Sclerosing epithelioid fibrosarcoma of the jaw: a case report and literature review.

INTRODUCTION: Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare form of bone and soft tissue sarcoma. It occurs mainly in the deep soft tissue of the lower extremities, with few cases reported in the head and neck region. Tumors involving the oral and maxillofacial region (OMFR) and intraosseous examples are rare. CASE PRESENTATION: We present a 52-year-old male with a radiolucent lesion at the apex of the left mandibular second molar tooth with the clinical impression of a periapical granuloma. The patient has a history of SEF of the skull, which was treated by chemotherapy and radiation. Histopathologic examination revealed islands of highly cellular, basophilic bone and osteoid surrounded by loose fibrous stroma, which contains large lobules and islands of round to oval cells with distinct cell borders and faintly granular eosinophilic cytoplasm. Tumor cells were strongly positive for MUC4 and INI-1. Based on these findings, a diagnosis of high-grade malignancy consistent with SEF was made, and correlation with the primary lesion was recommended. CONCLUSION: SEF is a rare tumor seen in OMFR, and therefore, it should be included in the differential diagnosis of any high-grade malignancy. Immunohistochemical stain for MUC-4 evaluation is important for the diagnosis of SEF.

Expansive Central Ossifying Fibroma of the Right Mandible.

This case report discusses a 28-year-old patient who presented with a large expansile lesion of the right mandible. A maxillofacial CT showed a 6.7 x 9.1 x 7.6 cm right mandibular cystic mass containing an internal matrix of ground glass bone, representing a huge odontogenic keratocyte. Upon biopsy of the lesion, the specimen consisted of non-decalcified irregular fragments of cemento-osseous material, embedded in a minimally hemorrhagic, cellular fibrous tissue stroma, suggestive of central ossifying fibroma. This case presents an ossifying fibroma that far exceeds the average size of these masses, which typically range from 1.0 to 2.5 cm at its greatest dimension. The immense size of the lesion seen in this case is rarely encountered. This case also helps to emphasize the importance of timely diagnosis and complete resection of the lesion to prevent mass recurrence and possible malignant transformation.

Bilateral Maxillary Langerhans Cell Histiocytosis in a Six-Month-Old Infant.

Langerhans cell histiocytosis (LCH) is a rare proliferative disease of myeloid cells that can affect multiple organs and present with diverse clinical manifestations. The skeleton, skin and lymph nodes are commonly affected sites, while oral involvement is rare. LCH is currently classified by disease extent into single system and multisystem forms, and further categorized by risk organs. The purpose of this report is to describe the case of a six-month-old girl who presented with a chief complaint of feeding difficulties, premature eruption of the primary left maxillary second molar, expansion of the maxillary alveolar ridges and ulceration of the posterior maxillary oral mucosa. The diverse presentations of pediatric LCH in the literature are reviewed and the role of pediatric dentists and oral surgeons in helping to diagnose LCH is highlighted.

Lymphoid neoplasms of the oral cavity with plasmablastic morphology-a case series and review of the literature.

Plasmablastic lymphoma (PBL) is a rare aggressive variant of large B-cell lymphoma defined as a proliferation of large neoplastic plasmablasts/immunoblasts. PBL was first described as a distinct entity in a group of 16 patients with lymphoma of the oral cavity. Most patients are HIV-positive men. The disease has also been reported in other patient groups, often in association with primary or other acquired immunodeficiency. PBL shows a predilection for the oral cavity, although extraoral involvement also occurs. Because of its rarity, unique clinical features, and overlapping morphologic/immunophenotypic features, care must be taken to distinguish PBL from diffuse large B-cell lymphoma and plasma cell neoplasms with plasmablastic features. We report 3 cases of neoplasms with plasmablastic histomorphology involving the oral cavity. The relevant clinical, morphologic, and immunophenotypic features and treatment are presented, along with a review of the literature.

Florid cemento-osseous dysplasia: review of an uncommon fibro-osseous lesion of the jaw with important clinical implications.

Florid cemento-osseous dysplasia (FCOD) is a rare, benign, multifocal fibro-osseous dysplastic process affecting tooth-bearing areas of the jaw, characterized by replacement of normal trabecular bone with osseous tissue and dense acellular cementum in a fibrous stroma. It is one clinicopathologic variant in a spectrum of related non-neoplastic fibro-osseous lesions known as cemento-osseous dysplasias (CODs), thought to arise from elements of the periodontal ligament. Diagnosis primarily relies upon radiographic and clinical findings; unnecessary biopsy should be avoided, as inoculation with oral pathogens may precipitate chronic infection in these hypovascular lesions. Appropriate management of uncomplicated FCOD consists of periodic radiographic follow-up. Accordingly, it is important that both radiologists and clinicians performing endodontic interventions possess familiarity with this entity in order to prevent misdiagnosis and inappropriate intervention, which may result in a protracted clinical course. Lesions are usually asymptomatic in the absence of infection, typically discovered on routine dental radiographs or imaging performed for unrelated indications. Radiographically, the condition typically manifests as widespread non-expansile intraosseous masses of varying internal lucency and sclerosis that surround the root apices of vital teeth or edentulous areas in the posterior jaw. While all CODs share similar microscopic features, FCOD is distinguished by its multifocal distribution, involving two or more quadrants of the maxilla and mandible, often in a bilateral symmetric fashion. The vast majority of cases are sporadic, though few exhibit an autosomal dominant familial inheritance pattern. In this pictorial review, we discuss the radiologic characteristics of this entity, pertinent clinical and histologic features, differential diagnoses, and management options.

