Dentinogenic Ghost Cell Tumor Research Articles

Central dentinogenic ghost cell tumor of the maxilla: a case report with new imaging findings and review of the literature

A dentinogenic ghost cell tumor (DGCT) is a rare benign odontogenic tumor that commonly shows characteristics of solid proliferation and has a relatively high risk of recurrence after surgical treatment. We herein report a case of a central DGCT that occurred in the maxilla and resulted in bone expansion. This study highlights new imaging findings (particularly magnetic resonance imaging) along with histopathological observations. In addition, we conducted a review of the existing literature on this rare tumor. A 37-year-old man developed swelling around the right cheek. A benign odontogenic tumor such as ameloblastoma was suspected based on the imaging examination findings (including bone expansion and the internal characteristics of the tumor) on panoramic imaging, computed tomography, and magnetic resonance imaging. The lesion was surgically excised from the right maxilla. Postoperative histopathological examination led to a definitive diagnosis of central DGCT. The tumor comprised epithelial neoplastic islands, resembling ameloblastoma, inside tight fibroconnective tissue; masses of ghost cells and formation of dentin were also observed. We had suspected that the minute high-density region around the molars on the imaging examinations represented alveolar bone change; however, it represented dentin formation. This led to difficulty diagnosing the lesion. Although DGCT may present characteristic findings on imaging examinations, its occurrence is infrequent, and in some cases, the findings may include the presence or absence of an impacted tooth without obvious calcification. The present case suggests that we should consider the possibility of an odontogenic tumor with calcification when high-density structures are observed inside the lesion.

The evolving molecular characterisation, histological criteria and nomenclature of adenoid ameloblastoma as a World Health Organisation tumour type.

Adenoid ameloblastoma (AA) was recently recognised as a separate tumour type in the most recent World Health Organisation (WHO) classification of head and neck tumours. This decision has been considered controversial by several groups, who have described AA as a subtype of ameloblastoma, a hybrid odontogenic tumour or to fall within the spectrum of other recognised odontogenic tumours, including dentinogenic ghost cell tumour and adenomatoid odontogenic tumour. Here we review the reasons for the WHO decision to classify AA as a separate tumour type. We also critique molecular and histological findings from recent reports published since the WHO classification. While acknowledging that the classification of tumours is constantly evolving, the balance of current evidence suggests that AA should remain a distinct tumour type, and not a subtype of ameloblastoma, pending further molecular characterisation.

A case of dentinogenic ghost cell tumor with odontoma

A case of dentinogenic ghost cell tumor with odontoma

Calcifying Odontogenic Cyst Demonstrates Recurrent WNT Pathway Mutations and So-Called Adenoid Ameloblastoma-Like Histology: Evidence Supporting Its Classification as a Neoplasm

Calcifying odontogenic cyst (COC), once called calcifying cystic odontogenic tumor, is classified under the category of odontogenic cysts. However, the proliferative capacity of the lesional epithelium and consistent nuclear β-catenin expression raise questions about its current classification. This study aimed to determine whether COC would be better classified as a neoplasm in the histologic and molecular context. Eleven odontogenic lesions diagnosed as COC or calcifying cystic odontogenic tumor were included in this study. The growth patterns of the lesional epithelium were analyzed histologically in all cases. β-catenin immunohistochemistry and molecular profiling using Sanger sequencing and whole-exome sequencing were performed in 10 cases. Of the 11 cases studied, histologic features reminiscent of so-called adenoid ameloblastoma were observed in 72.7% (8/11), and small islands of clear cells extended into the wall in 36.4% (4/11). Intraluminal and/or mural epithelial proliferation was found in 72.7% of the cases (8/11). Nuclear β-catenin expression was observed focally in all 10 cases studied, mainly highlighting epithelial cells forming morules and adjacent to dentinoid. CTNNB1 hotspot mutations were detected in 60.0% of the cases (6/10). All the remaining cases had frameshift mutations in tumor-suppressor genes involved in the WNT pathway, including APC and NEDD4L. Recurrent WNT pathway mutations leading to nuclear translocation of β-catenin and distinct epithelial growth patterns found in COC are the neoplastic features shared by its solid counterpart, dentinogenic ghost cell tumor, supporting its classification as a tumor rather than a cyst.

