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Purpose: Ossifying fibroma (OF) is one of the most frequent of the 3 fibro-osseous lesions of the jaws and has had an inconstant recurrence rate. A definitive surgical protocol has not yet been published. The aim of the present study was to determine the clinical and radiologic features of OF at a tertiary hospital in South Africa, assess its management and recurrence patterns, and develop a surgical protocol., Materials and Methods: A retrospective case-series analysis was performed of all histopathologically diagnosed OF cases from 1976 to 2014. The demographic data, clinical presentation, and radiographic features were analyzed. The management of each case and follow-up data were also documented. A surgical protocol was developed using these findings., Results: A total of 61 cases were included in the present study. Most of the patients were female (63.9%) and younger than 40 years old (73.9%), and only few cases were symptomatic (29.5%). The mandibular posterior region was affected most often (55.5%), and most lesions were radiopaque (49.2%) and had well-defined margins (93.6%). Most cases were managed by surgical curettage (68.2%). After an average follow-up period of 20 months, only 1 case had recurred for a recurrence rate of 6.7%., Conclusions: The lesions in our population were more radiopaque and larger than those in the published data. Surgical curettage is an acceptable management protocol with a low rate of recurrence, and resection should be reserved for aggressive and recurrent lesions., (Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Calcifications have been rarely reported in odontogenic myxoma. We describe here an additional case and review all reported cases. A 45-year-old female patient presented with a gingival swelling around a mobile mandibular left second molar. Radiographic investigation revealed a large multilocular radiolucent lesion of the posterior mandible. Microscopic examination revealed an odontogenic myxoma with numerous newly formed trabeculae of bone or cementum-like material present throughout the specimen, reminiscent of those seen in fibro-osseous lesions of the jaws. After total excision, regular follow-up of the patient showed gradual healing of the surgical defect. To our knowledge, only a few documented cases of odontogenic myxoma with calcifications have been reported in the literature. This histopathologic finding is rare but should not lead to the misdiagnosis of a central odontogenic fibroma, cemento-ossifying fibroma, fibro-osseous lesion, or low-grade osteosarcoma., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Two cases of a rare variant of adenomatoid odontogenic tumor encompassed by a prominent reactive cemento-osseous proliferation are reported. This unique variant of adenomatoid odontogenic tumor has only been seen twice in the authors' collective experience. Literature documenting the histopathologic patterns of adenomatoid odontogenic tumor and the occurrence of other combined lesions other is reviewed and discussed., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Clinical Case: The case concerns a 34 year-old man, who presented with pain in the medial canthus in his left eye, with proptosis and diplopia. The examination showed exophthalmus and a palpable mass at the inner canthus. The computed tomography revealed a lesion, which was confirmed by biopsy to be a ossifying fibroma., Discussion: Ossifying fibroma is a benign fibro-osseous lesion that mostly affects the jaw. Clinical presentation and complications vary according to its location. Clinical examination, imaging, and histopathology are essential for definitive diagnosis. Surgical treatment is multidisciplinary and long-term follow up is needed., (Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Accurate and early diagnosis of benign fibroosseous lesions is important because the treatment and resulting outcomes of each differ. Juvenile ossifying fibromas typically occur in young patients and grow rapidly with a high recurrence rate. Their monostotic nature has previously differentiated these tumors from other fibroosseous lesions. We describe an interesting and extremely rare case of polyostotic juvenile ossifying fibromas in a 14-year-old boy with involvement of the maxilla and mandible. The available literature on juvenile ossifying fibromas is also briefly reviewed. When diagnosing a polyostotic fibroosseous lesion, it is important to not exclude the possibility of juvenile ossifying fibromas because this may warrant a different treatment.

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain differentiation, initially described by Enzinger and colleagues. Until now, nearly 300 such cases have been reported worldwide. The histogenesis of these tumors remains controversial. These tumors show characteristic imaging findings and exhibit a spectrum of histopathologic features, including classical and atypical subtypes. Local recurrences and, occasionally, distant metastases have also been reported. A complete tumor resection forms the preferred treatment modality for these tumors, along with follow-up, as these tumors have an uncertain malignant potential. Lately, certain "molecular signatures" underlying OFMTs have been described that can further aid in reaching an accurate diagnosis for these tumors and unraveling their pathogenesis. This article is a review of the clinical, radiologic, histopathologic, and molecular features of OFMTs.

