Hair Follicle Nevus With Features of Comedo Nevus: An Expanding Spectrum.

Hair follicle nevus (HFN) is a rare hamartomatous lesion of the folliculosebaceous unit, with or without admixed fibroadipose or muscular tissue. It typically has a congenital presentation in the preauricular area of infants and is frequently confused with an accessory tragus. Acquired tumors with similar histopathologic features have been described infrequently during adolescence and adult life. We report yet another unique presentation of this unusual lesion in a 4-year-old girl who had a long-standing tumor of the nasal columella that started growing rapidly after trauma. Histopathologic examination revealed increased numbers of hair follicles, some of which were associated with diminutive sebaceous glands, with no associated central cystic structure. In addition, the infundibula of the follicles were dilated and filled with keratinous debris. Although these hamartomas are common in the head and neck region, to our knowledge, this is the first report of a HFN at this anatomic location. In addition, this tumor has an overall architecture of a HFN but is accompanied by features of a comedo nevus. We also present a review of the literature and summarize the current diagnostic criteria for HFN.

Evaluating a Web-Based Educational Module on Oral Cancer Examination Based on a Behavioral Framework.

Patients at risk of developing oral and/or oropharyngeal cancer (OPC) are more likely to see primary care providers (PCPs) than a dentist. Many PCPs do not regularly perform oral cancer examination (OCE). The purpose of this study was to design a web-based educational program based on a behavioral framework to encourage PCPs to conduct OCE. PCPs were solicited to provide feedback on the program and to evaluate their short-term knowledge. The integrated behavioral model was used to design the program. Fifteen PCPs (five in each group: physicians, physician assistants, and nurse practitioners) reviewed the program and took a posttest: (1) index of knowledge of risk factors for oral cancer (RiskOC) and (2) index of knowledge of diagnostic procedures for oral cancer (DiagOC). Findings from the process evaluation were mainly positive, with comments on the length of the program comprising the ten negative comments. No significant difference among groups of PCPs (physicians, physician assistants, and nurse practitioners) was detected for DiagOC (p = 0.43) or RiskOC (p = 0.201). A program on OPC for PCPs should be less than 40 min. Postviewing knowledge outcomes were similar for all PCPs. The web-based program on OPC based on a behavioral framework could have similar short-term knowledge outcomes for all PCPs and may increase the number of PCPs performing OCEs.

Human cytomegalovirus and mucoepidermoid carcinoma of salivary glands: cell-specific localization of active viral and oncogenic signaling proteins is confirmatory of a causal relationship.

Human cytomegalovirus (hCMV) infection is common. Although still controversial, there is growing evidence that active hCMV infection is associated with a variety of malignancies, including brain, breast, lung, colon, and prostate. Given that hCMV is frequently resident in salivary gland (SG) ductal epithelium, we hypothesized that hCMV would be important to the pathogenesis of SG mucoepidermoid carcinoma (MEC). This was initially supported by our finding that purified CMV induces malignant transformation in SG cells in an in vitro mouse model, and utilizes a pathogenic pathway previously reported for human MEC. Here we present the histologic and molecular characterizations of 39 human SG MECs selected randomly from a repository of cases spanning 2004-2011. Serial sections were obtained from formalin-fixed, paraffin embedded, tissue blocks from previous incisional or excisional biopsies. Immunohistochemical assays were performed for active hCMV proteins (IE1 and pp65) and the activated COX/AREG/EGFR/ERK signaling pathway. All four prospective causal criteria for viruses and cancer are fully satisfied: (1) protein markers for active hCMV are present in 97% of MECs; (2) markers of active hCMV are absent in non-neoplastic SG tissues; (3) hCMV-specific proteins (IE1, pp65) are in specific cell types and expression is positively correlated with severity; (4) hCMV correlates and colocalizes with an upregulation and activation of an established oncogenic signaling pathway (COX/AREG/EGFR/ERK). Thus, the evidential support reported here and previously in a mouse model is strongly confirmatory of a causal relationship between hCMV and SG mucoepidermoid carcinoma. To our knowledge, this is the first demonstration of hCMV's role in human oncogenesis that fully responds to all of Koch's Postulates as revised for viruses and cancer. In the absence of any contrary evidence, hCMV can reasonably be designated an "oncovirus."