Intraosseous Dentinogenic Ghost Cell Tumor – A Case Report and Review of Literature

Dentinogenic ghost cell tumor is an extremely rare odontogenic neoplasm that was initially considered to be a solid variant of Calcifying Odontogenic Cyst with locally aggressive nature. The main characteristic features of DGCT are ameloblastoma like odontogenic epithelial proliferation, an aberrant keratinization in the form of ghost cells and dysplastic dentin. DGCT occur as two forms intraosseous and extra osseous of which more aggressive intraosseous variety requires careful monitoring and aggressive local resection to prevent recurrence. Here we are reporting a case of intraosseous Dentinogenic ghost cell tumor in a 65 years old male patient with a clinical presentation of bony hard swelling in the right mandibular posterior region. This lesion was diagnosed with clinical, radiological, and histopathological investigations which revealed it to be rare entity. Key words: Calcifying odontogenic cyst, Dentinogenic ghost cell tumor

Dentinogenic Ghost Cell Tumor: An Unusual Aggressive Oral Lesion

The purpose of this report was to document a case with a 1-week and 2-month follow up and contribute to the small body of existing literature to better understand this rare odontogenic tumor. There have been few aggressive and large dentinogenic ghost cell tumor case reports found in this literature.

Adenoid Ameloblastoma Shares Clinicopathologic, Immunohistochemical, and Molecular Features With Dentinogenic Ghost Cell Tumor: A Comparative Analysis.

The updated classification of odontogenic tumors by the World Health Organization (WHO) has included adenoid ameloblastoma (AA) as a distinct entity. However, distinguishing between AA and dentinogenic ghost cell tumor (DGCT) can still be challenging due to their significant morphologic similarities. In this study, we aimed to compare the clinicopathologic, immunohistochemical, and molecular characteristics of AA and DGCT to aid in their differentiation and to shed light on their pathologic mechanisms. Thirteen cases of AA and 14 cases of DGCT (15 samples) were analyzed, along with 11 cases of adenomatoid odontogenic tumor (AOT) and 18 cases of conventional ameloblastoma (AM) for comparative purposes. The study found that AA and DGCT shared a similar long-term prognosis. Immunohistochemically, all cytokeratins detected, except CK8/18, were not statistically significant in differentiating AA and DGCT, while there was a statistically significant difference in the immunophenotype of CK7 and CK10/13 between AA and AM. Nuclear β-catenin accumulation were detected in all cases of AA and DGCT, while AOTs and AMs exhibited cytoplasmic β-catenin. Molecularly, CTNNB1 hotspot mutations were found in only 1 case of AA (1/13), but not found in the other 3 types of tumors. BRAF p.V600E mutation was positive in 2/13 (15%) AA, 1/15 (7%) DGCT, and 2/11 (18%) AOT cases. In comparison, conventional AM was positive for BRAF p.V600E mutation in 94% (17/18) of cases, while KRAS mutations were detected in 63% (7/11) of AOT cases. The study suggests that the so-called AA is a rare benign tumor that exhibits clinical, immunohistochemical, and molecular features similar to DGCTs. Based on these findings, AA should not be categorized as a standalone entity solely based on the presence of whorls/morules and cribriform/duct-like structures. Further studies are needed to investigate the pathologic mechanisms of these tumors and to identify potential therapeutic targets.

Dentinogenic ghost cell tumour: A case report

A 23-year-old Malay female patient presented with a history of pain and swelling over right maxilla. Imaging showed a well-defined unilocular radiolucency with areas of radiopacity in the right maxilla. The lesion was initially thought to be a unicystic ameloblastoma. However, histopathology of the excised lesion proved otherwise with a final diagnosis given as dentinogenic ghost cell tumour. The clinical presentation of the case, subtypes of DGCT, similarities with ameloblastoma, and treatment modalities are discussed in this paper.

