Dentinogenic ghost cell tumor invading the maxillary sinus: A rare case report with a review of the literature

Dentinogenic Ghost Cell Tumour (DGCT) is a rare, locally invasive, solid neoplastic variant of the Calcifying Odontogenic Cyst (COC).

Ghost Cell Odontogenic Carcinoma (GCOC) is a rare malignant neoplasm of odontogenic epithelium, characterized by aggressive behavior and distinctive histological features, including ghost cells and infiltrative growth. This study reports the case of an 80-year-old male patient who presented with right facial swelling and nasal obstruction. Computed tomography revealed an expansive, heterogeneous mass with cystic areas and coarse calcifications, causing bone destruction in the right maxillary region. Histopathological analysis showed odontogenic epithelial tissue, ghost cells, inflammatory infiltrate, and dentin deposits. Immunohistochemical studies confirmed an epithelioid neoplasm with atypia, mitotic figures (6/10 HPF), and diffuse positivity for cytokeratin, supporting the diagnosis of GCOC. Radiotherapy was initiated but discontinued after six sessions due to clinical deterioration, culminating in the patient’s death. This case underscores the diagnostic and therapeutic challenges of GCOC, reinforcing its aggressive nature, the role of immunohistochemistry in differentiating it from dentinogenic ghost cell tumor, and the importance of early diagnosis for improved prognosis. Keywords : Ghost cell odontogenic carcinoma, Ghost Cells, Odontogenic tumors.

The present study aimed to contribute to the biological characterization of odontogenic ghost cell lesions (OGCL). Sixty-nine OGCL consisting of 60 calcifying odontogenic cysts (COC) and nine dentinogenic ghost cell tumors (DGCT) were collected from a single center over a period of 63 years. The clinical, radiographic, and histopathological features were re-evaluated. Histochemical and immunohistochemical studies were performed in 37 COC and three DGCT. Molecular studies were performed in 17 COC to identify possible CTNNB1 gene mutations. COC was more frequent in women, in the second decade of life, involved the anterior region of both jaws, and manifested mainly as a unilocular radiolucency. DGCT was more frequent in women, in the ninth decade of life, involved the anterior region of mandible, and manifested as irregular mixed lesions. The ameloblastic/ameloblastomatous epithelium and ghost cells stained positive for AE1-AE3 (40/40), amelogenin (40/40), β-catenin (40/40), E-cadherin (40/40), S100 (22/40), and vimentin (15/40) in both the studied entities. TOM-20 (40/40), BCL-2 (40/40), BRAF V600E (4/40), and p63 (1/40) were only positive in the ameloblastic/ameloblastomatous epithelium, and lysozyme (40/40) and CD68 (35/40) were positive in the ghost cells. No single-nucleotide variants were detected in CTNNB1, except for a change at codon 38. The protein immuno-expression observed in ghost cells confirms an epithelial origin and suggests that these cells result from a degenerative process involving an increase in lysosomes and accumulation of proteins. Immuno-expression of β-catenin in the absence of CTNNB1 mutations suggests the presence of mutations in other genes associated with the WNT/β-catenin pathway.

Rationale:Dentinogenic ghost cell tumors (DGCTs) are rare odontogenic neoplasms constituting the solid variant of calcifying odontogenic cysts. Predominantly observed in adults, DGCTs are exceptionally rare in pediatric patients. This case report aims to contribute to the limited literature by presenting the clinical, radiographic, and histopathological findings of a pediatric patient with a DGCT and emphasizing the importance of early diagnosis.Patient concerns:A 14-year-old male presented with facial asymmetry in the left mandible.Diagnoses:Diagnostic imaging revealed a multilocular radiolucent lesion with impacted teeth, cortical expansion, and root resorption. Histopathological evaluation confirmed the DGCT diagnosis.Interventions:The lesion was treated conservatively with enucleation and curettage, followed by a 3-year follow-up.Outcomes:Regular follow-ups demonstrated progressive bone regeneration with no evidence of recurrence. No postoperative complications were observed.Lessons:Because of their high recurrence rate and potential for transformation into dentinogenic ghost cell carcinoma, early diagnosis and long-term follow-up are crucial for effective management in DGCT cases.