Multiple endocrine neoplasia-2B presenting with orthodontic relapse.

The multiple endocrine neoplasia (MEN) syndromes are a relatively uncommon group of genetic disorders characterized by the development of tumors in various endocrine organs. MEN type 2B is of particular interest to the dental profession because of its oral manifestations, which are often some of the earliest clinically detectable signs of the disorder. Early identification of this syndrome is critical because affected patients often develop a characteristic malignancy, medullary carcinoma of the thyroid, at a very early age. We describe a 17-year-old male whose initial diagnosis of MEN-2B was triggered by his orthodontist's request for an oral and maxillofacial pathology consultation to evaluate the patient's oral abnormalities.

Oral Mycosis Fungoides: A Report of Three Cases and Review of the Literature.

Mycosis fungoides (MF) and Sézary syndrome are clonal T-cell proliferations that exhibit skin homing and represent the majority of cutaneous T-cell lymphomas. Early MF is a diagnostic challenge as both the clinical and microscopic features often mimic benign inflammatory conditions. Oral MF is very rare and has been associated in the past with advanced disease and a poor prognosis. Skin lesions are present for an average of > 6 years before oral involvement occurs. The clinical appearance is highly variable with tongue, palate and gingiva most often affected. We report 3 additional cases of oral MF, including one in which oral lesions are the initial disease presentation. Survival in patients presenting with oral MF is improving and can be attributed to advances in therapy.

Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region.

Plexiform schwannoma is a rare variant of Schwann cell tumor. Occurring in either conventional or cellular type, they are characterized either grossly or histologically by a plexiform pattern of intraneural growth often with multinodularity. Ordinary as well as plexiform schwannoma typically arise in superficial soft tissues and show a predilection for the head and neck region. Infrequent examples arise in the setting of neurofibromatosis type 2 or schwannomatosis. The purpose of this study was to assess the frequency of plexiform schwannoma by location, to determine their syndromic association, and to analyze the clinicopathologic features of tumors affecting the head and neck region. It was found, in this not entirely random population, that plexiform schwannoma represented 4.3% of all schwannomas, 23% of head and neck region examples, 15% of cutaneous schwannomas, and lastly, 2% of 322 oral nerve sheath tumors made separately available for review. Furthermore, the association with neurofibromatosis type 2 and with schwannomatosis was 5% each.

AAOMP case challenge: a pigmented lesion of the mandibular facial gingiva.

A 55-year-old white male was referred by his dermatologist for evaluation of an asymptomatic dark brown lesion on the mandibular facial attached gingiva.

Opportunistic Oral Infections.

An opportunistic infection (OI) is a disease of microbial cause or pathogenesis generally thought to occur in hosts with weakened immunity. Oral OIs are associated with many risk factors and pathogens. Causative organisms for oral OIs have unique modes of transmission. The clinical presentation of oral OIs is heterogeneous and diagnosis can be challenging. Therefore, laboratory identification of causative pathogens is useful for definitive diagnosis and targeted therapeutics, and can be achieved by biological, serologic, histologic, and/or molecular methods. Clinical risk assessment and history with review of systems, and accurate diagnosis, treatment, and follow-up, are essential.

Gnathic osteoblastoma: clinicopathologic review of seven cases with long-term follow-up.

The clinical, radiographic and histopathologic features of seven cases of osteoblastoma of the jaw bones were analyzed. The mandible was involved in six cases and a periosteal presentation of the tumor was noted in three cases. Six tumors were symptomatic with pain being the common presentation. The radiographic appearance of the intraosseous tumors varied from well-defined radiolucencies to poorly-defined mixed lesions. A small periosteal tumor was undetectable radiographically. Histopathologically, the tumors exhibited variation in woven bone formation, osteoblastic rimming, lesional maturation as well as interaction of the tumor with surrounding host tissue. Intraosseous tumors were managed with aggressive surgical curettage and periosteal tumors were resected and the underlying bone was curetted down to normal host bone. Follow-up of cases ranged from 2 years to 18 years with no recorded recurrences. Gnathic osteoblastomas offer a unique diagnostic challenge as they resemble other tumors of the jaws including the cementoblastoma, cemento-ossifying fibroma, cemento-osseous dysplasia and most importantly the gnathic osteosarcoma. We stress on the need to correlate the histopathologic findings with clinical and radiographic features to reach an accurate diagnosis.