The management of patients with juvenile ossifying fibroma (JOF) remains controversial. To explore the correlations between different treatments and the patient prognosis, 15 cases of JOF of the jaw were reviewed. Five patients were male and 10 were female. Patient age at the time of disease onset ranged from 7 to 18 years (mean 10.9 years). Nine tumours were located in mandible and six in the maxilla. These cases typically manifested clinically as painless swelling of the jaw (9/15, 60%); 40% (6/15) of the cases were associated with pain, diplopia, stuffy nose, and/or rapid growth. Images of JOF can show a radiolucent, mixed, or ground glass-like appearance. Pathological examinations revealed 10 cases of juvenile trabecular ossifying fibroma (JTOF) and five cases of juvenile psammomatoid ossifying fibroma (JPOF). In terms of the treatment plan, six patients initially received radical surgery; nine patients underwent conservative treatment, among whom six (6/9, 66.7%) had one or more recurrence. At the end of the follow-up period, 12 patients had no evidence of tumour recurrence and three cases were alive with a tumour. In summary, surgeons should develop the surgical plan according to the extent of the lesion, relapse status, growth rate, and family choice, and these patients should be followed up closely., (Copyright © 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

The purpose of the present article was to present a clinical case of an 11-year-old girl with peripheral ossifying fibroma (POF). Additionally, after performing a literature review, we identified clinical information that occurs more frequently in association with POF, such evidence would help professionals in yielding a specific diagnosis and tailor a more specific therapeutic approach with the objective to decrease morbidities' associated with POF. This lesion represents the third most common lesion of all localized reactive hyperplastic lesions. Clinical aspects related to this pathology include the fact that it occurs most frequently in women between the first and second decades of life. It affects anterior maxillary region and interferes with normal functioning of this anatomical structure. After conducting the literature search, we found that it can also be presented in a considerable number of males with pain and hyperemia being the most common clinical manifestations. We found that often clinical cases are presented with incomplete information. It is important that in order to get to a consensus with respect to updates about information related to this lesion, new case series that include complete clinical information, radiographic analysis, and histopathology tests could be presented.

Introduction: Fibro-osseous lesions of the skull and facial bones are benign tumors, but they can be mistaken for malignant tumors due to their clinically aggressive behavior. Cemento-ossifying fibroma (COF) is a benign fibro-osseous lesion characterized by slow growth and fibrous and calcified tissue content. COFs are locally destructive lesions causing deformities in the bones. The recurrence risk is high if they are not completely removed., Case Report: In this case report we describe a giant COF mimicking chondrosarcoma in the oral cavity of a 55-year-old woman causing significant facial deformity and feeding problems., Conclusions: Giant COF occurs rarely in the jaws and given that this lesion has similar imaging and clinical features to several other tumors, the diagnosis is always a challenge for clinicians, radiologists and pathologists.

Terminal osseous dysplasia with pigmentary defects (TODPD) is a rare, X-linked syndrome classically characterized by distal limb anomalies, pigmented skin defects of the face, and recurrent digital fibromas. X-inactivation plays a major role in determining the range of phenotypic expression. Thus, patients can demonstrate a wide spectrum of disease severity, making accurate diagnosis more challenging. Recent studies have identified a FLNA c.5217G>A mutation as the cause of TODPD, allowing for diagnostic genetic testing. We present a case of molecularly confirmed TODPD in a girl with the 47,XXX chromosomal complement and deformities of the hands and feet, craniofacial abnormalities, and discolored, linear facial lesions. Skin biopsy of the patient's facial lesion revealed absent papillary dermal elastic fibers, consistent with anetoderma, which contrasts with the dermal hypoplasia described in the only other such facial biopsy reported in the literature. The finding of absent elastic fibers in the skin lesions suggests that mutated filamin A, in part, exerts its effects through dysregulated elastin biology, which may explain the nature of many connective tissue pleotropic effects in FLNA-related disorders., (© 2015 Wiley Periodicals, Inc.)

Background: The peripheral ossifying fibroma (POF) represents one of the most common lesions of the periodontal tissues that may originate from the gingival soft tissues, the periosteum, or the periodontal ligament., Aim: To investigate the immunohistochemical expression of runt-related transcription factor 2 (Runx-2), bone morphogenetic protein-2 (BMP-2), and cementum attachment protein (CAP) in oxytalan-positive POF, to establish the use of POF as an in vivo model for the study of the periodontal ligament., Materials and Methods: Thirty tumors that presented clinical and histologic features of POF, as well as oxytalan fibers, were included in the study. Immunohistochemical expression of Runx-2, BMP-2, and CAP was evaluated by light microscopy., Results: Runx-2, BMP-2, and CAP were abundantly expressed by POFs; 22 of 30 tumors expressed positive staining for Runx-2, twenty-six tumors for BMP-2, and twenty-five tumors for CAP. The expression of Runx-2 was abundant in POFs where bone was histologically present (P = 0.04) and of BMP-2 in POFs where dystrophic calcifications were present (P = 0.03)., Conclusion: It is suggested that oxytalan-positive POFs, purportedly originating from the periodontal ligament, express molecules that are specific to bone and cementum (Runx-2, BMP-2), or cementum only (CAP). Thus, the cell populations present in the lesion belong to the mineralized-tissue-forming cell lineages, the cementoblastic or osteoblastic lineage., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

A case of juvenile active ossifying fibroma affecting a 27 year-old man will be discussed. The characteristic radiologic and histologic features of the entity will be described.

