Central granular cell odontogenic tumors (CGCOTs) are rare, benign, slowly growing odontogenic neoplasms. Due to their uncertain histogenesis, CGCOTs are still not included as a distinct entity in the WHO classification (2017) of odontogenic tumors. We report a case of CGCOT involving the right side of maxillary anterior region of a 39-year-old white female. Immunohistochemical staining showed that granular cells positively expressed CD68 and vimentin, and negatively expressed S-100 protein. Meanwhile, we searched PubMed, Google Scholar, and Scopus databases to summary the clinico-pathological features of 51 reported cases of CGCOT. The results showed that the granular cells of 28.6% cases were immunopositive for vimentin and CD68, and odontogenic epithelial cells were positive immunoreactivity for cytokeratin. These findings reinforced the mesenchymal origin of granular cells and the odontogenic nature of epithelium islands.
Humans ; Female ; Adult ; Vimentin ; Odontogenic Tumors/pathology* ; Epithelial Cells/pathology* ; Keratins

Odontogenic tumors are rare lesions with unknown etiopathogenesis. Most of them are benign, but local aggressiveness, infiltrative potential, and high recurrence rate characterize some entities. The MAP-kinase pathway activation can represent a primary critical event in odontogenic tumorigenesis. Especially, the BRAF V600E mutation has been involved in 80-90% of ameloblastic lesions, offering a biological rationale for developing new targeted therapies. The study aims to evaluate the BRAF V600E mutation in odontogenic lesions, comparing three different detection methods and focusing on the Sequenom MassARRAY System. 81 surgical samples of odontogenic lesions were subjected to immunohistochemical analysis, Sanger Sequencing, and Matrix-Assisted Laser Desorption/Ionization-Time of Flight mass spectrometry (Sequenom). The BRAF V600E mutation was revealed only in ameloblastoma samples. Moreover, the presence of BRAF V600E was significantly associated with the mandibular site (ρ = 0.627; P value <0.001) and the unicystic histotype (ρ = 0.299, P value <0.001). However, any significant difference of 10-years disease-free survival time was not revealed. Finally, Sequenom showed to be a 100% sensitive and 98.1% specific, suggesting its high-performance diagnostic accuracy. These results suggest the MAP-kinase pathway could contribute to ameloblastic tumorigenesis. Moreover, they could indicate the anatomical specificity of the driving mutations of mandibular ameloblastomas, providing a biological rational for developing new targeted therapies. Finally, the high diagnostic accuracy of Sequenom was confirmed.
Ameloblastoma/pathology* ; Carcinogenesis ; Humans ; Mitogen-Activated Protein Kinases/genetics* ; Mutation ; Odontogenic Tumors/pathology* ; Proto-Oncogene Proteins B-raf/metabolism* ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

Objective: To improve the understanding of histological variants of calcifying epithelial odontogenic tumor (CEOT). Methods: In this retrospective study, 11 cases of CEOT diagnosed from January 2008 to March 2022 were enrolled in the Department of Oral Pathology of Nanjing Stomatological Hospital, Medical School of Nanjing University. Among them, 10 were male and 1 was female. The patients were 19 to 58 years old [(43.0±11.9) years] and the course of disease was 2 weeks to 5 years. The clinicopathological characteristics were analyzed and the follow-up of patients ranged from 1 to 8 years, including 8 cases with follow-up data and 3 cases lost to follow-up. Furthermore, the related domestic and international literature was reviewed. Results: Eleven cases of CEOT included 6 cases of classic CEOT, 2 cases of clear cell CEOT, 2 cases of Langerhans cell-rich variant of CEOT and 1 case of non-calcified CEOT. In 6 cases of classic CEOT, the ratio of occurrence in mandible to maxilla was 2∶1, the ratio in central parts to peripheral parts was 5∶1, 2 cases were associated with unerupted teeth and 3 cases showed local aggressiveness. Histopathologically, classic CEOT showed eosinophilic epithelial cells, amyloid and calcification with Ki-67 value<5%. Among 4 cases with follow-up information, 1 case recurred after 1 year and 3 cases did not recur for 3 to 8 years. In 2 cases of clear cell CEOT, they both occurred in the periphery of mandible, pathologically showing a mix of lamellar balloon-like clear cells and typical CEOT, positive for CK5/6 and p63 in the area where the epithelial cells and clear cells were located, scattered positive for periodic acid-Schiff (PAS) in clear cells, which indicated the presence of glycogen. The maximum Ki-67 value was 5% in this type. One case lost to follow-up and the other case did not recur for 1 year follow-up after surgery. In 2 cases of Langerhans cell-rich variant of CEOT, they were cystic solid lesions and both occurred in the anterior maxilla. Langerhans cells were scattered in the epithelium and non-calcified amyloid glomeruli were present. Two cases were followed up for 1 year and 2 years without recurrence after surgery. One case of non-calcified CEOT that occurs within the jan showed invasion of surrounding soft tissues and the highest of Ki-67 value at 8% in all 11 cases without recurrence at 1 year follow-up. Conclusions: The histological pattern of classic CEOT is unique, and it is necessary to prompt the understanding of several histological variants derived from it.
Humans ; Male ; Female ; Young Adult ; Adult ; Middle Aged ; Retrospective Studies ; Ki-67 Antigen ; Odontogenic Tumors/surgery* ; Skin Neoplasms/pathology*