Oral ulceration associated with concurrent herpes simplex virus, cytomegalovirus, and Epstein-Barr virus infection in an immunocompromised patient.

In immunocompromised patients, oral ulcerations are common and have a wide spectrum of causes, including herpesvirus infection. We report on a case in which an oral ulcer was simultaneously infected by herpes simplex (HSV), cytomegalovirus (CMV), and Epstein-Barr virus (EBV) in a kidney-pancreas transplant recipient. A 46-year-old woman presented with a clinically nonspecific dorsal tongue ulcer of 3 months duration. Histopathologic evaluation indicated keratinocytes exhibiting herpetic viral cytopathic effect. Nuclear and cytologic alterations suggestive of CMV infection were found in endothelial cells subjacent to the ulcer. Immunohistochemistry testing for HSV and CMV was positive in these cells. Large atypical mononuclear cells were also evident in the ulcer bed's inflammatory infiltrate, which had intense nuclear positivity for Epstein-Barr encoding region in situ hybridization. We believe this is the first well-documented report of the definitive concomitant presence of HSV, CMV, and EBV in an immunocompromised patient. Although the pathogenesis of coinfected ulcers remains unknown, a synergistic effect is possible.

The prevalence of pseudoxanthoma elasticum-like connective tissue changes in an oral biopsy service and review of the literature.

OBJECTIVE: The objective of this pilot study is to determine the prevalence of pseudoxanthoma elasticum (PXE)-like connective changes in an oral biopsy service and compare it with the estimated prevalence of PXE as well as to the prevalence of the mutated PXE gene ABCC6. STUDY DESIGN: This prevalence study utilized 500 oral mucosal biopsy specimens received from the biopsy service of the Oral Pathology Consultants at the Ohio State University. Each specimen was microscopically evaluated using hematoxylin and eosin, Verhoeff-van Gieson and von Kossa stains. RESULTS: A prevalence of 9.8% was identified for PXE-like changes in the connective tissue of oral biopsy specimens submitted to this service. CONCLUSIONS: The overall prevalence of PXE-like connective tissue changes found in routine oral mucosal biopsy specimens (9.8%) was much higher than either the suspected prevalence of PXE (0.001%-0.004%) or the estimated prevalence of the mutated gene ABCC6 (0.625%-1.25%).

Undifferentiated malignant neoplasm involving the interdental gingiva: a case report.

BACKGROUND: Metastases to the gingiva are uncommon. They can be a diagnostic challenge clinically because of their rarity and tendency to mimic benign lesions. In this report, we present a case of an undifferentiated malignant neoplasm of unknown origin presenting as benign inflammatory gingival lesions and we review the literature on metastases to the gingiva. METHODS: A 44-year-old female patient was referred by a local periodontist for evaluation of multiple painless gingival lesions that clinically resembled pyogenic granulomas or periodontal abscesses, but with an uncharacteristic multifocal presentation in the background of good oral hygiene. Her medical history was unremarkable except for recent weight loss. Periapical radiographs were obtained, as well as two incisional biopsies, one placed in formalin for routine histology and immunohistochemistry, the other in phosphate buffered saline for flow cytometry. RESULTS: Radiographic findings were non-contributory for hard tissue pathoses. Histopathological findings were consistent with a poorly differentiated malignancy, suggestive of metastatic disease. Immunohistochemical studies and flow cytometry were unsupportive in delineating any tumor differentiation. The patient subsequently developed multiple tumors throughout the body with similar histopathological findings, yet no primary tumor was identified and a definitive diagnosis could not be rendered. She was discharged one month later in poor condition with the principal diagnosis of hemorrhage/ pancytopenia, and a secondary diagnosis of metastatic head and neck cancer. CONCLUSION: This case of malignant metastatic tumor of unknown origin presenting as benign gingival lesions illustrates the importance of thorough patient evaluation, which should include a biopsy when necessary for definitive diagnosis.

Human papillomavirus, type 40-associated papilloma, and concurrent Kaposi's sarcoma involving the anterior hard palate of an HIV-positive man.

