a href="https://doi.org/10.4103/0973-029x.180985">doi:10.4103/0973-029x.180985</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27194885">Pubmed</a>
  • 5. Prætorius F, Hjørting-Hansen E, Gorlin R, Vickers R. Calcifying Odontogenic Cyst. Acta Odontol Scand. 1981;39(4):227-40. <a href="https://doi.org/10.3109/00016358109162284">doi:10.3109/00016358109162284</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/6948493">Pubmed</a>

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  • Head & Neck

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