Dentinogenic ghost cell tumor revisited from a single institution in China with focus on adenoid ameloblastoma-like features

ObjectiveThe present study aims to identify adenoid ameloblastoma (AdAM) from previously diagnosed cases of dentinogenic ghost cell tumor (DGCT), and gain insight to the possible relationship between AdAM and DGCT. MethodsDGCT cases diagnosed between 2006 and 2022 were re-examined with focus on the AdAM-like features. ResultsA total of nine patients were included. Seven patients were males and two were females. The mean age was 38.0 ± 16.0 years. Five tumors occurred in the maxilla and four in the mandible, with a remarkable predilection for the posterior regions of both jaws. Microscopically, dentinoid material deposition was present in all cases. The ghost cells were absent in two cases. Rare ghost cells (<1%) were observed in three cases, and a higher proportion of ghost cells (5%-20%) were present in the remaining four cases. All cases showed prominent AdAM-like features, including duct-like structures, whorls/morules, and cribriform architecture. According to the diagnostic criteria proposed by the 2022 WHO classification, five cases without or with rare ghost cells were reclassified as AdAM. The other four cases including a higher proportion of ghost cells consisted of a mixture of DGCT and AdAM histopathologic patterns. ConclusionOur results confirmed that the AdAM-like features had been largely overlooked in the diagnosis of DGCT at our institution in the past. Whilst a subset can now be more accurately classified as AdAM, some tumors showed overlapping morphological features between AdAM and DGCT, suggesting that the two may represent a spectrum of the same entity.

Dentinogenic ghost cell tumor associated with odontoma: report of a rare case and review of literature.

Dentinogenic ghost cell tumor (DGCT) is an uncommon odontogenic tumor considered to be the solid, tumorous variant of calcifying odontogenic cyst (COC). DGCT is characterized by islands of ameloblastoma-like epithelial cells that resemble the enamel organ, the presence of ghost cells, and dentinoid material. Here, we report a rare case of dentinogenic ghost cell tumor associated with an odontoma in an adult patient and conduct a review of the literature. To the best of our knowledge, there have only been 4 case reports of DGCT associated with odontoma, all of which occurred in children and adults younger than 30 years old.

Modifications of Gallego staining and its dental application.

The objectives of the study are to employ modified Gallego staining in pathology and to specifically evaluate the hard tissue apposed to odontogenic epithelium in order to aid in the diagnosis. Lillie's modification of Gallego's stain was utilized as a reference to create a fresh batch of the stain. The archival and running cases of 2021-2022 was screened for odontogenic pathologies, yielding approximately 46 cases, among these four cases were selected which required evaluation of the hard tissue matrix apposed to odontogenic epithelium for its characterization. The modified Gallego staining was employed in the soft tissue sections of these cases under controlled environment. The staining results were evaluated. The stain was used in cases to stain dentinoid deposition as green color in hybrid ameloblastoma, archegonous cystic odontoma, dentinogenic ghost cell tumor and also in other cases like calcifying odontogenic cyst. Bone presented as green in color, cells as pink color and collagen as green-pink. This helped in the correct diagnosis of these cases facilitating the correct treatment modality. In the field of oral pathology, there are a multitude of lesions which are odontogenic with the diagnosis of few of them hinging on the characterization of hard tissue matrix found in close approximation to odontogenic epithelium implying an inductive capability to the odontogenic epithelium. This particular modified Gallego stain has been able to help in diagnosis of few such cases in our repertoire of cases.

Dentinogenic ghost cell tumor treated with a combination of marsupialization and radical resection: a case report and review of the literature

BackgroundDentinogenic ghost cell tumor is a rare benign tumor that accounts for less than 3% of all cases and consists of the stellate reticulum, which is made up of enamel epithelioid and basaloid cells. Although DGCT is a benign tumor, the local infiltration of the odontogenic epithelium or recurrences have been reported, and its detailed pathology and treatments remain unclear.Case presentationThis report describes the case of a 60-year-old Japanese male diagnosed with a maxillary dentinogenic ghost cell tumor. Images showed well-circumscribed, multilocular cystic lesions with a calcified substance in the interior. Marsupialization was performed along with biopsy to prevent the expansion of the lesion, and a partial maxillectomy was performed 2 years after the initial examination. Histopathological findings showed ameloblastomatous proliferation containing clusters of ghost cells and dentinoid materials, resulting in the diagnosis of dentinogenic ghost cell tumor. This article also reviews recently reported cases of dentinogenic ghost cell tumor.ConclusionIt is important to perform marsupialization, proper resection, and postoperative follow-up because of possible recurrence.

