Multiple Endocrine Neoplasia Type 2b (MEN2B) in a 9-Year-Old Female.

Multiple endocrine neoplasia (MEN) is an uncommon genetic syndrome transmitted as an autosomal dominant condition characterized by multiple tumors or hyperplasia of neuroendocrine tissues. MEN type 2b (MEN2B) often has clinical signs of marfanoid facial appearance and mucosal neuromas of the head. This report describes the diagnosis of MEN2B in a previously undiagnosed 9 year old who presented for biopsy of an oral lesion.

Plasma Cell Gingivitis: Clinical and Pathological Case Report.

INTRODUCTION: Plasma cell gingivitis (PCG) is a rare benign condition usually found on marginal and attached gingiva. This case details a generalized PCG, to include the management of the patient and clinicopathologic characteristics of the disorder. CASE PRESENTATION: The patient, a 24-year-old African American female, was referred to the periodontics clinic for severe generalized gingival erythema and edema. Past medical history review was remarkable for sickle cell anemia and systemic lupus erythematosus. The patient was initially prescribed dexamethasone oral rinse (0.5 mg/5 ml) pending biopsy and medical consult for potential causes of any hypersensitivity reaction. The patient also was instructed to discontinue her current type of oral mouthrinse and dentifrice. Biopsy results confirmed a diagnosis of PCG. Resolution of signs and symptoms started 1 month later, and approximately 2 years after initial diagnosis the patient was clinically stable. CONCLUSION: This report describes the management of a diffuse plasma cell gingivitis and reviews pertinent literature on the lesion. The etiology of PCG, although unclear, may be attributable to a hypersensitivity reaction. PCG may mimic other pathological entities, which underscores the importance of microscopic examination in establishing a definitive diagnosis before treatment initiation.

Development of a rat model of bisphosphonate-related osteonecrosis of the jaw (BRONJ).

The purpose of this study was to develop a rat model predictive of bisphosphonate-related osteonecrosis of the jaw (BRONJ) after exodontias. Thirty female rats were randomized into 2 groups, control and experimental. The experimental group received 2 intravenous injections of zoledronate (20 µg/kg). The mesial root of the right mandibular first molar was extracted. Rats were euthanized at 0, 4, and 8 weeks. Bone mineral density (BMD), collagen breakdown (pyridinium [PYD]), vascular regeneration (VEGF), and histology were examined. A trend toward higher PYD values was suggested in control vs experimental groups after wounding. Serum VEGF increased significantly after wounding for both control and experimental groups. After 8 weeks, VEGF continued to rise for the experimental group only. In the extraction socket area, BMD was significantly lower after wounding in control vs. zoledronate-treated rats. Histology sections from experimental groups showed bacteria and bone necrosis. Consistent findings of BRONJ features similar to those in humans were observed after zoledronate treatment.

A Locally Aggressive Ameloblastic Fibro-Odontoma: A Case Report and Literature Review.

Ameloblastic fibro-odontoma (AFO) is a relatively rare, benign noninvasive mixed odontogenic neoplasm derived from epithelial and ectomesenchymal elements of the dental tissues. It usually presents with a mean age of 11.5 years and in the posterior segment of the mandible. It is extremely rare in the posterior maxilla. Although the latest WHO edition classified AFO as developing odontoma, here we present a locally aggressive AFO in a 21-year-old male involving the posterior maxilla and sinus with bone destruction. The patient presents with a two-year history of slowly progressive left facial swelling with malodorous drainage. The CT scan revealed a 5.5 x 4.3 cm well-circumscribed expansile mass with mixed attenuation and peripheral calcification occupying the left maxilla and sinus with bone destruction of the hard palate and orbital rim. According to the literature, most of the AFO cases were treated adequately through a conservative approach with just enucleation or surgical curettage. To our knowledge, our case is the first case treated aggressively with left maxillectomy, palatectomy, and reconstruction surgery because of its radiologic findings, which suggested a locally invasive neoplasm. Histologically, the specimen showed a mixture of proliferative epithelial, mesenchymal tissue elements, and variable amounts of mineralized deposits consisting of enamel matrix and dentinoid deposits, and the final diagnosis was AFO. In conclusion, we present a rare case of AFO with an unusual aggressive presentation, age group, and site involved. The radiographic, histopathologic features, and therapeutic approaches of this unusual locally aggressive tumor are presented with the review of relevant literature.