Ossifying fibroma (OF) and fibrous dysplasia (FD) are benign, intraosseous, proliferative fibro-osseous lesions (PFOLs) characterized by replacement of normal bone by a fibrous matrix with various degrees of mineralization and ossification. Osteomas are benign tumors composed of mature, well-differentiated bone. Clinical, imaging, and histologic features of 15 initially diagnosed benign PFOLs and osteomas of the canine oral cavity were evaluated. Final diagnoses after reevaluation were as follows: OF (3 cases), FD (4 cases), low-grade osteosarcoma (LG-OSA) (3 cases), and osteoma (5 cases). Histology alone often did not result in a definitive diagnosis for PFOL. OF appeared as a well-circumscribed, radiopaque mass with some degree of bone lysis on imaging. Most lesions of FD showed soft tissue opacity with bone lysis and ill-defined margins. Low-grade OSA appeared as a lytic lesion with a mixed opacity and ill-defined margins. Osteomas were characterized by a mineralized, expansile, well-circumscribed lesion. Although histologic features of PFOLs were typically bland, the lesions diagnosed as LG-OSA had some features of malignancy (eg, bone invasion or a higher mitotic index). Treatment varied widely. Of the 10 dogs with benign PFOL or osteoma with known outcome (10/12), 9 showed either complete response (6/10) or stable disease (3/10) after treatment. Of the 2 dogs with LG-OSA with known outcome, 1 showed complete response after curative intent surgery, but 1 patient had recurrence after partial maxillectomy. Definitive diagnosis of mandibular/maxillary PFOL is challenging via histopathologic examination alone, and accurate diagnosis is best achieved through assimilation of clinical, imaging, and histopathologic features., (© The Author(s) 2015.)

Introduction: Ossifying fibromyxoid tumor (OFMT) is rare and may present diagnostic difficulty. We describe 26 subcutaneous examples of OFMT emphasizing differential diagnosis and prognostic features., Methods: Histopathology and follow-up data from archival/consultation cases were reviewed. Prognostic features were assessed according to proposed criteria., Results: Patients (16 female, 10 male) ranged from 26 to 88 years (median 54). The tumors (median 2.3 cm, range 0.8-8.5) involved lower limb (11), trunk (7), head/neck (4), or arm (4). All showed combinations of corded, nested and trabecular patterns in a fibromyxoid stroma. Out of 26 cases 13 had peripheral ossification. Sixteen of 22 cases showed S100 protein expression. Nuclear grade was low (14); intermediate (8) and high (4) while cellularity was low (14); moderate (7) and high (5), with overall good interobserver agreement. Median mitotic rate was 3/50HPF (0-61). Five met criteria for malignant OFMT showing high nuclear grade or high cellularity and mitotic rate >2/50HPF or both. Thirteen OFMTs were atypical. Follow-up (16/26, median 45.5 months, range 8-108) showed that patients with typical OFMT (3) and atypical OFMT (9) remained disease-free. Three malignant examples of OFMT recurred and one metastasized to the lung. No deaths were recorded., Conclusions: Our results validate proposed prognostic classification of OFMT. Dermatopathologists should be aware of this unusual superficial tumor given its potentially aggressive behavior., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

In this article, we focus on the histologic features, differential diagnosis, and potential pitfalls in the diagnosis of epithelioid sarcoma, alveolar soft part sarcoma, clear-cell sarcoma, ossifying fibromyxoid tumor, and malignant extrarenal rhabdoid tumor. Numerous other soft tissue tumors also may have epithelioid variants or epithelioid features. Examples include epithelioid angiosarcoma, epithelioid malignant peripheral nerve sheath tumor, epithelioid gastrointestinal stromal tumor, and perivascular epithelioid cell tumor, among others., (Published by Elsevier Inc.)

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm with uncertain histogenesis. Most cases behave in a clinically benign fashion; however, a small percentage of tumors may locally recur or metastasize. Herein we present a case of a 56-year-old man who presented with an enlarging left groin mass, left inner thigh numbness, burning paresthesia and discomfort in his left groin. The mass sampled by fine- needle aspiration and needle core biopsy. Cytology showed bland-appearing epithelioid cells with round nuclei and fine chromatin, with fragments of fibromyxoid stroma in the background. Immunohistochemical stains performed on the core biopsy showed that the lesional cells were focally positive for S100 protein and negative for desmin, smooth muscle actin, CD34 and cytokeratin AE1/AE3. A benign neoplasm was favored with ossifying fibromyxoid tumor as the main entity in the differential diagnosis. A subsequent resection showed a well-circumscribed 5 cm mass with firm consistency and focal areas of calcifications. Histologically, the tumor had a nodular growth pattern with relatively bland spindle cells containing round to oval nuclei suspended in a variably collagenous to myxoid stroma. Significant ossification and bone formation was also noted. There was no significant atypia, necrosis or increased mitoses. Ossifying fibromyxoid tumors have distinct cytologic features and should be considered in the differential diagnosis of soft tissue tumors with prominent ossification., (© 2015 Wiley Periodicals, Inc.)