Dentinogenic Ghost Cell Tumour in Childhood Involving the Temporomandibular Joint: Considerations and Therapy.

Although the uncommon dentinogenic ghost cell tumour (DGCT) is a benign entity, it possesses the ability to cause widespread destruction of the jaws and to recur after bone-preserving therapy. Hence, clear margins should be achieved upon surgery, and reconstruction techniques must often be used to restore osseous defects. However, this can be challenging in cases with involvement of the temporomandibular joint (TMJ), and especially in children. We present a case of a DGCT in a 12-year-old boy with wide infiltration of the mandible and the TMJ. A two-staged reconstructive approach was performed. Upon primary surgery, tumour-free margins were obtained and mandibular anatomy was restored using an iliac crest graft and an alloplastic condyle implant for temporary TMJ reconstruction. In a second step 5months later, having received a customized TMJ prosthesis consisting of a fossa and a condyle component, the TMJ was completely replaced for definitive reconstruction. A customized TMJ prosthesis could be a solution for reconstruction of the TMJ in children. However, the further course with respect to growth disturbances must be evaluated upon short-term follow-ups and might require additional corrective interventions.

The molecular basis of odontogenic cysts and tumours.

The advances in molecular technologies have allowed a better understanding of the molecular basis of odontogenic cysts and tumours. PTCH1 mutations have been reported in a high proportion of odontogenic keratocyst. BRAF p.V600E are recurrent in ameloblastoma and KRAS p.G12V/R in adenomatoid odontogenic tumour, dysregulating the MAPK/ERK pathway. Notably, BRAF p.V600E is also detected in ameloblastic carcinoma, but at a lower frequency than in its benign counterpart ameloblastoma. Recently, adenoid ameloblastoma has been shown to be BRAF wild-type and to harbour CTNNB1 (β-catenin gene) mutations, further suggesting that it is not an ameloblastoma subtype. CTNNB1 mutations also occur in other ghost-cell-containing tumours, including calcifying odontogenic cysts, dentinogenic ghost cell tumours and odontogenic carcinoma with dentinoid, but the link between CTNNB1 mutations and ghost cell formation in these lesions remains unclear. Regarding mixed tumours, BRAF p.V600E has been reported in a subset of ameloblastic fibromas, ameloblastic-fibrodentinomas and fibro-odontomas, in addition to ameloblastic fibrosarcoma. Such mutation-positivity in a subset of samples can be helpful in differentiating some of these lesions from odontoma, which is BRAF-wild-type. Recently, FOS rearrangements have been reported in cementoblastoma, supporting its relationship with osteoblastoma. Collectively, the identification of recurrent mutations in these aforementioned lesions has helped to clarify their molecular basis and to better understand the interrelationships between some tumours, but none of these genetic abnormalities is diagnostic. Since the functional effect of pathogenic mutations is context and tissue-dependent, a clear role for the reported mutations in odontogenic cysts and tumours in their pathogenesis remains to be elucidated.

Adenoid Ameloblastoma Shares Clinical, Histologic, and Molecular Features With Dentinogenic Ghost Cell Tumor: The Histologic Spectrum of WNT Pathway–Altered Benign Odontogenic Tumors

An epithelial odontogenic tumor called adenoid ameloblastoma (AA) has recently been included in the new WHO classification. However, AA has considerable overlapping features with a preexisting entity, dentinogenic ghost cell tumor (DGCT). This study compared the clinical, histologic, and molecular characteristics of AA and DGCT. Eight cases of odontogenic tumors initially diagnosed as AA or DGCT were included in this study. Quantitative histologic analysis, β-catenin immunohistochemistry, and molecular profiling using next generation sequencing were performed. Additionally, accumulated clinical data of AA and DGCT were statistically analyzed. Nuclear β-catenin accumulation was detected in all cases in common, although the tumors studied histologically consisted of varying combinations of the AA-like phenotype, ghost cells, and dentinoid. However, CTNNB1 hotspot mutations were not found in any case. Instead, loss-of-function mutations in tumor suppressor genes involved in the WNT pathway, including the APC, SMURF1, and NEDD4L genes, were found regardless of histologic type. In addition, KRT13 mutations were detected in 2 cases with a high proportion of ghost cells. Finally, a literature analysis revealed clinical similarities between the previously reported cases of AA and DGCT. These findings suggest that from a clinical and molecular point of view, AA and DGCT represent a histologic spectrum of WNT pathway–altered benign odontogenic tumors rather than 2 distinct tumors. Moreover, previously unidentified keratin mutations may be associated with ghost cell formation found in specific types of odontogenic lesions.