Intravascular fasciitis: report of two intraoral cases and review of the literature.

Two unusual cases of intravascular fasciitis arising in a 25-year-old female and a 26-year-old male are presented here. The lesions apparently presented as firm, raised, submucosal nodules on the tongue. Intravascular fasciitis (IVF) shares the microscopic features of nodular fasciitis (NF), but with intraluminal, intramural, and extramural involvement of small- to medium-sized veins and arteries with a multinodular or serpentine growth pattern along the course of affected blood vessels. NF is a benign lesion occurring on the trunk and upper extremities with a strong predilection for young adults 20 to 40 years of age. Intraoral NF is uncommon, and intraoral IVF is extremely rare, with only sporadic reports in the literature. In both of our cases, the patient's main concern was rapid growth of the lesion, which was nontender, on the tongue. The clinical, histologic, and immunohistochemical features and treatment are presented, along with a review of the literature.

Radiographic evaluation of periapical lesions with and without biopsy-proven actinomyces: a pilot study.

OBJECTIVE: To explore the feasibility of identifying potential radiometric differences in periapical radiolucencies with and without actinomycotic colonization as seen on periapical radiographs using histopathology for ground truth. METHOD AND MATERIALS: Periapical radiographs demonstrating apical lesions with and without biopsy-proven actinomycosis were included in the study. Lesion sizes were estimated on standardized images using anatomical reference points. The reading session was repeated after 2 weeks, and mean lesion sizes were calculated. All measurements were made by a single, calibrated observer. RESULTS: The mean dimension of lesions with actinomyces was 13.51 × 16.43 mm, while lesions without actinomyces had a mean size of 10.24 × 11.27 mm. CONCLUSION: Apical lesions with biopsy-proven actinomyces may be larger in dimension than those without bacterial colonization. Further controlled studies are required to confirm this finding.

Altered miR-146a expression in Sjögren's syndrome and its functional role in innate immunity.

MicroRNAs (miRNAs), small non-coding RNA molecules that post-transcriptionally regulate gene expression, are known to play key roles in regulating immune responses and autoimmunity. We investigated miR-146a expression in Sjögren's syndrome (SjS) patients as well as in the SjS-prone C57BL/6.NOD-Aec1Aec2 mouse model, to elucidate its involvement in SjS pathogenesis. Expression of miR-146a was examined in the PBMCs of 25 SjS patients and ten healthy donors, as well as in PBMCs, salivary and lacrimal glands of SjS-prone mice and WT C57BL/6J mice. Functional assays using THP-1 human monocytes were conducted to determine the biological roles of miR-146a in innate immunity. Expression of miR-146a was significantly increased in SjS patients compared with healthy controls, and was upregulated in the salivary glands and PBMCs of the SjS-prone mouse at both 8 wk (prior to disease onset) and 20 wk (full-blown disease) of age. More importantly, functional analysis revealed roles for miR-146a in increasing phagocytic activity and suppressing inflammatory cytokine production while migration, nitric oxide production and expression of antigen-presenting/costimulatory molecules are not affected in human monocytic THP-1 cells. Taken together, our data suggest that abnormal expression/regulation of microRNAs in innate immunity may contribute to, or be indicative of, the initiation and progression of SjS.

Segmental odontomaxillary dysplasia: review of the literature and case report.

Segmental Odontomaxillary Dysplasia (SOD) is an uncommon, nonhereditary, recently recognized developmental disorder affecting the upper jaw and related dental components. It is a rare condition of uncertain etiology that results in painless unilateral expansion of the posterior dentoalveolar complex, gingival hyperplasia, lack of one or both premolars in the affected area, delayed eruption of adjacent teeth and malformations of the primary molars. Radiographically, the affected bone is thickened and irregular in outline, with coarse trabecular pattern that is vertically oriented resulting in a relatively radiopaque granular appearance. Only a few cases have been reported in the English literature. Considering the rarity of the condition, we report a case of SOD in a pediatric patient who was followed up over a period of over two years. The clinical, radiographic, and histologic features are presented along with a review of the literature.

Malignant cylindroma of the scalp arising in a setting of multiple cylindromatosis: a case report.

An unusual case of malignant cylindroma of the scalp arising in a 79-year-old white female with multiple cylindromatosis is presented. The tumor apparently arose from a cylindroma and had features of spiradenoma. Multiple cylindromatosis is an uncommon hereditary autosomal dominant disease, which is characterized by multiple skin adnexal tumors like cylindromas and trichoepitheliomas and occasional spiradenomas. Cylindroma is an uncommon benign tumor, which originates from skin appendages and is most commonly found on the scalp and face with a strong predilection for middle-aged and elderly females. Although cylindromas are usually benign neoplasms, carcinoma arising in such neoplasms is rare with only sporadic reports in literature. Her family history was negative for a similar disease. The patient's main concern was painful lesions over her right ear that interfered with wearing of her glasses. The clinical, histological immunohistochemical features, and treatment are presented along with a review of the literature.