Dentinogenic ghost cell tumor: A rare case report with insight into molecular profiling.

A rare case of dentinogenic ghost cell tumor: a case report

Although WNT pathway-altered odontogenic tumors (WNT-OTs) are a genetically distinct group of odontogenic tumors (OTs), they may histologically resemble other OTs. To investigate the utility of immunohistochemical markers in the diagnosis of WNT-OTs. Immunohistochemistry for SATB2 (SATB homeobox 2), CDX2 (caudal type homeobox 2), CD10, and β-catenin was performed in 37 OTs consisting of 19 WNT-OTs (10 calcifying odontogenic cysts, 7 dentinogenic ghost cell tumors [DGCTs]/adenoid ameloblastomas [AAs], 2 ghost cell odontogenic carcinomas) and 18 non-WNT-OTs (7 unicystic ameloblastomas, 7 solid/multicystic ameloblastomas, 4 ameloblastic carcinomas). All WNT-OTs were positive for SATB2, whereas all but 1 of the non-WNT-OTs were SATB2-negative, resulting in a sensitivity of 100% (19 of 19) and a specificity of 94.4% (17 of 18) for WNT-OTs. About two-thirds of WNT-OTs were positive for CDX2, whereas all non-WNT-OTs were CDX2-negative, resulting in a lower sensitivity of 63.2% (12 of 19) and a specificity of 100% (18 of 18). Although both CD10 and β-catenin showed 100% sensitivity (19 of 19), their specificities were low at 16.7% (3 of 18) and 44.4% (8 of 18), respectively; nevertheless, CD10 highlighted morular structures in most WNT-OTs. No differences in immunohistochemical profiles were observed between DGCT and AA. This study presents novel findings on the immunoreactivity of intestinal markers SATB2 and CDX2 in WNT-OTs. SATB2 is a sensitive and specific marker for the diagnosis of WNT-OTs, and CDX2 and CD10 may serve as additional markers for WNT-OTs. Additionally, the immunohistochemical similarities between DGCT and AA further support the view that these 2 tumors represent the histologic spectrum of the same entity.

Successful treatment of mandibular central dentinogenic ghost cell tumor with en bloc resection and dental rehabilitation: A case report.

Dentinogenic ghost cell tumor (DGCT) is an uncommon odontogenic neoplasm that accounts for less than 0.5% of all tumors with odontogenic origin. Its rarity, along with identical radiological and clinical features as other odontogenic lesions, often makes the diagnosis challenging. The current case report discusses the clinical, radiological, and histopathological features of DGCT in a 71‐year‐old female patient presenting with facial swelling and pain in the maxillary anterior region. Clinical examination revealed a hard, tender, localized swelling from the infraorbital to the zygomatic regions. Intraoral exam revealed a sessile swelling buccopalatally extending and involving the labial vestibule and hard palate. Imaging showed a unilocular, expansile lytic lesion with hyperdense contents and a thinned cortex, suggestive of odontogenic pathology. Histopathology confirmed the diagnosis of DGCT on the basis of ghost cells with calcification, ameloblastoma‐like epithelial islands, and dentinoid material in connective tissue. Treatment was done by wide local excision with a maxillary obturator prosthesis. Six‐month postoperative follow‐up showed no recurrence or complication. This case highlights the importance of combining clinical, radiological, and histopathological examination to diagnose rare odontogenic tumors like DGCT. Despite being benign, the tumor′s aggressive potential demands radical resection and extended follow‐up for monitoring recurrence. The case also highlights the importance of raising awareness among clinicians to achieve optimum diagnostic yield and patient outcomes.

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.

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

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

Unravelling the mystery of the central dentinogenic ghost cell tumor- a rare case report and recurrent insights

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

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.

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.

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