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The aim of this study is to report an original case series of synchronous jawbone diseases. Data of patients seen over 13 years were extracted from the files of three Oral Radiology and Pathology diagnostic centers in Brazil. The clinical, radiographic, and laboratory characteristics were tabulated and analyzed by the authors; the patients were described according to lesion type. Seventy-two synchronous jawbone diseases were included in this study. Florid osseous dysplasia, Gorlin-Goltz syndrome, and cherubism were the most frequent disorders reported in this case series. In addition, the posterior mandible area was the main site of manifestation. Florid osseous dysplasia and Gorlin-Goltz syndrome represented two-thirds of our samples. With the utilization of adequate demographic, clinical, and radiologic information, it is possible to diagnose most of the synchronous lesions of jawbones. Sometimes, however, we need complementary exams, such as histopathologic and biochemical analysis or dosing of calcium, phosphorus, and alkaline phosphatase.

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Abstract Introduction: Fibrous dysplasia is a benign disorder, in which normal bone is replaced by fibrosis and immature bone trabeculae, showing a similar distribution between the genders, and being more prevalent in the earlier decades of life. Fibrous dysplasia of the temporal bone is a rare condition, and there is no consensus as to whether it is more common in monostotic or polyostotic forms. External auditory meatus stenosis and conductive dysacusis are the most common manifestations, with cholesteatoma being a common complication, whereas the involvement of the otic capsule is an unusual one. Surgical treatment is indicated to control pain or dysacusis, otorrhea, cholesteatoma, and deformity. Objectives: To describe the clinical experience of a tertiary referral hospital with cases of fibrous dysplasia of the temporal bone. Methods: Sampling of patients diagnosed with fibrous dysplasia of the temporal bone, confirmed by tomography, treated at the pediatric otology and otorhinolaryngology outpatient clinics, between 2015 and 2018. The assessed variables were age, gender, laterality, external auditory meatus stenosis, deformity, hearing loss, presence of secondary cholesteatoma of the external auditory meatus, lesion extension and management. Results: Five patients were included, four females and one male, with age ranging from 13 to 34 years. Three had the polyostotic form and two the monostotic form of fibrous dysplasia of the temporal bone. Four patients had local deformity and external auditory meatus stenosis, two of which progressed to cholesteatoma. All patients showed some degree of hearing impairment. All had preserved otic capsule at the tomography. Two patients are currently undergoing clinical observation; two were submitted to tympanomastoidectomy due to secondary cholesteatoma; one was submitted to lesion resection, aiming to control the dysacusis progression. Conclusion: Five cases of fibrous dysplasia of the temporal bone were described, a rare disorder of which the otologist should be aware.

Resumo Introdução: Displasia fibrosa é uma desordem benigna, na qual o osso é substituído por fibrose e trabeculado ósseo imaturo, com distribuição semelhante entre sexos, mais comum nas primeiras décadas de vida. O acometimento do osso temporal pela displasia fibrosa é raro, não há consenso se é mais comum nas formas monostóticas ou poliostóticas. Estenose do meato acústico externo e disacusia condutiva são as manifestações mais comuns. Colesteatoma é também uma complicação comum e o acometimento da cápsula ótica incomum. O tratamento cirúrgico está indicado para controle de dor ou disacusia, otorreia, colesteatoma, deformidade. Objetivos: Descrever a experiência clínica de hospital terciário de referência com casos de displasia fibrosa do osso temporal. Método: Amostragem dos pacientes com diagnóstico de displasia fibrosa do osso temporal, confirmado pela tomografia, atendidos nos ambulatórios de otologia e otorrinolaringologia pediátrica, entre 2015 e 2018. As variáveis avaliadas foram idade, gênero, lateralidade, estenose do meato acústico externo, deformidade, perda auditiva, presença de colesteatoma secundário de meato acústico externo, extensão da lesão e conduta adotada. Resultados: Foram incluídos cinco pacientes, quatro do sexo feminino e um masculino, de 13-34 anos. Três apresentaram a forma poliostótica da displasia fibrosa do osso temporal e dois a forma monostótica. Quatro apresentaram deformidade local e estenose do meato acústico externo, dois desses evoluíram com colesteatoma. Todos manifestaram algum grau de comprometimento auditivo. Todos apresentaram cápsula ótica preservada na tomografia. Duas pacientes estão em observação clínica; duas foram submetidas a timpanomastoidectomia devido a colesteatoma secundário; um foi submetido a ressecção da lesão para controle de progressão da disacusia. Conclusão: Foram descritos cinco casos de displasia fibrosa do osso temporal, desordem rara para a qual o otologista deve estar atento.

