Mandible & maxilla

Malignant odontogenic tumors

Ghost cell odontogenic carcinoma



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Last staff update: 29 October 2020

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PubMed Search: Ghost cell odontogenic carcinoma

Kelly Magliocca, D.D.S., M.P.H.
Anthony Martinez, M.D.
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Cite this page: Magliocca K, Martinez A. Ghost cell odontogenic carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mandiblemaxillaghostcellodontogeniccarcinoma.html. Accessed March 28th, 2024.
Definition / general
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
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
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)
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