Dentinogenic ghost cell tumor (DGCT) is an uncommon locally invasive odontogenic tumor regarded by many as a variant of calcifying odontogenic cyst. The peripheral variant of this clinical rarity appears as a well-circumscribed mass mimicking a nonspecific gingival enlargement. Microscopic appearance of odontogenic epithelium admixed with focal areas of dentinoid formation and sheets of ghost cells giving the definitive diagnosis of dentinogenic ghost cell tumor imply that microscopic examination is compulsory for any gingival mass. Van Gieson histochemical stain further confirmed the nature of dentinoid-like material. A complete workup of a case of peripheral dentinogenic ghost cell tumor is presented in this paper and the current concept as well as the appraisal of literature is presented.
Calcifying odontogenic cyst (COC) is a unique jaw lesion, first recognized as a distinct entity by Gorlin et al. [
The term DGCT is commonly used, and the peripheral variant of this neoplastic entity is rare; only few reports with clinical, radiographic, and histologic documentation can be found in the English literature. A report of peripheral dentinogenic ghost cell tumor (PDGCT) and characterization of dentinoid material using Van Gieson special stain for the confirmation adds a new dimension to the diagnosis of DGCT.
A 40-year-old male patient reported to the dental clinics at Manipal College of Dental Sciences, Manipal University, with a complaint of missing teeth. Clinical examination disclosed a swelling measuring about 5 mm in diameter in the region of lower left premolars which was non tender, and the over lying mucosa was normal in color. Clinically, a provisional diagnosis of epulis was made (Figure
A nontender, persistent swelling in the region of lower left posterior region.
Histological examination revealed a solid well-circumscribed, encapsulated soft tissue mass surrounded by a dense fibrous connective tissue covered by stratified squamous epithelium (Figure
Solid wellcircumscribed encapsulated mass (a) showing ameloblastomatous islands of odontogenic epithelium (b) and ghost cells (c) with overlying epithelium (d) [Hematoxylin and Eosin,
Ameloblastomatous island of odontogenic epithelium with columnar basal cells (a) enclosing stellate reticulum like cells (b) [Hemataoxylin and Eosin,
Ghost cells (a) and surrounding dentinoid-like material (b) [Hemataoxylin and Eosin,
Irregular foci of dentine/osteo-dentine-like material (a) and calcifying ghost cells (b) and giant cells (c) [Hemataoxylin and Eosin,
Van Gieson stains showing ghost cells staining yellow (a) and surrounding dentinoid-like material staining pink (b) [Van Gieson stain,
DGCT is a distinct but a rare histological entity among odontogenic ghost cell lesions which have been recently classified into the simple cystic type or COC; cysts associated with odontogenic hamartomas or benign neoplasms; solid benign odontogenic neoplasm, which is same as COC but with dentinoid formation, the DGCT; malignant odontogenic neoplasm-ghost cell odontogenic carcinoma [
The usual presentation of the peripheral variant is a nodular swelling on the edentulous alveolar mucosa of denture wearers, a feature that implicates trauma/irritation. This clinical presentation could lead to the provisional diagnosis of epulis as was true in our case as well. Due to the dearth of documented cases, it is difficult to typify the exact location, age, and gender distribution of PDGCT. However based on the existing literature it appears to be more common in the canine-premolar region, with a predilection for the elderly age group [
Reported cases of peripheral dentinogenic ghost cell tumor.
Sl no. | Reference | No. of cases | Age in years/sex | Site | Radiographic features |
---|---|---|---|---|---|
1 | Abrams and Howell [ | 1 | 13/M | Extraosseous | No significant findings |
2 | Sauk [ | 1 | 67/F | Extraosseous | No radiographic changes |
3 | Praetorius et al. [ | 2 | 52/M | Maxillary left lateral incisor and canine region | Slight erosion of underlying bone |
41/F | Mandibular anterior region | Slight erosion of underlying bone | |||
4 | Hirshberg et al. [ | 1 | 42/M | Mandibular left premolar region (lingual gingiva) | No bone involvement |
5 | McClatchey et al. [ | 1 | 57/M | Mandibular anterior region | No bone involvement |
6 | Buchner et al. [ | 3 | 10/M | Mandibular central incisor | — |
53/F | Mandibular edentulous ridge | — | |||
92/F | Mandibular edentulous cuspid-premolar region | — | |||
7 | Günhan et al. [ | 1 | 71/F | Maxillary anterior region | Slight erosion of underlying bone |
8 | Raubenheimer et al. [ | 1 | 82/M | Mandibular right alveolar ridge (edentulous) | No bone involvement |
9 | Castro et al. [ | 1 | 83/F | Anterior ridge of edentulous mandible | Cup shaped resorption |
10 | Wong et al. [ | 1 | 71/M | Maxillary canine region | Slight erosion beneath the growth |
11 | Iezzi et al. [ | 1 | 43/M | Maxillary canine region | No bone involvement |
12 | Ledesma-Montes et al. [ | 1 | NS | NS | NS |
13 | Candido et al. [ | 1 | 45/M | Mandibular canine region | No bone involvement |
14 | Our case | 1 | 40/M | Mandibular premolar area | — |
NS: Not Specified.
Based on this histological diversity, the origin of this lesion has been attributed to cell rests of Serres or the surface epithelium [
Due to its biological behavior COC, and its variants are thought to resemble a benign odontogenic tumor rather than a cyst [
In our case as well, the presence of keratinizing ghost cells and mineralization was an outstanding feature. The lesion did not exhibit any cystic areas, and its features were consistent with those of DGCT. The calcifications seen as dentoid, frequently described in connection with masses of ghost cells, were a characteristic finding of this lesion. These calcified areas were thought to represent juxtaepithelial osteoid formation by granulation tissue due to induction by ghost cells [
Conservative but aggressive local resection has been the treatment of choice with no recurrences found in any patient. However patients with a DGCT should remain in long-term follow-up [
Dentinogenic ghost cell tumor
Calcifying odontogenic cyst
Peripheral dentinogenic ghost cell tumor.