Mandible & maxilla
Malignant odontogenic tumors
Ghost cell odontogenic carcinoma
Last author update: 1 October 2016
Last staff update: 29 October 2020
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PubMed Search: Ghost cell odontogenic carcinoma
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Etiology | Clinical features | Diagnosis | Radiology description | Prognostic factors | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Immunohistochemistry & special stains | Molecular / cytogenetics description | Differential diagnosisCite this page: Magliocca K, Martinez A. Ghost cell odontogenic carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mandiblemaxillaghostcellodontogeniccarcinoma.html. Accessed November 27th, 2024.
Definition / general
- 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
- Classified as an odontogenic carcinoma, which includes:
- Metastasizing (malignant) ameloblastoma
- Ameloblastic carcinoma
- Primary intraosseous squamous cell carcinoma
- Solid type
- Derived from keratocystic odontogenic tumor
- Derived from odontogenic cysts
- Clear cell odontogenic carcinoma
- Ghost cell odontogenic carcinoma
Terminology
- Odontogenic ghost cell tumor
- Calcifying ghost cell odontogenic carcinoma
- Malignant epithelial odontogenic ghost cell tumor
- Aggressive epithelial ghost cell odontogenic tumor
- Carcinoma arising in a calcifying odontogenic cyst
- Malignant calcifying ghost cell odontogenic tumor
- Malignant calcifying odontogenic cyst
Epidemiology
- Rare, < 40 cases reported in literature and more than half from Asia
- More common in males
Sites
- Maxilla more common than mandible
Etiology
- Malignancy thought to arise from calcifying ondontogenic cysts (COCs) with features of either calcifying cystic odontogenic tumor or dentinogenic ghost cell tumor
Clinical features
- Can present as painful, hard swelling in the maxilla or mandible
- Can also have paresthesia associated with root resorption or tooth displacement
Diagnosis
- Diagnosis dependent on clinical, radiologic and pathologic correlation
Radiology description
- Most commonly seen as a mixed radiolucency and radio opacity with ill defined margins
Prognostic factors
- Overall 5 year survival rate is 73% and recurrence is common
Case reports
- 21 year old man with ghost cell odontogenic carcinoma (GCOC) (Pan Afr Med J 2015;21:260)
- 54 year old man with tender swelling in the malar region (J Oral Maxillofac Pathol 2015;19:371)
- 64 year old woman with metastatic odontogenic carcinoma arising from dentinogenic ghost cell tumor (Rare Tumors 2015;7:5813)
- 70 year old woman with rapid onset of painful swelling right maxillary tumor (J Clin Exp Dent 2014;6:e602)
Treatment
- Composite surgical excision, may be followed by radiation
Clinical images
Images hosted on other servers:
Diffused swelling on the left side of face obliterating the nasolabial fold
Clinical and MRI examination
Axial section CT scan
Microscopic (histologic) description
- Ameloblastomatous areas: peripheral palisading, reverse polarization, stellate reticulum
- Ghost cells (polygonal epithelial cells with eosinophilic cytoplasm that have lost their nuclei but maintain a faint outline of cellular and nuclear membrane)
- Ghost cells may be calcified
- Atypia with changes such as increased cellularity, pleomorphism, mitosis, necrosis and infiltrative growth
Microscopic (histologic) images
Images hosted on other servers:
Ameloblastoma-like areas
Ghost cells
Cytologic atypia with increased mitotic rate
Ghost cells
Immunohistochemistry & special stains
- Positive for pan-cytokeratin, p63
- Often have an increased Ki67 proliferative index
- Can also show p53 expression
Molecular / cytogenetics description
- Limited literature, but aberrations of Wnt signaling pathway with beta catenin overexpression have been shown in calcifying cystic odontogenic tumors (APMIS 2008;116:206)
Differential diagnosis
- Ameloblastic carcinoma
- Variable features of ameloblastoma: peripheral palisading, reverse polarization, stellate reticulum-like cells
- Features of malignancy include cytological atypia, high N:C ratio, increased mitoses with atypical forms, necrosis
- Can also metastasize
- No ghost cells
- Benign epithelial odontogenic neoplasm
- Occurs in posterior mandible, intraosseous location
- Variably sized polyhedral eosinophilic epithelial cells with distinct cell borders are arranged in small clusters, trabeculae, islands or a sheet-like pattern
- Nuclear pleomorphism is expected, but without appreciable mitotic activity
- Eosinophilic amyloid-like matrix material is haphazardly deposited in association with the tumor islands, and calcified concentric profiles (Liesegang rings) are often identified
- Calcifying cystic odontogenic tumor (CCOT)
- Benign cystic tumor of odontogenic origin, aka “Gorlin cyst” or “Calcifying odontogenic cyst”
- Can have “ameloblastic” features: columnar or cuboidal basal cells with lumen lined by tissue resembling stellate reticulum
- Will have ghost cells or anucleate epithelial cells
- Should not have cytologic atypia, increased mitotic activity, necrosis
- Calcifying epithelial odontogenic tumor (CEOT)