Benign fibro-osseous lesions (BFOL) of the craniofacial area are represented by a variety of morphologic processes that are characterized by pathologic ossifications and calcifications in association with a hypercellular fibroblastic marrow element. The current classification includes neoplasms, developmental dysplastic lesions and inflammatory/reactive processes [5]. The final diagnosis depends on-clinical, radiological and pathological features. The clinico-pathologic features of this heterogeneous group of diseases are presented in this article.

Delleman syndrome is a rare neurocutaneous disorder characterized by congenital anomalies affecting the eye, skin, and central nervous system. This disorder was first raised as a distinct syndromic entity by Delleman and Oorthuys in 1981 under the term "oculocerebrocutaneous syndrome" (OCCS). Since then, fewer than 40 cases have been reported. All cases have been sporadic, and no patient with an abnormal karyotype has been reported. Although the etiology is still unknown, several theories have been advanced. Most of the tissues affected by OCCS are neural crest derivatives, with a striking homogeneity of the tissues and regions involved. It is this recurring craniofacial pattern of OCCS that lends support to the hypothesis that the most probable pathogenesis is a neurocristopathy that interferes with craniofacial morphogenesis. Ossifying fibroma is a destructive, potentially deforming, benign tumor that can occur almost anywhere in the facial skeletion. Some cases of ossifying fibroma have occurred in patientswith underlying neurocutaneous syndromes such as neurofibromatosis, Sturge-Weber syndrome, and encephalocraniocutaneous lipomatosis (ECCL). We report a patient with OCCS who presented with an ossifying fibroma. This has not been reported previously and is noteworthy in that it might be part of a wider phenotypic spectrum of the less common manifestations associated with OCCS., (Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Ossifying fibroma of the jaw is a benign fibroosseous tumour. The growth of it is slowly and it is well circumscribed. Occurrence of multiple ossifying fibromas (synchronous) is rare in the jaw, and only a few cases have been documented. The most of these cases were in only maxilla. The fewer cases were reported in both of maxilla and mandible. We report a case of bilateral synchronous ossifying fibromas involving the mandible of a 37 years old male. The importance of our case is that bilaterality and synchronous of the lesions. Our case is the first synchronous mandibler lesion in literature reported.

A peripheral ossifying fibroma (POF) is a type of reactive hyperplasia that is found exclusively on the gingiva. It is identified through histologic evidence of calcifications and ossifications that are seen in the context of a hypercellular fibroblastic stroma. This article presents the case of a POF in a 7-year-old girl, and examines the pediatric considerations for diagnosing and managing this lesion.

We describe a case of superficial low-grade fibromyxoid sarcoma (LGFMS) in a 12-year-old boy, confirmed by the detection of FUS-CREB3L2 fusion transcripts by reverse-transcription polymerase chain reaction and FUS rearrangement with fluorescence in situ hybridization, which had morphological features similar to ossifying fibromyxoid tumor (OFMT), including an almost complete rim of mature, metaplastic bone. LGFMS and OFMT can appear morphologically similar, with bland ovoid cells within a fibrous to myxoid matrix. Both can occur superficially; and whereas MUC4 immunoreactivity is characteristic of LGFMS, this can also be seen in some OFMTs. As the morphological spectrum of LGFMS is wide, we highlight the potential for diagnostic confusion with OFMT, which is clinically pertinent as most OFMTs behave in a benign manner whereas LGFMS is a malignant neoplasm with a propensity for local recurrence and a significant metastatic rate., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Preoperative diagnosis of jaw lesions is not always possible on the basis of clinico-radiological findings alone and needs to be confirmed before attempting any surgical intervention. Fibro-osseous lesions of the jaw comprise a spectrum of diseases which include cement-osseous dysplasia, fibrous dysplasia, and ossifying fibroma. The cytomorphological distinction between these individual entities is difficult. We present a case of maxillary fibro-osseous lesion in an adolescent girl diagnosed and categorized as juvenile ossifying fibroma preoperatively on cytology and confirmed on histopathology. Although aspirates are usually paucicellular in fibro-osseous lesions, certain cytological features if present in cellular cytosmears can offer further categorization and a definitive diagnosis may be possible in light of clinico-radiological correlation., (© 2014 Wiley Periodicals, Inc.)