Keratocystic odontogenic tumors (KCOT) are benign, locally aggressive intraosseous tumors of odontogenic origin. KCOT have a higher stromal microvessel density (MVD) than dentigerous cysts (DC) and normal oral mucosa. To identify genes in the stroma of KCOT involved in tumor development and progression, RNA sequencing (RNA-Seq) was performed using samples from KCOT and primary stromal fibroblasts isolated from gingival tissues. Seven candidate genes that possess a function potentially related to KCOT progression were selected and their expression levels were confirmed by quantitative PCR, immunohistochemistry and enzyme-linked immunosorbent assay. Expression of lysyl oxidase-like 4 (LOXL4), the only candidate gene that encodes a secreted protein, was enhanced at both the mRNA and protein levels in KCOT stromal tissues and primary KCOT stromal fibroblasts compared to control tissues and primary fibroblasts (P<0.05). In vitro, high expression of LOXL4 could enhance proliferation and migration of the human umbilical vein endothelial cells (HUVECs). There was a significant, positive correlation between LOXL4 protein expression and MVD in stroma of KCOT and control tissues (r=0.882). These data suggest that abnormal expression of LOXL4 of KCOT may enhance angiogenesis in KCOT, which may help to promote the locally aggressive biological behavior of KCOT.
Adult ; Amino Acid Oxidoreductases ; genetics ; Cell Movement ; genetics ; Cell Proliferation ; Dentigerous Cyst ; enzymology ; pathology ; Disease Progression ; Female ; Fibroblasts ; pathology ; Gene Expression Regulation, Enzymologic ; genetics ; Gingiva ; pathology ; Human Umbilical Vein Endothelial Cells ; pathology ; Humans ; Male ; Microvessels ; pathology ; Neovascularization, Pathologic ; genetics ; Odontogenic Tumors ; blood supply ; enzymology ; pathology ; Sequence Analysis, RNA ; Stromal Cells ; pathology ; Young Adult

A 55 year old man complained of a painless mass at the left maxillofacial region. The mass had continuously grown over 10 years. Upon physical examination a cystic mass with size of 5 cm in length with bulging smooth surface was seen on the left maxillofacial region. Computed tomography (CT) scan showed a giant cyst with bone destruction and invasion in the left maxilla, maxillary sinus and nasal cavity. Needle aspiration of the mass yielded 80 milliliter of brown fluid. The excisional biopsy was made which revealed ghost cells and dysplastic dentin that were features of dentinogenic ghost cell tumor. Finally, a dentinogenic ghost cell was diagnosed.
Cysts ; pathology ; Diagnosis, Differential ; Humans ; Male ; Maxilla ; Maxillary Sinus ; Middle Aged ; Odontogenic Tumors ; pathology