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INTRODUCTION: Fibrous dysplasia is a benign disorder, in which normal bone is replaced by fibrosis and immature bone trabeculae, showing a similar distribution between the genders, and being more prevalent in the earlier decades of life. Fibrous dysplasia of the temporal bone is a rare condition, and there is no consensus as to whether it is more common in monostotic or polyostotic forms. External auditory meatus stenosis and conductive dysacusis are the most common manifestations, with cholesteatoma being a common complication, whereas the involvement of the otic capsule is an unusual one. Surgical treatment is indicated to control pain or dysacusis, otorrhea, cholesteatoma, and deformity. OBJECTIVES: To describe the clinical experience of a tertiary referral hospital with cases of fibrous dysplasia of the temporal bone. METHODS: Sampling of patients diagnosed with fibrous dysplasia of the temporal bone, confirmed by tomography, treated at the pediatric otology and otorhinolaryngology outpatient clinics, between 2015 and 2018. The assessed variables were age, gender, laterality, external auditory meatus stenosis, deformity, hearing loss, presence of secondary cholesteatoma of the external auditory meatus, lesion extension and management. RESULTS: Five patients were included, four females and one male, with age ranging from 13 to 34 years. Three had the polyostotic form and two the monostotic form of fibrous dysplasia of the temporal bone. Four patients had local deformity and external auditory meatus stenosis, two of which progressed to cholesteatoma. All patients showed some degree of hearing impairment. All had preserved otic capsule at the tomography. Two patients are currently undergoing clinical observation; two were submitted to tympanomastoidectomy due to secondary cholesteatoma; one was submitted to lesion resection, aiming to control the dysacusis progression. CONCLUSION: Five cases of fibrous dysplasia of the temporal bone were described, a rare disorder of which the otologist should be aware.

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La displasia fibrosa (DF) es una enfermedad infrecuente del hueso, no hereditaria, producida por una mutación activadora del gen GNAS, responsable de codificar la unidad a-estimuladora de la proteína G (Gsa). La presentación clínica de la enfermedad es muy variada, pues adopta desde formas asintomáticas hasta otras marcadamente sintomáticas. En los últimos años, el análisis exhaustivo de bases de datos de pacientes con DF ha permitido conocer más sobre su historia natural. En este artículo se revisa la información actualmente disponible sobre algunos aspectos que ayudarán al mejor enfoque clínico del paciente, como son: la utilidad clínica de los marcadores óseos, los factores pronósticos para el desarrollo de fracturas, la DF como condición predisponente para el desarrollo de tumores específicos, nuevas perspectivas sobre la fisiopatología del dolor óseo y nuevas estrategias terapéuticas. Un mayor conocimiento sobre la historia natural de esta enfermedad finalmente redundará en la mejor calidad de vida de los pacientes con DF. (AU)

Fibrous dysplasia (FD) is an infrequent, non-hereditary bone disease caused by a somatic mutation of the GNAS gene, responsible for encoding the a-subunit of the G-protein (Gsa). The clinical presentation of the disease varies greatly, with some patients being asymptomatic and others markedly symptomatic. The exhaustive analysis of the database from patients with FD has allowed to learn more about the natural history of this disease. This article reviews the current information available on the clinical utility of bone markers, the prognostic factors for the occurrence of fractures, the evidence supporting as a predisposing condition for the development of specific tumors, new perspectives on the pathophysiology of bone pain, and emerging therapeutic strategies. A greater understanding of the natural history of this disease will allow to make better medical decisions, which will ultimately contribute to improve FD patients' quality of life. (AU)