Importance: Pediatric jaw tumors are a rare clinical entity and are not well addressed in the otolaryngology literature. It is important that otolaryngologists be familiar with the clinical features, management, and outcomes associated with these lesions., Objective: To review the clinical presentation, management, and outcomes of jaw tumors in children treated at a tertiary care academic center., Design, Setting, and Participants: Retrospective medical record review of children 16 years or younger who presented to the departments of Oral-Maxillofacial Surgery and Otorhinolaryngology at the University of Maryland Medical Center between 1992 and 2012 and were diagnosed as having a jaw tumor. A PubMed review of literature from 1992 to 2013 on jaw tumors in children was also conducted., Main Outcomes and Measures: Medical records were reviewed for data on symptoms, physical findings, pathologic diagnosis, intervention, and outcomes., Results: The medical records of 76 patients evaluated for a jaw mass were reviewed, and 20 were found to have a diagnosis of a jaw tumor. The 2 most common pathologic diagnoses were ameloblastoma (n = 5) and juvenile ossifying fibroma (n = 4). Two tumors were malignant, a rhabdomyosarcoma and a teratoma. Thirteen patients presented with evidence of a mass or swelling, 5 patients were asymptomatic with a lesion found on surveillance panoramic radiography, and 1 patient presented with epistaxis and 1 with facial weakness and pain. All tumors excluding a lymphangioma and a rhabdomyosarcoma were managed surgically. Eight patients underwent more than 1 procedure including secondary reconstruction prior to a satisfactory outcome., Conclusions and Relevance: Pediatric jaw tumors are rare lesions most commonly presenting with a swelling or mass. Patients can be asymptomatic with the lesion identified on routine imaging. Certain clinical features such as age, location of tumor, and presence or absence of bone and soft tissue can narrow the differential diagnosis and identify tumors that may be malignant. Incisional biopsy is an important first step. A majority of jaw tumors are benign but require surgical intervention for eradication of disease. Multiple procedures, including reconstruction, may be required for certain lesions prior to cure.

Benign fibro-osseous lesions of the craniofacial skeleton (BFOL) are a variant group of intraosseous disease processes that share similar microscopic features characterized by hypercellular fibroblastic stroma containing various combinations of bone or cementum-like tissue and other calcified structures [1-6]. Whereas some are diagnosable histologically, most require a combined assessment of clinical, microscopic and radiologic features. Some BFOL of the craniofacial complex are unique to that location whereas others are encountered in bones from other regions. Reactive, neoplastic, developmental and dysplastic pathologic processes are included under the rubric of BFOL and treatment varies from disease to disease. This review will discuss the clinical, microscopic and radiologic aspects of the more important types of BFOL of the craniofacial complex with updated information on underlying genetic and molecular pathogenic mechanisms of disease. Four main groups of BFOLs will be addressed.

Tumors in the supraorbital region are most commonly accessed through transcranial approaches, including fronto-orbital, orbitozygomatic, and eyebrow supraorbital keyhole approaches. Purely endoscopic endonasal approaches (EEA) are more challenging to perform because of limitations in access and visualization for lateral extension beyond the midline corridor. The modified hemi-Lothrop procedure, a variation of an extended EEA, allows for binostril access and visualization of the lateral supraorbital region while preserving the contralateral frontal sinus drainage pathway. The operative technique and nuances are illustrated in a rare case of a supraorbital juvenile psammomatoid ossifying fibroma (JPOF) causing symptomatic orbital compression. The key components of the approach consisted of an endoscopic Draf IIB (left frontal sinusotomy) ipsilateral to the tumor, and a superior septectomy for binostril bimanual instrumentation. Excellent visualization, access, and tumor removal of the supraorbital region was achieved with angled endoscopy and curved instrumentation from the contralateral nasal cavity and through the septectomy window ("cross-court" trajectory). The modified hemi-Lothrop procedure with angled endoscopy is a safe and effective alternative route to traditional transcranial approaches to access the supraorbital region. To our knowledge, this is the first case of a supraorbital JPOF that was successfully resected via a purely EEA., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

The most commonly encountered fibro-osseous lesions of the skull bone are fibrous dysplasia and ossifying fibroma. Two cases of a unique "protuberant fibro-osseous lesion of the temporal bone" were first described by Selesnick and colleagues in 1999, and 2 further cases were reported in 2010 under the name "Bullough lesion". We recently found 2 new cases of this rare entity. Two Korean female patients aged 70 and 54 years presented with slow growing postauricular masses without pain or tenderness for 6 and 7 years, respectively. Computed tomography revealed a 2.9 cm calcified mass in the temporal bone of the first patient, and a 5.5 cm enhancing mass with internal cartilaginous matrix in the temporal bone of the second patient. Intramedullary or intracranial extension was not found in either case, and en bloc removals were performed. Microscopically, multiple round to oval osseous islands were scattered throughout the bland fibrous stroma in both cases. The osseous islands varied in size and were lamellar or woven, without osteoblastic rimming, and surrounded by fibroblastic bands. Neither patient has shown evidence of postoperative recurrence for 18 months. The location, histology, and clinical course of these 2 cases were identical to the 4 cases previously reported, although age and sex varied. The lesions were tested for the R201H mutation in the GNAS gene, which is present in fibrous dysplasia. No mutations were found, suggesting a different genetic background for these lesions.