Bacterial Invasion of Pulp Blood Vessels in Teeth with Symptomatic Irreversible Pulpitis.

INTRODUCTION: This study described the degenerative changes and infection patterns of the pulp tissue associated with symptomatic irreversible pulpitis. METHODS: The material consisted of 32 extracted teeth with untreated deep caries that were clinically and histologically diagnosed with irreversible pulpitis and were part of the histopathologic collection of 1 of the authors. The controls consisted of intact teeth with normal uninflamed pulps and teeth with reversible pulpitis. Teeth were processed for histopathologic and histobacteriologic analyses. RESULTS: All teeth with irreversible pulpitis showed areas of severe acute inflammation, necrosis, microabscesses, and bacterial infection in the pulp chamber. These areas were surrounded by a chronic inflammatory infiltrate, and, at the distance, the pulp tissue was often uninflamed. Bacteria were also observed in the areas surrounding the necrotic foci, both as scattered cells through the extravascular space and at varying numbers within the blood vessel lumen. The number of bacteria and the density of the intravascular bacterial aggregations varied considerably. In one third of the cases, bacteria occurred in the lumen of venules in areas at a considerable distance from the necrotic focus in the coronal third of the root. No intravascular bacteria were noted in the middle and apical segments of the canal. No bacteria were found in the pulps of any of the control specimens. CONCLUSIONS: Bacterial invasion and colonization of necrotic areas were observed in the pulp of all teeth with caries exposure and symptomatic irreversible pulpitis. Bacterial penetration of blood vessels occurred in all cases, suggesting that this may be an important mechanism of spread of bacterial infection through the pulp tissue in an endodontic infection.

A dual role of 12/15-lipoxygenase in LPS-induced acute renal inflammation and injury.

Recent studies suggest a potential role of bioactive lipids in acute kidney injury induced by lipopolysaccharide (LPS). The current study was designed to determine the profiling activities of various polyunsaturated fatty acid (PUFA) metabolizing enzymes, including lipoxygenases (LO), cyclooxygenase, and cytochrome P450 in the plasma of LPS-injected mice using LC-MS. Heat map analysis revealed that out of 126 bioactive lipids screened, only the 12/15-LO metabolite, 12-HETE, had a significant (2.24 ±â€¯0.4) fold increase relative to control (P = 0.0001) after Bonferroni Correction (BCF α = 0.003). We then determined the role of the 12/15-LO in LPS-induced acute kidney injury using genetic and pharmacological approaches. Treatment of LPS injected mice with the 12/15-LO inhibitor, baicalein, significantly reduced levels of renal injury and inflammation markers including urinary thiobarbituric acid reactive substance (TBARs), urinary monocyte chemoattractant protein-1 (MCP-1), renal interleukin-6 (IL-6), and tumor necrosis factor-α (TNF-α). Similarly, knocking-out of 12/15-LO reduced levels of renal inflammation and injury markers elicited by LPS injection. Next, we tested whether exogenous supplementation with docosahexaenoic acid (DHA) as a substrate would divert the role of 12/15-LO from being pro-inflammatory to anti-inflammatory via increased production of the anti-inflammatory metabolite. DHA treatment restored the decreased in plasma level of resolvin D2 (RvD2) and reduced renal injury in LPS-injected mice whereas DHA treatment failed to provide any synergistic effects in reducing renal injury in LPS injected 12/15-LO knock-out mice. The ability of RvD2 to protect kidney against LPS-induced renal injury was further confirmed by exogenous RvD2 which significantly reduced the elevation in renal injury in LPS injected mice. These data suggest a double-edged sword role of 12/15-LO in LPS-induced acute renal inflammation and injury, depending on the type of substrate available for its activity.

