Mandible & maxilla
Other benign tumors / tumor-like conditions
Dentinogenic ghost cell tumor


Topic Completed: 5 October 2020

Minor changes: 12 October 2020

Copyright: 2020, PathologyOutlines.com, Inc.

PubMed Search: Dentinogenic ghost cell tumor

Frederic Carr Jewett III, D.O.
Brenda L. Nelson, D.D.S., M.S.
Page views in 2019: 128
Page views in 2020 to date: 324
Cite this page: Jewett FC, Nelson BL. Dentinogenic ghost cell tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/mandiblemaxilladentinogenicghost.html. Accessed October 20th, 2020.
Definition / general
  • Benign, locally aggressive odontogenic neoplasm of maxilla and mandible with a predominantly solid pattern of growth
Essential features
  • Neoplasm with predominantly solid growth of islands of odontogenic and ameloblastoma-like epithelium
  • Ghost cells composed of anucleate epithelial cells with pale cytoplasm
  • Focal stellate reticulum-like epithelium
  • Varying levels of calcified material to include products of odontogenesis and calcification of the ghost cell
  • Locally aggressive with high rates of recurrence
  • Histopathologic overlap with calcifying odontogenic cysts
Terminology
  • Epithelial odontogenic ghost cell tumor
  • Calcifying ghost cell odontogenic tumor
  • Previously classified along with calcifying odontogenic cyst (J Periodontol 1985;56:340)
ICD coding
  • ICD-10: D16.4 - benign neoplasm of bones of skull and face
Epidemiology
Sites
  • Intraosseous sites: posterior maxilla and mandible
  • Rarely reported as gingiva or alveolar mucosal tumor
Pathophysiology
Etiology
  • Unknown
Clinical features
  • Patients frequently present with asymptomatic swelling of the jaw
Diagnosis
  • Radiologic and histopathologic correlation required for diagnosis
Radiology description
Radiology images

Contributed by Brenda L. Nelson, D.D.S., M.S.
Unilocular lesion Unilocular lesion

Unilocular lesion


Contributed by Kelly Magliocca, D.D.S., M.P.H.
Axial CT

Axial CT

Prognostic factors
Case reports
Treatment
  • Rarity of this tumor limits study of the optimal form of treatment
  • Simple enucleation, curettage: recurrence rate of up to 73% after a follow up period of 1 - 20 years
  • Recurrence rates vary by surgical approach; recent recommendation includes wide local resection
Gross description
  • Predominantly solid tumor; limited macrocystic change
Gross images

Contributed by Kelly Magliocca, D.D.S., M.P.H.
Cross sections of mandible tumor

Cross sections of mandible tumor

Microscopic (histologic) description
  • Predominantly solid mass consisting of sheets of anastomosing cords and strands of odontogenic epithelium; microcystic development possible
  • Admixed ghost cells: anucleate epithelial cells with pale cytoplasm containing cytoplasmic clearings representing the location of a previously resorbed nucleus or organelles
  • Interspersed with islands of swirling cells with squamous differentiation
  • Ameloblastic-like areas with palisading of basaloid cells
  • Odontogenic epithelial cells demonstrate round uniform basophilic nuclei and pale eosinophilic to clear cytoplasm
  • Background stellate reticulum-like proliferation
  • Varying levels of dentinoid and cementum-like calcified collagenous matrix
  • Mitosis rare
  • Reference: J Oral Maxillofac Surg 2016;74:307
Microscopic (histologic) images

Contributed by Brenda L. Nelson, D.D.S., M.S.
DGCT with calcifications and squamous morules

Calcifications and squamous morules

Ghost cells

Ghost cells


Contributed by Anne McLean, D.M.D.
Microcyst formation Microcyst formation

Microcyst formation

Ghost cells and dentinoid Ghost cells and dentinoid

Ghost cells and dentinoid

Beta catenin

Beta catenin

Positive stains
  • Positive but nonspecific:
Negative stains
Sample pathology report
  • Posterior mandible, right, segmental mandibulectomy:
    • Dentinogenic ghost cell tumor (3.2 cm) (see comment)
    • Comment: Tumor confined to bone and measures 0.5 cm from anterior and posterior bone margins
  • Posterior mandible, right, excision / curettage:
    • Dentinogenic ghost cell tumor, in fragments
Differential diagnosis
  • Ameloblastoma:
    • Second most common odontogenic tumor; however, most clinically significant odontogenic tumor after odontoma
    • May have similar basaloid epithelial cells, reverse polarity, stellate reticulum but no ghost cells
    • Typically does not have calcifications
    • Conventional (nonunicystic), commonly solid and multilocular
  • Calcifying odontogenic cyst:
    • Similar histology, ameloblast-like epithelium, stellate reticulum-like proliferation, ghost cells, dentinoid and calcifications
    • Grossly, predominantly cystic; small satellite cysts, islands of epithelium or ghost cells may be seen in the fibrous capsule
    • Likely on a spectrum with dentinogenic ghost cell tumor
  • Craniopharyngioma:
    • Similar histology, ghost cells and islands of squamous cells present but originates in the sella turcica
  • Ghost cell odontogenic carcinoma:
    • Extremely rare with only isolated case reports
    • Demonstrates pleomorphism and malignant cytology with invasive features
Board review style question #1

Which of the following features favors a dentinogenic ghost cell tumor over a calcifying odontogenic cyst?

  1. Ameloblastic-like epithelium
  2. Anucleate pink ghost cells
  3. Islands of squamous differentiation
  4. Predominantly solid pattern of growth
  5. Stellate reticulum
Board review answer #1
D. Predominantly solid pattern of growth. Dentinogenic ghost cell tumor and calcifying odontogenic cyst have significant histopathologic overlap. Both demonstrate amelobastic-like epithelium, ghost cells, stellate reticulum, squamous differentiation and varying levels of dentin and calcification; however, dentinogenic ghost cell tumor is a predominantly solid neoplasm and calcifying odontogenic cyst is a single chamber, unilocular cyst.

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Reference: Dentinogenic ghost cell tumor
Board review style question #2
Which radiographic features distinguishes an ameloblastoma from a dentinogenic ghost cell tumor?

  1. Air fluid levels
  2. Calcifications
  3. Cystic spaces
  4. Invasive features
Board review answer #2
B. Calcifications. Ameloblastoma and dentinogenic ghost cell tumor have significant radiographic and histopathologic overlap; however, radiographically ameloblastoma generally does not have calcifications. Radiographically, ameloblastoma typically demonstrates a multilocular appearance. Ameloblastoma is the most common clinically significant odontogenic tumor and by far more common than dentinogenic ghost cell tumor.

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Reference: Dentinogenic ghost cell tumor

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