Comparative Analysis Between Dentinogenic Ghost Cell Tumor and Ghost Cell Odontogenic Carcinoma: A Systematic Review

Head Neck Pathol. 2021 Dec;15(4):1265-1283. doi: 10.1007/s12105-021-01347-z. Epub 2021 Jun 14.

Abstract

Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The aim of this study was to perform a comparative systematic review of the clinicopathological, genetic, therapeutic, and prognostic features of DGCT and GCOC. The electronic search was performed until December 2020 on seven electronic databases. Case reports, series, and research studies with enough histopathological criteria for diagnosis and all genomic studies were included. Both DGCT and GCOC showed a male prevalence (p = 0.043), with mandibular and maxillary predilections, respectively (p = 0.008). Peripheral DGCT (DGCTp) affected most elderly people (p < 0.001), and central DGCT (DGCTc) and GCOC occurred mainly in younger individuals. Unilateral enlargement of maxilla or mandible was the most common clinical sign associated with a radiolucent or mixed image. Ameloblastomatous epithelium was often present in both neoplasms. Basaloid and large cells with vesicular nuclei were also frequently seen in GCOC. β-catenin expression and mutations (CTNNB1 gene) were found in DGCT and GCOC. Conservative surgery was mostly used for DGCTp, while radical resection was chosen for DGCTc and GCOC. High recurrence rates were found in DGCTc and GCOC. Metastasis occurred in 16.7% of GCOC cases and the 5-year survival rate was 72.6%. DGCT and GCOC share numerous clinicopathological features and demand a careful histopathological evaluation, considering the overlap features with other odontogenic tumors and the possibility of malignant transformation of DGCT. A strict regular post-operative follow-up is mandatory due to high recurrence rates and metastatic capacity in GCOC.

Keywords: Dentinogenic ghost cell tumor; Ghost cell odontogenic carcinoma; Mandible; Maxilla; Odontogenic tumors.

Publication types

  • Comparative Study
  • Systematic Review

MeSH terms

  • Age Factors
  • DNA Copy Number Variations
  • Humans
  • Jaw Neoplasms / genetics
  • Jaw Neoplasms / pathology*
  • Keratins / metabolism
  • Ki-67 Antigen / metabolism
  • Mutation
  • Neoplasm Recurrence, Local
  • Odontogenic Tumors / genetics
  • Odontogenic Tumors / pathology*
  • Sex Factors
  • Tumor Suppressor Protein p53 / metabolism
  • beta Catenin / genetics

Substances

  • CTNNB1 protein, human
  • Ki-67 Antigen
  • MKI67 protein, human
  • Tumor Suppressor Protein p53
  • beta Catenin
  • Keratins