|Year : 2013 | Volume
| Issue : 4 | Page : 436-439
Peripheral dentinogenic ghost cell tumor
Sushant S Kamat1, Gajendra S Diwakar1, Mujib BR Ahmed2, Prashant R Shetty3
1 Department of Oral Pathology and Microbiology, Yogita Dental College and Hospital, Khed, Maharashtra, India
2 Department of Oral Pathology and Microbiology, Bapuji Dental College, Davangere, Karnataka, India
3 Department of Periodontology, Yogita Dental College and Hospital, Khed, Maharashtra, India
|Date of Web Publication||17-Sep-2013|
Sushant S Kamat
Department of Oral Pathology and Microbiology, Yogita Dental College and Hospital, Khed, Maharashtra
Source of Support: None, Conflict of Interest: None
Dentinogenic ghost cell tumors (DGCT) are uncommon lesions mainly with rare peripheral types. This report presents a case of peripheral DGCT on the left side of the mandibular alveolar ridge of a heavy smoker, a 68-year-old man, with main presenting feature as a mild pain. Submandibular lymphadenopathy and radiological "saucerization" were evident. Differential diagnosis included fibroma, neurofibroma, peripheral ameloblastoma, peripheral odontogenic fibroma, and peripheral giant cell granuloma. Histologically, ameloblastoma-like epithelial elements were seen in association with grouped ghost cells. Proliferating polyhedral cells and stellate reticulum-like cells with various densities were spread over a wide range of the field. The lesion was curetted and after 2 years of follow up, it did not recur.
Keywords: Dentinogenic ghost cell tumors, peripheral lesion, pain, saucerization
|How to cite this article:|
Kamat SS, Diwakar GS, Ahmed MB, Shetty PR. Peripheral dentinogenic ghost cell tumor. Med J DY Patil Univ 2013;6:436-9
| Introduction|| |
Dentinogenic ghost cell tumor (DGCT) has been reported in the literature under different names starting with "calcifying ghost cell odontogenic tumor" by Fejerskov and Krogh in 1972.  In an excellent historical review, Juneja and George (2009)  have provided an account of the transition of tumor names in the published literature. The term DGCT was, however, first coined by Pretorius et al  in 1981 for the lesion that consisted of ameloblastoma-like strands and islands of odontogenic epithelium growing in an infiltrative manner in a mature connective tissue with varying amounts of ghost cells in the epithelium and dentinoid material in contact with the odontogenic epithelium. The term "Dentinogenic ghost cell tumor" is suggested for this lesion. It is also reported that this lesion predominantly occurs in the later part of life and can occur as an extra-osseous as well as an intra-osseous lesion. 
Many benign jaw tumors and cysts of odontogenic and non-odontogenic origin can exhibit a biologically aggressive course and can be difficult to diagnose.  The WHO defined DGCT as a locally invasive neoplasm characterized by ameloblastoma-like islands of epithelial cells in a mature connective tissue stroma. Aberrant keratinization may be found in the form of ghost cells in association with varying amounts of dysplastic dentin. 
DGCT is usually considered to be a rare condition. The aim of this paper is to report a case of DGCT and briefly review the scientific literature about it.
| Case Report|| |
A 68-year-old male reported to Bapuji Dental College and Hospital, Karnataka, India, complaining of a swelling in the left lower posterior edentulous ridge for a month. Although initially painless, the swelling developed pain since the past fortnight. The pain was continuous, dull aching, localized, and aggravated on chewing. The patient was a smoker for 40 years and smoked around 25 beedies (filterless tobacco rolled in natural leaves and smoked just like cigarettes) per day. The patient was wearing a set of complete denture at presentation. On examination, a solitary, oval, sessile, well-circumscribed swelling was present on mandibular posterior edentulous left side ridge measuring roughly 1.5 cm x 1.0 cm [Figure 1]. Mucosa over the swelling was normal. The swelling was tender to touch, firm in consistency, non-compressible, non-fluctuant, and was fixed. Left submandibular lymphadenopathy was present.
Radiographic evidence (intra-oral periapical and panoramic radiographs) suggested slight erosion of the bone on the alveolar ridge forming a small cup-shaped base, right below the swelling ("saucerization"), consistent with the size of the tumor. Normal bone pattern was noted around the depression [Figure 2] and [Figure 3].
|Figure 2: Panoramic radiograph showing "saucerization" on the left side mandibular alveolar ridge|
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|Figure 3: Intra-oral peri-apical radiograph showing erosion of bone at the site of the lesion|
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Based on the clinical features, a differential diagnosis list including fibroma, neurofibroma, peripheral ameloblastoma, peripheral odontogenic fibroma, and peripheral giant cell granuloma was developed.
Routine blood and biochemical investigations were conducted before treating the lesion. The lesion was surgically curetted and the tissue was sent for histopathological assessment.