Metastatic tumors in the jaws: a retrospective study of 114 cases.

BACKGROUND: Malignancies involving the bones are metastatic tumors more commonly than primary tumors. In this retrospective study, the authors review metastatic disease in the jaws. METHODS: The authors retrieved cases of metastatic disease in the jaws over a 45-year period from the pathology archives at the University of Michigan School of Dentistry, Ann Arbor, and Indiana University School of Dentistry, Indianapolis. RESULTS: The authors conducted a retrospective analysis of 114 cases of metastatic disease in the jaws and found that approximately 60 percent of subjects had no history of malignancy. The sex distribution was equivalent. Mandibular predilection was more prominent in females than in males. Metastases from the breast were significantly greater than those from the lung and prostate (P < or = .05), the second and third most frequent sites, respectively. Women exhibited twice as many jaw metastases as did men 31 to 40 years of age and significantly fewer metastases than did men 71 to 80 years of age (P < or = .05). CONCLUSION: In the majority of cases, subjects had an undiagnosed primary cancer at the time the metastatic jaw disease presented. The most common site of origin of the primary cancer was the breast, when primary sites were considered independent of sex. CLINICAL IMPLICATIONS: Patients with metastatic disease in the jaws may have innocuous dental symptoms, such as pulpal or periodontal pain; therefore, clinicians will play a significant role in diagnosing the life-threatening disease.

Lingual Gingival Cyst of the Adult.

INTRODUCTION: Gingival cysts of the adult (GCAs) are rare developmental soft tissue cysts usually found on the facial attached gingiva. Documented cases on the lingual aspect are extremely uncommon. This report details a lingual GCA along with distinguishing characteristics of this lesion. CASE PRESENTATION: The patient, a 37-year-old female, presented with a firm, round-shaped, bluish swelling on the lingual gingiva in the interproximal area between teeth #21 and #22. Radiographs revealed a circular radiolucency at the osseous crest. Surgical exploration showed cystic-appearing tissue with accompanying bone resorption. Histologic examination, along with clinical presentation, confirmed a diagnosis of gingival cyst. Healing was uneventful with no evidence of recurrence at a 5-month recall. CONCLUSIONS: A GCA is usually of odontogenic origin and occurs typically in the attached gingiva of the mandibular premolar region. Occasionally, it demonstrates radiographic evidence of bone loss from pressure resorption. Its more frequent intraosseous counterpart, the lateral periodontal cyst, shares histopathologic characteristics with the GCA. These findings underscore the importance in establishing a definitive diagnosis.

Retrospective study on idiopathic bone cavity and its association with cementoosseous dysplasia.

OBJECTIVE: The goal of this study was to reveal clinical and pathologic findings on idiopathic bone cavity lesions (IBC). STUDY DESIGN: A retrospective analysis of 20 IBC cases diagnosed from 2004 to 2014 from a university-based maxillofacial pathology service was performed and included all pertinent clinical, histologic, and radiographic findings. RESULTS: Eleven women (age = 36 ± 12.7) and 9 men (age = 23 ± 17.9) diagnosed with IBC were selected for analysis. There was a higher African-American female predilection (40%). Thirty percent of the cases were associated with florid cementoosseous dysplasia (COD) (all middle-aged African-American women). The location of the lesions was mandibular in 85% of the patients. All symptomatic patients (25%) had concomitant COD. Only 1 patient reported previous trauma, and only 1 patient had prior orthodontic treatment. Follow-up period ranged from 1 to 8 years, with only 1 recurrence 3 years after surgery. CONCLUSIONS: The results suggest that IBC concurrent with COD may not be as rare as the literature implies. Clinicians must be attentive to this possible relationship to ensure accurate diagnosis and appropriate treatment.