Background: Biopsy is an important diagnostic tool used in the diagnosis of lesions ranging from simple non-neoplastic, tumor-like lesions to malignancies, and is often the only way to diagnose oral lesions and diseases. The gingiva is the most common site for some kind of irritation or low-grade injury, resulting in localized overgrowths that are considered to be reactive and non-neoplastic lesions. This aim of this study is to analyze the frequency and distribution of gingival lesions in the Gujarati population., Materials and Methods: In this retrospective study, gingival biopsies submitted for a period of five years were included. Microscopic slides of all the cases were reviewed by two observers for confirmation of the diagnosis., Results: Among the 106 cases of gingival biopsies, the most frequent category of lesions encountered was the non-neoplastic category, which accounted for 73.58% of the cases. Both benign and malignant neoplasms constituted 26.42% of the cases. Among the non-neoplastic lesions, Pyogenic granuloma was the most frequent lesion (38.46%), followed by fibrous hyperplasia (20.51%), inflammatory hyperplasia (19.23%), and Epulis (8.97%). Neoplasms accounted for 26.42% of the gingival biopsies (92.85% benign and 7.15% malignant). Among the benign neoplastic lesions, Fibroma (30.76%) and Fibrolipoma (26.92%) were the most frequent, followed by peripheral ossifying fibroma (23.08%) and peripheral giant cell granuloma (11%)., Conclusion: It is difficult to compare studies carried out in various countries due to differences in people's attitudes toward oral health and the accessibility of various population groups to biopsy services. Nevertheless, this study has provided some information about the frequency and distribution of biopsied gingival lesions in the Gujarati population over a period of five years.

Juvenile psammomatoid ossifying fibroma (JPOF) is a histologic variant of an ossifying fibroma. While an ossifying fibroma varies in appearance, it consists of fibrocellular tissue and mineralized material. JPOF is a rare benign lesion; however, it can be extended locally and mimic a malignant tumor. It is considered a specific clinicopathologic entity, due to its occurrence in children and adolescents, the presence of psammomas, and its purported tendency for local aggressive growth and recurrence. It commonly affects the maxilla, nasal cavity, paranasal sinuses, and ethmoid region. This article describes a rare, aggressive case of a large JPOF that led to the total destruction of the mandible.

Buschke-Ollendorff syndrome represents an autosomal dominant disorder characterized by connective tissue nevi and osteopoikilosis. Cutaneous lesions may contain either predominantly elastic fibers or predominantly collagen fibers or may show both connective tissue components. The disease results from mutations in LEMD3 (MAN1), which lead to enhanced transforming growth factor-β (TGF-β) signaling and resultant changes in fibroblast function. TGF-β alterations have been implicated in a number of fibrotic disorders, and it is therefore not surprising that a range of cutaneous and skeletal abnormalities have been associated with Buschke-Ollendorff syndrome. Herein, we report a novel association between ossifying fibroma and Buschke-Ollendorff syndrome and discuss how these conditions are likely to be mechanistically linked., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: This study aims at contributing to the definitive diagnosis of ossifying fibroma (OF) based on histomorphological features. The study also aims to determine some demographic features of OF, common sites of occurrence and to determine whether behavior correlates well with the histomorphological variations seen in the lesion., Materials and Methods: A total of 80 patients who were diagnosed either as cementifying fibroma CF, OF and cemento-ossifying fibroma (COF) of the jaws from the files of the Oral Pathology Department of the University of the Witwatersrand Dental School were retrieved and the histology slides of each case were reviewed with the most recent diagnostic criteria for OF and the authors additional criteria. A total of 56 cases that met the set criteria were analyzed., Results: The patients were clustered within the third and fourth decades of life ( n = 39, 69.6%). Majority of the patients were black (83.93%), whereas the rest were whites (12.50%) and Asians (3.57%). There were 17 males (30.4%) and 39 females (69.6%), giving a male to female ratio of 1:2.3. Most of the lesions (70.3%) occurred in the mandible, involving the premolar molar region (56.7%). Scanty fibrous tissues in highly cellular lesions were found in 36 (64.3%) of the cases. There were globular, dystrophic or granular calcifications mixed with irregularly shaped trabeculae of lamellar or cellular woven bone or osteoid were found, in 36 (64.3%) cases., Conclusion: Demographic data, clinicoradiologic features, combined with histopathology will continue to be relevant in the definitive diagnosis of OF and in predicting its behavior. Highly aggressive lesions with shorter duration in people below 15 years were called juvenile OF and treated as such, while OF applies to other conventional ones.