A number of oral lesions have been reported in association with HIV, including lesions caused by other viruses such as the epitheliotropic human papillomavirus (HPV). More than 90 types of HPV have been identified, with the less commonly encountered strains of HPV tending to show association with immunodeficiency states. In addition, HIV-infected patients may have Kaposi's sarcoma develop, a malignancy thought to be caused by human herpes virus, type 8. Recent evidence suggests a sexual mode of transmission for this virus. We report an HIV-positive man with a large, HPV type 40-associated papilloma of the anterior palate and a previously undiagnosed focus of Kaposi's sarcoma.

Celiac disease and recurrent aphthous stomatitis: a report and review of the literature.

Celiac disease (CD) is a condition related to the small intestine's intolerance to gluten. The diagnosis of CD can be difficult, especially because patients may exhibit a wide spectrum of signs and symptoms. It is important to identify this disease process early because affected individuals have an increased risk for developing lymphoma of the gut. Our objective was to evaluate whether patients with CD have a significantly higher prevalence of recurrent aphthous stomatitis compared with the general population, as some investigators have speculated. Therefore, we screened 61 patients with diagnosed CD for the presence of, or a positive history of, aphthous ulcerations. We then statistically compared this data with a randomly selected control population, matched for age and gender, but without CD. Our results demonstrated no significant differences between groups for age, gender, or prevalence of recurrent aphthous stomatitis.

Pagetoid reticulosis: a case report and review of the literature.

The histologic finding of a pagetoid epidermotropic growth pattern is associated with a variety of conditions that range from reactive to neoplastic in nature. Included among these conditions is the uncommon skin disorder pagetoid reticulosis. Pagetoid reticulosis may present clinically as a solitary, indolent plaque-like lesion. Conversely, it may manifest as a more generalized clinically aggressive disease. The generalized form may develop into systemic lymphoma, leading to severe morbidity and death. We present a case of pagetoid reticulosis of the perioral region, a rare anatomic site for this condition. Histopathologic and immunohistochemical findings were consistent with a diagnosis of isolated pagetoid reticulosis. This review discusses the nature of pagetoid reticulosis and addresses past and current concepts regarding the condition.

The histologic differentiation of oral condyloma acuminatum from its mimics.

OBJECTIVE: The purpose of this study was to determine which histologic features could enable one to distinguish oral condyloma, as defined by the detection of human papillomavirus (HPV) DNA through in situ hybridization, from its mimics. STUDY DESIGN: Thirty-two paraffin-embedded specimens from 28 patients that were clinically suggestive of oral condyloma were analyzed histologically and through in situ hybridization with a consensus HPV probe. RESULTS: HPV DNA was detected in 17/32 (53%) of the lesions; no additional positive cases were detected after polymerase chain reaction amplification. Only 5 of the 17 virus-positive cases were considered to be histopathologically unequivocal for condyloma. The histologic features significantly associated with HPV detection were nonuniform perinuclear halos, often in association with epithelial crevices (P =.02), and papillomatosis (P =.02). Each of the 17 patients who were HPV-positive had either HPV-6 or HPV-11, a finding that is similar to those for condyloma involving the penis and vulva/vagina. CONCLUSION: Differentiation between oral condyloma and its mimics is best accomplished by using a combination of histologic, clinical, and in situ viral studies.

Post-irradiation leiomyosarcoma of the maxilla: report of a case in a patient with prior radiation treatment for retinoblastoma.

Post-irradiation sarcoma is a well-defined complication of radiation therapy, yet few reports document such lesions in the head and neck. A 30-year-old man presented for evaluation of an expansile lesion of the left posterior maxilla. His medical history was significant for a childhood ocular malignancy - unilateral retinoblastoma - which was treated with a combination of surgical enucleation of the eye and external beam radiation therapy. Biopsy of his maxillary lesion revealed a spindle cell malignancy that was morphologically and immunohistochemically consistent with a diagnosis of leiomyosarcoma. Further investigation into the case revealed that the patient had three children, every one of whom developed unilateral retinoblastoma in infancy. Compared to the more frequent presentation of bilateral tumors in hereditary cases of retinoblastoma, such cases of heritable unilateral retinoblastoma are exceptional. Importantly, heritable forms of retinoblastoma confer a significant risk for development of second primary cancers, necessitating long-term clinical follow-up in these patients.