Sclerosing Odontogenic Carcinoma With a Prominent Clear Cell Component Mimicking Odontogenic Clear Cell Carcinoma: An Extremely Rare Case With a Fatal Clinical Outcome.

Sclerosing odontogenic carcinoma (SOC) is an exceedingly rare odontogenic carcinoma known for its locally aggressive yet indolent behavior. There have been no reports of metastasis to distant organs, except a single case involving lymph node metastasis. This report details the case of a 49-year-old female who presented with a well-demarcated radiolucent lesion in the mandible, accompanied by root resorption and tooth displacement. Microscopically, the lesion exhibited a distinctive composition, with two distinct components: cords of epithelium embedded within an abundant collagenous stroma and solid nests of clear polygonal cells surrounded by hyalinized stroma. Notably, the tumor exhibited direct invasion into the submental muscles, accompanied by perineural and vascular invasion, as well as cortical bone loss. Additionally, the clear cells contained diastase-sensitive periodic acid-Schiff-positive granules. Immunohistochemically, the tumor cells displayed positivity for cytokeratin 19 and p63 while testing negative for myoepithelial markers. The Ki-67 index was measured at 23%. Importantly, neitherEWSR1 nor MAML2 rearrangements were detected through fluorescence in situ hybridization (FISH) analysis. Over several years, this patient experienced three instances of local recurrence; notably, four years after the initial surgery, fludeoxyglucose F18-positron emission tomography (18FDG-PET)/CT scans confirmed the presence of pulmonary metastasis. This case presents an unusual histological variation of SOC, marked by vascular invasion, and is notably the first documented case of a fatal outcome in this context.

Comprehensive cornified envelope protein profile of odontogenic keratocysts clarifies the characteristics of non-keratinized oral epithelium.

BACKGROUND: Odontogenic keratocysts constitute 10%-20% of odontogenic cysts and exhibit a distinctive corrugated parakeratinized lining epithelium. Considering that cornified envelope formation is an important phenomenon during keratinocyte differentiation, this study aimed to clarify the characteristics of cornified envelope formation in odontogenic keratocysts. METHODS: We investigated the cellular distribution of cornified envelope-related proteins (transglutaminases and their substrates), as well as the upstream regulatory protein c-Fos, by immunohistochemical analysis of the lining epithelium of 20 odontogenic keratocysts. We examined the corresponding mRNA levels by quantitative polymerase chain reaction. Ten dentigerous cysts served as control non-keratinized cysts. RESULTS: The distributions of transglutaminase and their substrates except loricrin and small protein-rich protein 1a significantly differed between odontogenic keratocysts and dentigerous cysts. There was no significant difference in c-Fos expression between odontogenic keratocysts and dentigerous cysts. The mRNA levels of transglutaminases and their substrates were significantly higher in odontogenic keratocysts than in dentigerous cysts. However, c-Fos mRNA levels did not significantly differ between groups. CONCLUSION: Surprisingly, the overall appearance of cornified envelope-related proteins of odontogenic keratocysts was consistent with the characteristics of non-keratinized oral mucosa identified in previous studies. These findings indicate that the contribution of cornified envelope-related molecules in odontogenic keratocysts is similar to that in non-keratinized oral epithelium, rather than keratinized oral epithelium, suggesting that odontogenic keratocysts are not genuine keratinized cysts. The upregulation of cornified envelope-related genes in odontogenic epithelium could be an important pathognomonic event during odontogenic keratocyst development.

A Case of Oral Glomeruloid Hemangioma Without Systemic Conditions.

Glomeruloid hemangioma is a rare variant of hemangioma that is accompanied by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities (POEMS) syndrome and, rarely, by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome. This report presents the case of a 78-year-old male who presented with a hemorrhagic nodule on the tongue without any other systemic diseases. Microscopically, the lesion was a lobular proliferation extending from the lamina propria to muscular tissue. Some intravascular nodules with irregular vascular lumens closely resembled renal glomeruli. Each nodule consisted of plump endothelial and stromal cells that partially showed vacuolated cytoplasm containing eosinophilic and periodic acid-Schiff (PAS)-positive globules. Immunohistochemically, IgG-positive deposition was noted within CD31-positive cells. Many plump stromal cells were positive for CD31, CD146, nestin, and type IV collagen but not α-smooth muscle actin (αSMA). These results reflect the proliferation of immature endothelial cells and pericytes, which might characterize this unique lesion. Microscopically, this case revealed glomeruloid hemangioma without systemic conditions related to POEMS, and composed of an intravascular proliferation of immature endothelial and pericytic stromal cells.

Mammaglobin protein localization and gene expression in the salivary glands.

PURPOSE: This study aims to delve deeper into the hypothesis that normal salivary gland tissue expresses both protein and mRNA of mammaglobin (MGB). METHODS: Formalin-fixed paraffin-embedded samples of submandibular (10), parotid (5), palatal (5) and labial glands (30) salivary glands were immunohistochemically investigated. The labial samples were used to examine the MGB positive ratio (MGB-PR), and localize MGB by double immunofluorescence staining and quantitative mRNA gene expression. Mann-Whitney U and Kruskal Wallis rank-sum test for group comparison, and Spearman's rank correlation coefficient for correlation analysis were used. RESULTS: The distribution of MGB-positive cells was variable throughout samples with significantly higher MGB-PR of acini than ducts (P = 0.00376), and there was no difference when compared based on age (P = 0.0646) and gender (P = 0.245). Besides acinar cells, a number of myoepithelial cells and ductal cells also demonstrated strong MGB reactivity with varying MGB mRNA expression levels in 6 of the 7 samples (with MGB-PR > 20%) tested. CONCLUSION: This novel study shows that unlike aberrant protein expression in some carcinomas, MGB expression in salivary gland neoplasms represents the nature of original cells, giving a better insight into the neoplasms expressing MGB.