Ulcerative and Inflammatory Lesions of the Oral Mucosa.

Ulcerated and inflammatory lesions of the oral mucosa are not rare. A detailed patient medical and social history including habits and abuses, as well as the duration, location, focality and presence, or lack of local and/or systemic symptoms is critical in establishing a proper diagnosis. This article discusses the clinical presentation, management, and histopathologic characteristics of a variety of ulcerative and inflammatory lesions seen in the oral cavity.

PIK3CA and p53 Mutations Promote 4NQO-Initated Head and Neck Tumor Progression and Metastasis in Mice.

The PI3K signaling pathway is frequently mutated in head and neck squamous cell carcinoma (HNSCC), often via gain-of-function (GOF) mutations in the PIK3CA gene. Here, we present novel genetically engineered mouse models (GEMM) carrying a GOF allele Loxp-STOP-Loxp(LSL)-PIK3CAH1047R (E20) alone or in combination with heterozygous LSL-p53+/R172H (p53) mutation with tissue-specific expression to interrogate the role of oncogenic PIK3CA in transformation of upper aerodigestive track epithelium. We demonstrated that the GOF PIK3CA mutation promoted progression of 4-nitroquinoline 1-oxide-induced oral squamous cell carcinoma (OSCC) in both E20 single mutant and E20/p53 double mutant mice, with frequent distal metastasis detected only in E20/p53 GEMM. Similar to in human OSCC, loss of p16 was associated with progression of OSCC in these mice. RNA-seq analyses revealed that among the common genes differentially expressed in primary OSCC cell lines derived from E20, p53, and E20/p53 GEMMs compared with those from the wild-type mice, genes associated with proliferation and cell cycle were predominantly represented, which is consistent with the progressive loss of p16 detected in these GEMMs. Importantly, all of these OSCC primary cell lines exhibited enhanced sensitivity to BYL719 and cisplatin combination treatment in comparison with cisplatin alone in vitro and in vivo, regardless of p53 and/or p16 status. Given the prevalence of mutations in p53 and the PI3K pathways in HNSCC in conjunction with loss of p16 genetically or epigenetically, this universal increased sensitivity to cisplatin and BYL719 combination therapy in cancer cells with PIK3CA mutation represents an opportunity to a subset of patients with HNSCC. IMPLICATIONS: Our results suggest that combination therapy of cisplatin and PI3K inhibitor may be worthy of consideration in patients with HNSCC with PIK3CA mutation.

Oral Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature.

Pseduomyogenic hemangioendothelioma (PMH) is a vascular neoplasm of intermediate biological potential first described by Hornick and Fletcher (Am J Surg Pathol 35:190-201, 2011). Despite its initial categorization as a malignant entity, PMH often demonstrates an indolent behavior profile, and thus was classified as a rarely metastasizing endothelial neoplasm in the 2013 WHO Classification of Tumors of Soft Tissue and Bone. It is a tumor primarily of skin and soft tissue, with most reported cases involving the trunk or extremities. To date, only one case of PMH involving the oral cavity has been reported. Herein, we present a case of PMH involving the mandibular gingiva and vestibule of a 33-year-old female and discuss the salient features of this entity.

Stafne Bone Defect Involving the Ascending Ramus.

The Stafne bone defect (SBD) classically presents as a well-defined, radiolucent lesion in the molar-mandibular angle region beneath the level of the mandibular canal. Due in large part to this prototypical appearance, most cases of SBDs are radiographically diagnostic without the need for tissue biopsy. A diagnostic challenge may arise, however, when the SBD occurs in other locations in the mandible. The authors report a case of a 57-year-old male who presented with a SBD of the left ascending ramus. The lesion was found incidentally on a panoramic radiograph. Additional imaging studies confirmed a lingual concavity of the mandibular bone consistent with a SBD, and a retrospective study of the patient's records revealed the presence of the lesion 5 years prior without any radiographic alterations. To date, only 10 documented cases of an SBD involving the ascending ramus have been reported. The rarity of such a presentation may obscure the diagnosis and lead to unwarranted surgical procedures. It is important to recognize atypically located SBDs, because unlike other radiolucent lesions, imaging studies are diagnostic. With proper diagnosis, additional surgical procedures and any associated morbidities can be avoided.

Which Clinicopathologic Factors Affect the Prognosis of Gingival Squamous Cell Carcinoma: A Population Analysis of 4,345 Cases.

