Mandible-Maxilla
Malignant tumors
Odontogenic ghost cell tumor

Author: Kelly Magliocca, D.D.S., M.P.H., Anthony Martinez, M.D. (see Authors page)

Revised: 2 November 2016, last major update October 2016

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Odontogenic "ghost cell" [title] tumor

Cite this page: Odontogenic ghost cell tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/mandiblemaxillaodontogenicghost.html. Accessed December 6th, 2016.
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

Micro 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
Micro Images

Images hosted on other servers:

Ameloblastoma-like areas

Ghost cells

Cytologic atypia with increased mitotic rate

Ghost cells

Immunohistochemistry
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)