Unusual presentation of localized gingival enlargement associated with a subjacent tumoural pathology is reported. The patient was a 55-year-old black male, whose chief complaint was a progressive gingival overgrowth for more than ten years, in the buccal area of the anterior left mandible. According to the clinical features and the radiological diagnosis of odontogenic keratocyst, a conservative surgery with enucleation and curettage was performed. Tissue submitted for histopathological analysis rendered the diagnosis of odontogenic myxoma. After 12-month of follow-up, no evidence of recurrence was found. Clinicians should be cautious when facing any gingival enlargement to avoid diagnostic pitfalls and to indicate the appropriate treatment.
Diagnosis, Differential ; Gingival Overgrowth ; etiology ; pathology ; Humans ; Male ; Mandibular Neoplasms ; complications ; pathology ; surgery ; Middle Aged ; Myxoma ; complications ; pathology ; surgery ; Odontogenic Tumors ; complications ; pathology ; surgery

We present an uncommon case (female patient aged 59 years) of the clear-cell variant of calcifying epithelial odontogenic tumor (CEOT) (also known as Pindborg tumor) in the mandible. The clinical characteristics and probable origins of the clear tumor cells of previously reported cases of clear-cell variant of intraosseous CEOT are also summarized and discussed.
Biopsy ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Keratins ; analysis ; Mandibular Neoplasms ; diagnosis ; pathology ; Middle Aged ; Odontogenic Tumors ; diagnosis ; pathology ; Radiography, Panoramic ; Skin Neoplasms ; diagnosis ; pathology

Humans ; Odontogenic Tumors ; diagnosis ; pathology

OBJECTIVETo study the expression of HOXC13 mRNA in ameloblastoma (AB), and to investigate its biological significance.

METHODSHOXC13 mRNA was examined in 47 cases of AB (primary AB 29 cases, recurrent AB 14 cases, malignant AB 4 cases). 2 cases of fibrous dysplasia of bone, 10 cases of keratocystic odontogenic tumor (KCOT) and 7 cases of normal oral mucosa were selected as control.

RESULTSThe positive rates of HOXC13 mRNA in AB, KCOT, and normal oral mucosa were 97.9% (46/47), 7/10 and 3/7, respectively. There was a significant difference among AB, OKC and normal mucosa (chi(2) = 21.665, P = 0.001). For HOXC13, the keratinizing cells and granulizing cells in AB were negative, some fibroblasts were positive, 2 cases of fibrous dysplasia of bone were positive.

CONCLUSIONSHOXC13 was highly expressed in AB. The expression of HOXC13 mRNA in AB had heterogeneity, which could improve the epithelial proliferation, and its loss may lead to the cornification and degeneration of epithelial cells.

Adolescent ; Adult ; Ameloblastoma ; genetics ; metabolism ; pathology ; Female ; Gene Expression ; Genes, Homeobox ; Homeodomain Proteins ; metabolism ; Humans ; Male ; Middle Aged ; Mouth Mucosa ; metabolism ; Odontogenic Tumors ; genetics ; metabolism ; RNA, Messenger ; metabolism ; Young Adult

OBJECTIVEThe expression of Ki-67 antigen of ameloblastoma was examined in order to investigate the different proliferation activity of histological variants of ameloblastoma and its clinical significance.

METHODS70 cases of different histological specimen of ameloblastoma were analyzed by immunohistochemical method using Ki-67 antibody. The Label Index was calculated in percentage of Ki-67 positive cells after examined with an image analysis system.

RESULTSThe results showed that the Labeled Index in malignant ameloblastoma was the highest 14.72% +/- 2.87%. The Labeled Index in solid ameloblastoma was in the middle, among which the follicular 4.42% +/- 1.05% was higher than the plexiform 3.64% +/- 1.23%. The Labeled Index in mono-cystic ameloblastoma was the lowest 2.21% +/- 1.09%.

CONCLUSIONThe results demonstrated that the proliferation activity varied according to the histological pattern of ameloblastoma. The prognosis with different proliferation activity was also varied accordingly.

Ameloblastoma ; metabolism ; pathology ; Humans ; Image Processing, Computer-Assisted ; Jaw Neoplasms ; metabolism ; pathology ; Ki-67 Antigen ; biosynthesis ; Neoplasm Recurrence, Local ; Odontogenic Tumors ; metabolism ; pathology