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Differential diagnosis among fibrous dysplasias, cemento-ossifying fibromas and cemento-osseous dysplasias is difficult, since there is considerable overlap of histologic features, but also extremely important, since they differ greatly in etiology, clinical behaviour, prognosis and terapeuthic approach. There is no data about the use of immunohistochemistry, a viable and accessible technique, for this purpose. The objective of this study was to investigate, comparatively, the immunohistochemical expression of major non-collagenous proteins (osteonectin [ON], osteopontin [OP], bone sialoprotein [BSP] and osteocalcin [OC]) of mineralized tissue extracellular matrix in 22 cases of fibrous dysplasias, 16 of cemento-ossifying fibromas and 16 of cemento-osseous dysplasias. ON maintained the same expression profile in all cases; the staining for OP was negative in fusiform cells producing cementoid globules and weak, as well as heterogeneous, in high mineralized matrixes; there was negativity for BSP in cementoid globules and in the fusiform cells that produce them, differently from the strong positive expression found in the majority of bone trabeculae and their peripheral cuboidal osteoblasts; and finally, the immuno-reactivity for OC was weak, except in cuboidal osteoblasts and osteocytes. We can conclude that the nature of mineralized structure and the cellular phenotype are much more responsible for variability in immunohistochemical profile than the type of lesion (fibrous dysplasias, cemento-ossifying fibromas and cemento-osseous dysplasias) which makes difficult, at least for a while, the use of these proteins with diagnosis purpose.

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Introdução: a displasia cemento-óssea periapical (DCOP) é uma lesão idiopática benigna mais prevalente na região de incisivos centrais inferiores, em mulheres negras, na faixa etária dos 30 aos 50 anos. Apresenta características radiográficas que podem levar o cirurgião-dentista a um diagnóstico e plano de tratamento equivocados, por ser confundida com periapicopatias. Objetivo: o objetivo do presente artigo foi, por meio de uma revisão de literatura, descrever essa patologia. Métodos: essa revisão foi feita por meio de buscas em duas das principais bases de dados mundiais: PubMed e SciELO. Para isso, foram usados os descritores "periapical cementoosseus dysplasia" e "displasia cemento-óssea periapical", com o objetivo de se avaliar o conteúdo sobre essa temática na literatura atual. Resultados: foram coletados 24 artigos científicos que obedeciam aos seguintes critérios de inclusão: ser uma revisão de literatura ou caso clínico; escrito em língua portuguesa ou inglesa, nos períodos de 1989 a 2016; contemplando a etiologia, características clínicas e radiográficas, diagnóstico, plano de tratamento e prognóstico referentes à displasia cemento-óssea periapical. Conclusão: é importante para o profissional reconhecer os aspectos relevantes da DCOP, a fim de elucidar o diagnóstico diferencial e tratamento e, assim, evitar procedimentos iatrogênicos, tais como terapias endodônticas desnecessárias.

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INTRODUCTION: The florid cemento-osseous dysplasia is an uncommon condition nonneoplastic, of unknown cause with higher prevalence in melanodermic women, limited the maxillary bones, is characterized by the presence of dispersed and diffuse radiopaque calcifications, constituted of bone and dense cemento; however, when the bone is infected it induces the suppuration and formation of osseous sequestra, thus resulting in an osteomyelitis frame. CLINICAL REPORT: The patient was attended in a Dental Specialties Center in the state of Alagoas, Brazil, presenting on clinical examination edema and extra oral fistula with pus drainage in hemiface submandibular of the right side. Radiographically it was possible to observe area of sclerosis and osseous sequestra involving the right side region of the mandible body, and it increases zones of the bone density. In association with clinical data and complementary diagnosis examinations, the option of treatment adopted was the complete removal of the bone fragment, followed by adaptation and plate fixation and titanium screws to reduce the risk of mandibular fracture. OBJECTIVE: The aim of the present paper was to relate a clinical patient of florid cemento-osseous dysplasia simultaneous the chronic suppurative osteomyelitis, highlighting their clinical, radiographic, and histological characteristics, as well as their diagnosis and treatment.