Introduction: The aim of this study was to perform a retrospective study of histopathologic features of a series of cases of pyogenic granuloma (PG), peripheral giant cell lesion (PGCL), and peripheral ossifying fibromas (POF) that constitutes the group called reactional lesions, located in gingiva and alveolar ridge., Study Design: Cases of PG, PGCL, and POF were selected for this study. The morphological analysis of the lesions constituted the following: intensity of inflammatory infiltrate (IF), presence of vascular proliferation (VP), fibroblastic proliferation (FP), areas of ulceration (AU), bacterial colony (BC), presence of mineralization (PM), multinucleated giant cells (MGC), hemosiderin deposition (HD), hemorrhage area (HA)., Results: Of the 288 cases analyzed, 162 (56.3%) were PG, 72 (25%) were PGCL, and 54 (18.8%) were POF. The IF, VP, AU, and BC were more prominent in PG (85.8%, 98.8%, 91.4%, and 46.9%, respectively) and PM in POFs (98.1%). FP was more frequent in POF (98.1%) and PGCL (100%) and MGC in PGCL (100%), although some cases of POF (7.4%) and PG (0.6%) exhibited MGC. HD was more frequent in PGCL (40.3%) and HA in PG (53.1%)., Conclusions: This study demonstrated that IF, VP, AU, BC, and HA are the common features in PG, MGC, FP, and HD are the most common in PGCL, and PM associated with FP are the most common in POF, which can help in the histopathologic differential diagnosis between these lesions. In addition, it may suggest a possible development and maturation of the PG in POF with reduction in the inflammatory component and increase in the fibrous component.

Peripheral ossifying fibromas are rare pathologies characterized by the replacement of bone to fibrous tissue. In this article, we report a 64-year-old female case admitted to our clinic with a mass in the oral cavity for 10 years. On physical examination, a 5x4 cm, well-defined, pedunculated mass was detected in the anterior arch of the right maxilla. Based on the clinical, radiographic and histopathological findings, the mass was diagnosed as an ossifying fibroma.

Peripheral ossifying fibroma is a common solitary gingival overgrowth thought to arise from the gingival corium, periosteum or periodontal ligament. Ossifying fibroma has the propensity to occur in cranial bones, but the peripheral variety shows a close relationship with the periodontal ligament and adjacent alveolar bone. It is more commonly seen in women (predilection of 3:1) and usually at a young age. This report describes an unusual presentation in an elderly man and presents a systematic way of diagnosing and treating peripheral ossifying fibroma., (2014 BMJ Publishing Group Ltd.)

The peripheral odontogenic fibroma is a relatively rare, benign, unencapsulated and gingival mass of fibrous connective tissue, considered to be the extraosseous counterpart of the central odontogenic fibroma. Peripheral odontogenic fibroma was earlier quite commonly confused with peripheral ossifying fibroma, until WHO classification (1992), classified it under odontogenic tumours. Peripheral odontogenic fibroma is seen so infrequently that little is known about this neoplasm; the largest series of cases have been that of Farman who found five cases in the literature and added another 10 cases. Through this case report we try to clarify the features of this condition and attempt to clear its distinction with other commonly occurring conditions., (2014 BMJ Publishing Group Ltd.)

Benign fibro-osseous lesion (BFOL) is a distinct group of jaw entities composed of fibrocellular tissue and mineralized materials. In this study, we examined the epidemiological, clinical, and pathological features of patients with BFOL. Records and microslides of 207 BFOLs submitted to pathology service were retrospectively reviewed. Overall, fibrous dysplasia (FD) was the most prevalent (36.7%), followed by ossifying fibroma (OF; 32.4%), osseous dysplasia (OD; 24.6%), and juvenile ossifying fibroma (JOF; 6.3%). Female predilection was noted. FD and JOF were common in maxilla, whereas most OF and OD affected the mandible. Most patients with FD and OF presented with painless swelling, while patients with OD were symptomless. The majority of FD specimens showed woven bone, while a mixture of woven bone and cementum-like materials was often noted in OF and OD. Our data show variations in the clinicopathologic features of BFOLs. A thorough examination of all aspects of BFOL patients is imperative for accurate diagnosis., (© The Author(s) 2013.)