PURPOSE: The purpose of this study was to present the characteristics and survival outcomes of primary gingival squamous cell carcinoma (SCC). MATERIALS AND METHODS: We performed a retrospective cohort study using data from the Surveillance, Epidemiology, and End Results (SEER) tumor registry. Patients who received a diagnosis of primary gingival SCC were included in the analyses. The predictor variables were chosen from baseline demographic and tumor characteristics. The outcome of interest was survival, and Kaplan-Meier analyses were used to estimate rates of overall survival (OS) and disease-specific survival (DSS). Cox proportional hazards regression models were used for multivariate analyses. RESULTS: In total, 4,345 patients (mean age, 70.2 years) with primary gingival SCCs were identified. There was a significantly positive association between T category and both nodal and distant metastases. The 2-, 5-, and 10-year OS rates were 63.1%, 46.5%, and 28.1%, respectively, whereas the DSS rates were 78.2%, 70.7%, and 62.2%, respectively. Multivariate Cox proportional hazards regression showed that the independent predictors of overall and disease-specific death were older age, intermediate and high histologic grade, tumor size, nodal disease, and the absence of surgery. T4 classification was significantly associated with only DSS. Race, gingival location (maxillary vs mandibular), verrucous histology, and the presence of distant metastases did not significantly affect survival when all other predictors were controlled for. CONCLUSIONS: Older age at diagnosis, higher grade, increased tumor size, nodal disease, and the absence of surgery were each individually associated with lower OS and DSS. Because of low nodal disease rates, both T1N0M0 lesions and verrucous subtypes may be candidates for neck observation.

Characteristics and Prognosis of Primary Head and Neck Angiosarcomas: A Surveillance, Epidemiology, and End Results Program (SEER) Analysis of 1250 Cases.

Head and neck angiosarcomas (HN-AS) are rare malignancies with a poor prognosis relative to other soft tissue sarcomas. To date, the HN-AS literature has been limited to short reports and single-institution experiences. This study evaluated patients registered with the Surveillance, Epidemiology, and End Results (SEER) program who had been diagnosed with a primary HN-AS. Predictors were drawn from demographic and baseline tumor characteristics. Outcomes were survival months and cause of death. Kaplan-Meier analyses were used to estimate overall (OS) and disease-specific survival (DSS) rates. Cox proportional hazards regression models were used for multivariate analyses. A total of 1250 patients (mean age 73.3 years) were identified, and nearly all lesions (93.5%) were cutaneous. Two- and 5-year OS rates were 47.3% (95% CI 44.3-50.3) and 26.5% (95% CI 23.7-29.3), while 2- and 5-year DSS rates were 66.6% (95% CI 63.6-69.6) and 48.3% (95% CI 44.5-52.1). In the univariate analyses, age, race, tumor grade, tumor size, AJCC stage, SEER historic stage, and surgery were significant predictors of both OS and DSS. Multivariate regression revealed that independent predictors of poor OS and DSS were older age [OS: HR 1.04 (95% CI 1.02-1.05), p < 0.01; DSS: HR 1.03 (95% CI 1.01-1.05), p < 0.01], increased tumor size [OS: HR 1.01 (95% CI 1.01-1.01), p < 0.01; DSS: HR 1.01 (95% CI 1.01-1.02), p < 0.01], and distant disease [OS: HR 2.97 (95% CI 1.65-5.34), p < 0.01; DSS: HR 4.99 (95% CI 2.50-9.98), p < 0.01]. Age, tumor size, and disease extent were determinants of HN-AS survival. When all other factors were controlled, lower histologic grade and surgery did not improve the risk of death.

Ameloblastic Fibro-Odontoma in an Adolescent: A Case Report and Review of Literature.

Ameloblastic fibro-odontoma (AFO) is a rare benign odontogenic tumor with the histologic features of ameloblastic fibroma (AF) but also contains enamel and dentin. It is most commonly observed in the pediatric population. Distinction between AFO and AF becomes important as ameloblastic fibromas are associated with higher recurrence rates of up to 18%, and 35% of these recurrent lesions can undergo malignant transformation to ameloblastic fibrosarcoma. Hence, for amelobastic fibroma, conservative curettage is recommended for the initial lesion and marginal resection is considered for recurrent cases. In contrast, AFO can be treated with simple curettage and the recurrence rate is approximately seven percent. Malignant transformation of AFO is exceedingly rare. Therefore, the treatment and prognosis differs for these two histologically similar neoplasms. We present a case of a 17-year-old boy who was initially diagnosed with ameloblastic fibroma upon biopsy, with subsequent curettage specimen showing AFO, which carries a better prognosis.

Pyogenic Granuloma in the Tongue in a Five Year Old: A Case Report.

Pyogenic granuloma, also called lobular capillary hemangioma, is a benign vascular lesion of the skin and mucous membranes. While the majority of pyogenic granulomas in the oral cavity involve the gingiva, they also infrequently present at other sites including lips, oral mucosa, palate, and tongue. We report a case of a pyogenic granuloma of the tongue in a five-year old female patient.

Renal cell carcinoma metastatic to the maxillary gingiva: A case report and review of the literature.

Tumor metastasis to the oral cavity is rare and is usually an indication of late-stage disease and poor prognosis. While, there are reports of renal cell carcinoma (RCC) metastatic to oral cavity, vast majority of them are to the jaw. Herein, we present a case of a 78-year-old woman with RCC metastasis limited to the oral soft tissue without any bone involvement. As the lesion solely involved maxillary gingiva, it clinically mimicked that of a pyogenic granuloma, which is a reactive, nonneoplastic condition. This case was further complicated as the patient was unaware of primary cancer and appeared to be in good physical health. Her oral metastasis marked the initial manifestation of an otherwise silent primary renal cancer.