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As lesões fibro-ósseas benignas (LFOBs) são condições raras caracterizadas pelo desenvolvimento de doenças neoplásicas, reativas ou displásicas, nas quais o osso normal é substituído por tecido conjuntivo composto por colágeno, fibroblastos e diferentes materiais mineralizados semelhantes ao osso ou cemento. Os principais tipos são: displasia fibrosa (DF): monostótica (DFM) ou poliostótica (DFP); displasia óssea (DO): periapical (DOP), focal (DOFoc) ou florida (DOFlor); e o fibroma ossificante (FO): convencional (FOC) ou juvenil (FOJ). O objetivo deste estudo foi descrever o perfil epidemiológico das LFOBs dos maxilares diagnosticados no serviço de referência em Patologia, Estomatologia e Radiologia da Faculdade de Odontologia da Universidade Federal de Minas Gerais e comparar com os resultados encontrados em outros estudos. Os dados foram obtidos dos arquivos dos serviços clínico e laboratorial, no período de 26 anos (1990-2015). Informações de gênero, idade, raça dos pacientes e principais características clínicas e radiográficas da lesão foram coletadas. Foi realizada a análise estatística descritiva das variáveis e teste de associação entre os grupos de lesões e o gênero, faixa etária, aumento de volume e osteomielite. Entre todos os diagnósticos do período avaliado (27998 prontuários), as LFOBs representaram 1,36% (n=383). As DOs (n=187, 48,8%) foram as mais frequentes, seguidas pela DF (n=103, 26,9%) e FO (n=93, 24,3%). Quanto aos subtipos de LFOBs, as mais frequentes foram a DOFlor e a DFM, ambas com 101 casos (26,4%). As mulheres foram a maioria no estudo, com frequência de 82,0% (n=314), assim como os pacientes não negros (59,0%, n=226). Entre todas as LFOBs a média de idade foi de 38,52 ± 17,54, acometendo mais a 4ª e 5ª décadas. As características radiográficas mais comuns foram imagens mistas: radiopaca e radiolúcida (51,7%). A mandíbula foi a região mais acometida (n=247, 64,5%), exceto para DF que envolveu mais a maxila (n=68, 66,0%). A DF foi observada mais entre os homens que em mulheres, assim como entre os pacientes mais jovens. DOs foram mais comuns em mulheres e em pacientes mais velhos. Enquanto que no FO não foi observada diferença significativa entre o gênero. A associação entre a presença de osteomielite e DO foi estatisticamente significante (p=0,0001). O aumento de volume foi significante na DF e FO (p=0,0001). A frequência de LFOB é semelhante à previamente reportada na literatura no mesmo país, mas difere dos dados observados em outras populações. É importante considerar a correlação de dados clínicos, radiográficos e histopatológico para o diagnóstico definitivo de LFOBs. Estudos epidemiólogicos podem orientar o clínico sobre a perfil do paciente acometido por uma doença. Nossos resultados são importantes para auxiliar no diagnóstico e manejo clínico das lesões fibro-ósseas benignas