Introduction: Ossifying fibroma (OF) is a rare benign fibro-osseous tumor, mainly located in the head and neck region. Most often, it affects the mandible but rare involvement of paranasal sinuses has been reported, associated with more locally aggressive behavior., Case Report: We report the case of an 8-year-old boy with OF of the middle turbinate, revealed by ethmoiditis. Total resection was performed on an endoscopic approach. The patient was free of clinical or radiological recurrence at 3 years' follow-up. This was the youngest patient with OF of the middle turbinate so far reported in the international literature., Discussion: Presumptive diagnosis is established by clinical examination and CT scan (location, oval-shaped mass, heterogeneous tumor with a thin bony rim). Definitive diagnosis is founded on histological examination (psammomatous bodies, osteoblastic rim, trabecular bone). Treatment in paranasal sinus OF is surgical, preferentially on an endoscopic approach. Resection should be as complete as possible to minimize risk of recurrence, especially in sinonasal locations, known to be more aggressive. Ethmoiditis in an unusual age-range should suggest tumoral etiology., (Copyright © 2013. Published by Elsevier Masson SAS.)

Ossifying fibromyxoid tumors are rare soft-tissue neoplasms, usually with an indolent course. However, atypical/malignant variants have been described, showing either local recurrence after complete excision or metastasis to the lungs, mediastinum, adrenals, or soft tissue. We report the case of an ossifying fibromyxoid tumor of the left ankle that metastasized to the lung and thyroid gland 12 years after the initial diagnosis and surgical treatment. To our knowledge, this is the first reported case of this neoplasm metastasizing to the thyroid gland.

Juvenile ossifying fibroma is a rare, benign tumor that may present in the sinonasal area but has never been described arising from the middle turbinate. The lesion's locally aggressive behavior and close proximity to orbit and anterior skull base create a significant challenge for the patient and practitioner. This is a case presentation of a 7-year-old female with an invasive recurrent juvenile ossifying fibroma arising from the middle turbinate.

Background: The clinicopathologic characteristics of multiple ossifying fibroma (OF) are unclear due to the condition's rarity, making diagnosis challenging. Sporadic multiple OFs must be distinguished from hyperparathyroidism-jaw tumour syndrome (HPT-JT) related OF and other fibro-osseous lesions., Methods: Multiple OF cases were identified from ossifying fibroma cases. Clinical data including age, sex, anatomic site, radiographic features, clinical impression, treatment and available follow-up data as well as serum calcium, phosphorus, and parathyroid hormone (PTH) were recorded. GNAS and HRPT2 genetic mutations were examined in the two present cases. Case reports of sporadic multiple ossifying fibroma and HPT-JT-related OF were also reviewed., Results: The two present cases were confirmed as sporadic multiple OF, with no genetic GNAS and HRPT2 mutations found. The incidence of sporadic multiple ossifying fibroma was 2.0% (2/102). The total 18 sporadic multiform OF cases were characterized as followed: 13 (72.2%) female; 5 (27.8%) male; mean age 28.6 years; 2/16 (11.1%) cases only in the mandible; 4/18 (22.2%) cases only in the maxilla; and 12/18 (66.7%) cases in both the maxilla and mandible. Radiographically, the lesions were radiolucent in 5/18 (27.8%) cases and mixed density in 13/18 (72.2%) cases. Along with 24 cases of HPT-JT related OF were reviewed, sixteen (66.7%) patients were diagnosed with a single lesion, and 8 patients (33.3%) were diagnosed with multiple jaw lesions., Conclusions: Sporadic multiple OFs are very rare, but must be distinguished from HPT-JT related OF. We strongly recommend that patients diagnosed with multiple ossifying fibromas receive serum PTH testing and mutation screening of HRPT2., Virtual Slides: http://www.diagnosticpathology.diagnomx.eu/vs/1194507146115753.

Background: The group of fibro-osseous lesions from the maxillofacial region is very heterogeneous, but what they all have in common is the substitution of normal bone by fibroblasts with the consequent formation of collagen fibers, as well as substitution by different types of mineralized tissues which may be similar to bone or cement. Within this group of lesions, the juvenile ossifying fibroma is found, considered a rare and recurrent benign fibro-osseous neoplasia. The term juvenile ossifying fibroma has been used in the literature to describe two histopathological variations of conventional ossifying fibroma. These variations are trabecular juvenile ossifying fibroma and psammomatoid juvenile ossifying fibroma. Psammomatoid juvenile ossifying fibroma is an uncommon bone-forming neoplasm with aggressive local growth. Diagnostic of this lesion occurs after the correlation of clinical, imaging, and histopathological findings. Proposed treatments range from enucleation and curettage to resection of the tumor., Objectives: The present article has as its objectives to report an uncommon case of a 4-year-old male patient treated by conservative approach and revise the literature on juvenile ossifying fibroma., Conclusions: Psammomatoid juvenile ossifying fibroma, for its being very uncommon, warrants further investigation in order to establish the best treatment, principally in children, with a view to minimizing mutilating treatments. In the case examined, a conservative treatment was chosen, where the surgeon established curette and cryotherapy, and the reintegration of the child in his social environment, without relapse during the first year of therapy.