Parathyroid hormone-related protein (PTHrP) expression in ameloblastoma.

OBJECTIVE: Parathyroid hormone-related protein (PTHrP) production has been demonstrated in a variety of tumor subtypes. Local production of PTHrP by metastatic tumor cells in bone has been linked to bone destruction and tumor growth. Ameloblastoma (AB) is a relatively common odontogenic epithelial neoplasm that manifests local infiltrative intraosseous growth. AB recapitulates the developing enamel epithelium, in which PTHrP recently has been demonstrated. Yet PTHrP expression in a series of ABs has not been studied to date. The purpose of this investigation is to assess the expression of PTHrP in ameloblastoma. STUDY DESIGN: Formalin-fixed, paraffin-embedded tissue sections of ameloblastoma (n = 30; 24 conventional, 4 unicystic, and 2 arising in dentigerous cyst) were immunostained with anti-PTHrP antibody using a multistep streptavidin-peroxidase technique. Semiquantitative scoring of immunoreactivity was assessed as mild, moderate, and intense. RESULTS: All cases (100%) demonstrated positive immunoreactivity, with mild reaction in 3 conventional ABs, 1 unicystic and 1 AB arising in dentigerous cyst, and with moderate reaction in 12 conventional ABs, 3 unicystic and 1 AB arising in dentigerous cyst. Intense immunoreactivity was seen in 9 cases of conventional AB. This difference in immunostaining was not statistically significant (Sigma2 = 4.41, df = 4, P = .358). CONCLUSION: The results of this investigation suggest that PTHrP may play a significant role in local bone resorption, offering at least partial explanation for the tumor's infiltrative growth and destructive behavior. The uniformity of PTHrP expression by AB, as detailed in this study, may harbor significant therapeutic implications, particularly through PTHrP-blocking treatment modalities.

Oral precancerous and malignant lesions associated with graft-versus-host disease: report of 2 cases.

The development of secondary malignancies has been recognized as a potential iatrogenic complication in patients who have graft-versus-host disease secondary to bone marrow transplantation. Lymphohematopoietic cancer is most frequent, although solid malignancies have also been reported. We describe 2 patients with graft-versus-host disease who developed oral precancerous and malignant lesions. The first patient, a 24-year-old white man, had erythroplakia of the buccal mucosa that proved to be carcinoma in situ histopathologically. The second patient, a 14-year-old Hispanic boy, developed synchronous cutaneous and lingual squamous cell carcinomas. The current cases and similar sporadic case reports found in the literature highlight the susceptibility of patients with graft-versus-host disease to the development of oral cancer. Therefore, it is recommended that thorough evaluation of the oral mucosa and close follow-up be offered to all patients treated with bone marrow transplantation and particularly to those who develop graft-versus-host disease.

Prevalence of ciliated epithelium in apical periodontitis lesions.

INTRODUCTION: This article reports on the morphologic features and the frequency of ciliated epithelium in apical cysts and discusses its origin. METHODS: The study material consisted of 167 human apical periodontitis lesions obtained consecutively from patients presenting for treatment during a period of 12 years in a dental practice operated by one of the authors. All of the lesions were obtained still attached to the root apices of teeth with untreated (93 lesions) or treated canals (74 lesions). The former were obtained by extraction and the latter by extraction or apical surgery. Specimens were processed for histopathologic and histobacteriologic analyses. Lesions were classified, and the type of epithelium, if present, was recorded. RESULTS: Of the lesions analyzed, 49 (29%) were diagnosed as cysts. Of these, 26 (53%) were found in untreated teeth, and 23 (47%) related to root canal-treated teeth. Ciliated columnar epithelium was observed partially or completely lining the cyst wall in 4 cysts, and all of them occurred in untreated maxillary molars. Three of these lesions were categorized as pocket cysts, and the other was a true cyst. CONCLUSIONS: Ciliated columnar epithelium-lined cysts corresponded to approximately 2% of the apical periodontitis lesions and 8% of the cysts of endodontic origin in the population studied. This epithelium is highly likely to have a sinus origin in the majority of cases. However, the possibility of prosoplasia or upgraded differentiation into ciliated epithelium from the typical cystic lining squamous epithelium may also be considered.