Oral manifestations of lipoid proteinosis in a 10-year-old female: A case report and literature update.

Lipoid proteinosis (LP) is a rare autosomal recessive disorder characterized by the deposition of amorphous hyaline material in the dermis and submucosal connective tissue. Here, we present a case of LP with significant oral, dermatologic, and neurologic manifestations occurring in a 10 year-old female of Asian descent. In addition to the more typical oral findings of restricted tongue movement and labial and buccal mucosal involvement, this case highlights an unusual pattern of gingival enlargement infrequently reported in the literature. As LP almost always involves the oral cavity, often before the onset of cutaneous lesions, it is important for dental providers to be familiar with the oral manifestations of this disease. Early detection and diagnosis of LP by the dental practitioner can help expedite proper multidisciplinary care and lead to significant reductions in patient morbidity and mortality.

Mandibular embryonal rhabdomyosarcoma with cartilaginous metaplasia: report of a case and review of literature.

Rhabdomyosarcoma (RMS) is a malignant tumor of skeletal muscle origin and frequently involves the head and neck region. It represents the second most common pediatric soft tissue sarcoma and accounts for 3% of all childhood cancers. Here, we report a case of embryonal RMS presenting as a right-sided facial swelling in a 7-year-old boy. Histologically, the tumor consisted of classic embryonal rhabdosarcomatous areas with metaplastic cartilage, in both initial biopsy and final resection specimens. Cartilaginous metaplasia arising in the background of RMS is a rare finding, thus raising a diagnostic challenge. To the best of our knowledge, this represents the first case of RMS with cartilaginous metaplasia in the oral cavity.

Langerhans Cell Histiocytosis Mimicking Periapical Pathology in a 39-year-old Man.

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type dendritic cells, with more than 50% of cases of LCH seen in children younger than 15 years of age. The most common clinical presentation of LCH is solitary or multiple bony lesions. The jaws are affected in approximately 10%-20% of cases, with a strong predilection for the mandible. The maxilla is involved in only 1% of head and neck cases. When the jaws are involved, lesions of LCH may mimic periapical pathology as seen in patients requiring endodontic therapy or bone loss as seen in periodontal disease. We report the case of a 39-year-old man with LCH involving the posterior maxilla. This is a rare presentation of LCH with respect to both location and patient age. Clinicians should consider LCH when developing a differential diagnosis of an apical radiolucency of vital teeth or teeth that fail to respond to endodontic therapy and be aware of its clinical and radiographic mimics.

Chronic lymphocytic leukemia in association with a ranula: a report and review of the literature.

Chronic lymphocytic leukemia (CLL), an indolent neoplasm of B lymphocytes, is the most common adult leukemia in the Western Hemisphere. Despite this, however, the intraoral presentation of CLL is quite rare. We report the case of an 83-year-old woman with CLL incidentally involving minor salivary glands in association with a ranula (floor-of-mouth mucocele).

Primary Ewing sarcoma of the anterior mandible localized to the midline.

Ewing sarcoma is a malignant, small, round blue-cell tumor of the bone that is usually located in the long bones and the pelvis. Fewer than 3% of all Ewing sarcomas originate in the head and neck region and these are mostly located in the posterior mandible. We report the case of a 17-year-old girl with a primary Ewing sarcoma localized at the midline of the anterior mandible.

Topical Application of Green Tea Polyphenol (-)-Epigallocatechin-3-gallate (EGCG) for Prevention of Recurrent Oral Neoplastic Lesions.

OBJECTIVE: A preliminary study was conducted to investigate feasibility of using an oral cancer chemopreventive agent (-)-epigallocatechin-3-gallate (EGCG), the most biologically active component in the green tea extract, in a form of 'swish-and-spit' mouthwash. Such application of EGCG is beneficial as it maximizes exposure of the oral mucosa to the agent but minimizes systemic side effect. STUDY DESIGN: The study was conducted on individuals suspected to have oral field cancerization who are at a high risk for developing recurrent oral precancerous and carcinomatous lesions. EGCG was used as a daily mouthwash for 7 days. EGCG's ability to modulate target molecules implicated in oral carcinogenesis was assessed by measuring the change in expression level of biomarkers. RESULTS: Immunohistochemical expression of phosphoactivated epidermal growth factor receptor (pEGFR), cyclooxygenase-2 (cox-2) and ki-67 were evaluated at baseline and at the endpoint (day 8). Although not statistically significant, overall decrease in expression levels of pEGFR (27.5%), cox-2 (15.9%) and ki-67 positive cells (51.8%) were observed following EGCG treatment. Moreover, a detectable level of EGCG was found in saliva but not in plasma after the one-week treatment regime, demonstrating local availability of EGCG in oral mucosa without significant systemic absorption. CONCLUSION: To best of our knowledge this is the first study to explore use of oral cancer chemopreventive agent in a form of mouthwash in patients with oral field cancerization. Although a definitive conclusion was not reached due to limited sample size, if proven effective, EGCG therapy may offer a non-invasive preventive modality for oral field cancerization.