Dentinogenic ghost cell tumor: A case report with review of literature

Dentinogenic ghost cell tumor is a rare odontogenic neoplasm first described by Praetorious et al in 1981. It is a solid variant of calcifying odontogenic cyst. This tumor shows no gender predilection and is seen more in mandibular anterior and molar region. Clinically, this tumor presents as two forms, Intraosseous &amp; Extraosseous. Ghost cells are pathognomonic in diagnosing this tumor. Very few cases of this rare dentinogenic ghost cell tumor have been reported in the literature till date.

Dentinogenic ghost cell tumor: A case report with review of literature

Dentinogenic ghost cell tumor: A case report with review of literature

Dentinogenic Ghost Cell Tumour, a rare case of an African young female patient: Review of literature and report of a case

Dentinogenic ghost cell tumour (DGCT) is a very rare aggressive benign odontogenic tumour with high recurrence rate and a potential to transform into malignancy. It can render facial disfigurement. The tumour is most frequently encountered in males than females with a ratio of 2:1. The peak incidence is in patients aged 40 - 60 years and the posterior mandible is slightly more affected than the maxilla. Segmental resection is a recommended surgical treatment and long-term postsurgical follow up is essential. This paper discusses a case of a 20-year-old African female patient who was diagnosed with a DGCT.

DENTINOGENIC GHOST CELL TUMOR: REPORT OF THREE NEW CASES

This case series contributes with 3 new cases of dentinogenic ghost cell tumor (DGCT). Case 1 is a 44-year-old male patient with an expansive swelling in the posterior mandible of 2 years duration. It appeared as a well-defined mixed radiolucent-radiopaque (RL-RO) lesion associated with an unerupted first molar. Case 2 is a 22-year-old female patient referred for evaluation of a slow-growing asymptomatic swelling of unknown duration in the left posterior maxilla; it was a 4-cm well-defined unilocular RL-RO lesion causing resorption of adjacent molars and encroachment of the maxillary sinus. Case 3 is a 37-year-old male patient with an expansive well-defined radiolucency in the lower premolar region exhibiting root resorption. Microscopically, prominent ameloblastomatous proliferation with areas of ghost cells and dentinoid material were observed. The patients are under long-term follow-up with no signs of recurrence to date. DGCT should be microscopically distinguished from adenoid ameloblastoma with dentinoid.

DENTINOGENIC GHOST CELL TUMOR WITH FOCAL FEATURES SUGGESTING GHOST CELL ODONTOGENIC CARCINOMA: A CHALLENGING DIAGNOSIS

Dentinogenic ghost cell tumor and ghost cell odontogenic carcinoma form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. A 24-year-old White woman complained of a painful enlargement and dental mobility in the maxilla for approximately 1 year. An intraoral examination showed a 3 × 3 cm firm exophytic mass in the left molar region. Tomographic sections showed a well-defined hypodense expansion with hyperdense foci inside invading the maxillary sinus. Microscopically, incisional sections showed a predominantly solid basaloid epithelium presenting atypical features, mitotic activity, ghost cells, positivity for p53, and high Ki-67 labeling, suggesting malignancy. Partial maxillectomy was performed. Surprisingly, the surgical specimen presented an important variation of the epithelial pattern and lacked any sign of malignancy, with abundant ghost cells and dentinoid, confirming the diagnosis of dentinogenic ghost cell tumor. A strict regular follow-up was done with no signs of recurrence or metastasis over 2 years.