Five whitish-grey soft tissue bits which were soft to firm in consistency were obtained during surgery. Microscopic examination of hematoxylin and eosin sections revealed hyperplastic squamous epithelium with the subepithelial tissue showing mild fibrosis [Figure 4]. [Figure 5] shows low power view of the neoplastic groups and ghost cells and [Figure 6] shows high power view of a group of ghost cells surrounded by neoplastic cells. [Figure 7] represents high power view showing dentinoid material along with Ghost cells (Van Gieson special stain). [Figure 8] shows tiny epithelial islands entrapped with peripherally lined basaloid ameloblast-like cells. Several large areas of eosinophilic cells with distinct outline characteristic of ghost cells were noted. These were present in singles, groups, and sheets. Ghost cells exhibited abundant granular eosinophilic cytoplasm and faint nuclear outline. Ameloblastoma like epithelial elements were seen in association with grouped ghost cells. The large eosinophilic areas seen throughout the section were suggestive of dentinoid material. The dentinoid material was presented as hyalinized eosinophilic material suggestive of immature or dysplastic dentin and was located closely near epithelial sheath. Based on the histological findings, dentinogenic ghost cell tumor (DGCT) was diagnosed.
|Figure 4: H and E stained section showing hyperplastic squamous epithelium|
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|Figure 6: High power view showing the group of ghost cells surrounded by neoplastic cells|
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|Figure 7: High power view showing dentinoid material along with Ghost cells (Van Gieson special stain)|
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|Figure 8: Histological section showing dentinoid material (a), ghost cells (b) and ameloblast like cells(c)|
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After surgical curettage, the wound healed completely and no recurrence was noted after 3 months and later after 2 years.
| Discussion|| |
The DGCT is defined as a solid neoplastic growth formed by groups and islands of epithelial cells showing an ameloblastoma-like basal cell layer that sometimes shows nuclear polarization. In the central part of the ameloblastomatous islands, tissue resembling the stellate reticulum of the enamel organ can also be found. Characteristically, it contains variable quantities of dentin-like material in the surrounding connective tissue and in close contact with the epithelial islands. Groups of ghost cells within the epithelial islands can be found. Two variants can be identified as aggressive central and non-aggressive peripherally located tumors.  The term was first used by Pretorius et al  in their article published in 1981. This was used while proposing a widely used classification in order to resolve the question on the cystic or neoplastic nature of the calcifying odontogenic cyst (COC).  Pretorius et al  proposed the term DGCT for the neoplastic counterpart of COC. Prior to this, DGCT was known by a variety of names such as epithelial odontogenic ghost cell tumor,  calcifying ghost cell odontogenic tumor, and dentinoameloblastoma. 
Ledesma-Montes et al  who described the two variants of DGCT reported that peripheral occurrence of DGCT is rare and only few reports with clinical radiographic documentations are available. This is in consensus with Bello et al  who reported that only 24 cases were stated in the literature prior to their case in 2011. The peripheral, extra-osseous lesion can be easily confused with other gingival lesions such as reactive or inflammatory lesions or other peripheral odontogenic tumors. The clinical appearance of all of these lesions is similar; therefore, the definitive diagnosis depends on histology and biopsy with a mandatory microscopic examination. 
Many benign jaw tumors and several cysts, of both odontogenic and non-odontogenic origin, can exhibit a biologically aggressive course and can be diagnostically difficult. Traditional histopathology continues to be the mainstay for the diagnosis of these lesions, as immunohistochemistry and molecular techniques have had, as yet, a little impact in this area.  However, immunohistochemical analysis demonstrated positivity for pan-cytokeratin, cytokeratin-14, and 2 neural markers. Denditric cells (Langerhans cells and melanocytes) are identified inside the tumor islands.  Geng et al have studied matrix metalloproteinases (MMP-9) and tissue-specific inhibitors of metalloproteinases (TIMP-1) in two ghost cell odontogenic lesions i.e., dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) and concluded that the expressions of MMP-9 and TIMP-1 are elevated in GCOC which may influence the behavior of GCOC.  Although MMP-9 protein is detected in stromal cells, it is significantly weaker in DGCT. Thus, it is suggestive that MMP-9 in stroma is associated with invasive ability of DGCT and closely associated lesions.  Another study has demonstrated that, although the expression of Ki-67 is noted in DGCT, it is lower than the most other odontogenic tumors and cysts. 
DGCTs are usually treated by surgical removal and recurr rarely. However, a recent case series reported that 10 of 14 DGCT's (71%) recurred after curettage of the initial tumors.  The main manifestation observed was progressive bone bulging, and radiographically the tumor was characterized by ill-defined radiolucency. Most of the recurrent tumors had also entrenched the surrounding tissues.  Multiple recurrence increases the risk of malignant transformation. Curettage alone might not be adequate for the management of DGCT. Maxillary cases with ill-defined borders, especially those showing an increased proliferative activity in biopsy or frozen section examination, should be treated more radically.  It has been suggested that intraosseous DGCT should be treated by resection with an adequate safety margin of at least 0.5 cm, as recommended for ameloblastoma. Patients with DGCT should continue follow-up for longer periods. 
| Acknowledgement|| |
The authors wish to thank Medilinkers Research Consultancy for rendering help while manuscript preparation.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]