Tophaceous pseudogout of the temporomandibular joint: a series of 3 cases.

OBJECTIVE: We report a series of 3 cases of tophaceous pseudogout of the temporomandibular joint (TMJ). STUDY DESIGN: Three patients, two men and one woman, ranging in age between 60 and 75 years, presented with unilateral painful swelling of the TMJ area associated with limitation of mouth opening. RESULTS: Radiographic and computed tomographic images showed opaque masses in the supracondylar region of the TMJ. The preoperative clinical impression was a "neoplastic lesion" in the 3 cases. Microscopic examination revealed numerous deeply basophilic masses of calcified deposits, exhibiting birefringence under polarized light and morphologically consistent with calcium pyrophosphate dihydrate deposition, referred to in these cases as "tophaceous pseudogout." CONCLUSION: Tophaceous pseudogout is a rare benign arthropathy that presents with clinical and radiographic features mimicking neoplastic conditions of the TMJ. Therefore, it is recommended that tophaceous pseudogout be considered in the differential diagnosis when a TMJ is involved with "neoplasm-like" lesions clinically and radiographically.

Fibrous cortical defect (nonossifying fibroma) of the mandibular ramus: report of 2 cases.

Fibrous cortical defect, also known as metaphyseal fibrous defect and nonossifying fibroma, among other terms, is a benign, non-neoplastic proliferative process that is relatively common in the long bones of skeletally immature adolescents. Although the lesion is thought to be a developmental abnormality or defect, the exact etiopathogenesis is largely unknown. The lesion has a limited growth potential and good treatment outcome. This process is very rare in the gnathic bones, and only a few sporadic case reports are documented. This article presents the clinicopathologic features of 2 additional cases and reviews the relevant literature in attempt to identify key features and facilitate rendering definitive diagnosis for this rare entity.

Two members of the SIBLING family of proteins, DSPP and BSP, may predict the transition of oral epithelial dysplasia to oral squamous cell carcinoma.

BACKGROUND: : Patients with oral premalignant lesions (OPL) present with oral squamous cell carcinomas (OSCC) at a much higher rate than the general population. There are currently no useful markers that indicate specifically which OPLs are most likely to progress. Three small integrin binding ligands N-linked glycoprotein (SIBLING) family proteins, bone sialoprotein (BSP), osteopontin (OPN), and dentin sialophosphoprotein (DSPP), have been shown to be up-regulated in many cancers, including OSCC. The status of SIBLING expression in OPLs and their correlation to transition to oral cancer are unknown. METHODS: : Sixty archival surgical biopsies of dysplastic OPLs were evaluated by immunohistochemistry for expression of BSP, DSPP, and OPN and correlated with local transformation to OSCC at sites adjacent to surgically removed dysplastic OPL. RESULTS: : The OPL patient population was representative of previous studies with 20% progressing to OSCC, and no correlation between degree of dysplasia and progression. Eighty-seven percent were positive for at least 1 SIBLING protein. OPN expression had no correlation with progression. The BSP+/DSPP- expression pattern however correlated with decreased transformation to OSCC (point prevalence = 0%; 95% confidence interval [CI], 0-20.6), whereas the BSP-/DSPP+ pattern was associated with more frequent progression (point prevalence = 77.8%; 95%CI, 47.8-95.4). Incrementally higher expression scores (0 to 3) of BSP and DSPP were also associated with increased predictive values (odds ratio, 25.53; 95% CI, 2.14-304.7 and 10.13; 95% CI, 2.0-50.0, respectively, for each increment). CONCLUSIONS: : BSP and DSPP are excellent candidate markers for successful OPL surgical intervention and may be predictors of OPL-OSCC progression. Cancer 2010. (c) 2010 American Cancer Society.