Benign fibro-osseous lesions (BFOLs) are rare conditions characterized by the development of neoplastic, reactive or dysplastic disease, in which is observed replacement of the normal bone by collagen, fibroblasts and different mineralized materials like bone and cementum. The main types are: fibrous dysplasia (FD): monostotic (MFD) or polyostotic (PFD); osseous dysplasia (OD): periapical (POD), focal (FocOD) or florid (FlorOD); and ossifying fibroma (OF): conventional (COF) or juvenile (JOF). The aim of this study was to describe the epidemiological profile of BFOLs of the jaws diagnosed in the reference service in oral pathology at the Universidade Federal of Minas Gerais and compare it with the results found in other studies. Data were obtained from the archives of clinical and laboratory services in the period of 26 years (1990-2015). Gender, age, race and main clinical and radiographic characteristics of the lesion were collected. Descriptive statistical analysis of each variable and associations test between groups of lesions and gender, age, swelling and osteomyelitis were made. Among all diagnoses in the period (n=27998 records), the BFOLs represented 1.36% (n=383). The ODs (n=187, 48.8%) were the most frequently diagnosed, followed by FD (n=103, 26.9%) and OF (n= 93, 24.3%). Regarding the subtypes, the most common were FlorOD and MFD, both with 101 cases. Women were the majority in the study, with frequency of 82.0% (n=314), as well as non-black patients with (n=226, 59.0%). Among all LFOBs the average age was 38.52 ± 17.54, affecting more the 4th and 5th decades. The most common radiographic features were mixed images: radiopaque and radiolucent (51.7%). The mandible was the most affected region (n=247, 64.5%), except for DF involving more maxilla (n=68, 66.0%). The DF was observed more in men than in women, as well as among younger patients. DOs were more common in women and older patients. The FO was no significant difference between genders. The association between the presence of osteomyelitis and OD was statistically significant (p=0.0001). The swelling was significant in FD and FO (p=0.0001). The frequency of LFOB is similar to that previously reported in the literature in the same country, but differs from data observed in other populations. It is important to consider the correlation of clinical, radiographic and histopathologic for definitive diagnosis of LFOBs. Epidemiological studies can guide the clinician on the patient's profile affected by a disease. Our results are important to assist in the diagnosis and clinical management of benign fibro-osseous lesions

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La displasia fibrosa es una lesión congénita, lentamente progresiva que puede provocar graves alteraciones morfológicas y funcionales, y estar sujeta a complicaciones de tipo infeccioso. En este reporte de caso se presenta a un paciente masculino de 8 años de edad diagnosticado con una displasia fibrosa madura luego de un hallazgo incidental durante un examen de rutina, el paciente durante el curso de los últimos cinco años ha presentado osteomielitis a repetición en el sitio de biopsia y de exfoliación dentaría, el cuadro clínico se ha tratado mediante curetajes y aseos quirúrgicos y con la indicación antibiótica de clindamicina sin resultados positivos. Se concluye que el manejo de las displasias fibrosas maduras puede ser difícil una vez que se ha instalado un proceso infeccioso crónico sin poder dar de alta al paciente, manteniéndolo permanentemente en control. (AU)

Fibrous dysplasia is a slowly progressive congenital lesion that can cause serious morphological and functional alterations , and complications of infectious type . This case report presents a 8 years old male patient diagnosed with a mature fibrous dysplasia after an incidental finding during a routine examination, the patient during the course of the last five years has been presented recurrent osteomyelitis episodes in the biopsy site and temporal tooth during exfoliation, it has been treated by surgical curettage and clindamycin with no positive results. We conclude that the management of mature fibrous dysplasia can be difficult once a chronic infectious process has been installed without being able to discharge the patient, constantly keeping it in control.(AU)

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Florid cemento-osseous dysplasia (FCOD) belongs to the group of fibro-osseous lesions in which normal bone is replaced by fibrous connective tissue and calcified cementum tissue of the avascular type. Among the various types of fibro-osseous lesions, FCOD is one of the most commonly encountered diseases in clinical practice and may involve 3 or 4 of the quadrants. FCOD is located in the periapical regions of teeth, and the lesions are predominantly radiolucent (osteolytic phase), become mixed over time (cementoblast phase), and ultimately become radiopaque (osteogenic phase) with a thin radiolucent peripheral halo. The characteristics of FCOD in the initial stages are similar to those of periapical lesions of inflammatory origin, which may lead to misdiagnosis. A 38-year-old woman sought dental care because of complaints of pain on the right side of her face. A clinical examination revealed no marked alterations; a panoramic radiograph was therefore requested and revealed the presence of radiolucent lesions associated with the periapical regions of some of the lower teeth. Thus, the professional referred the patient for endodontic treatment of the associated teeth with the justification that the lesions were of endodontic origin. However, the endodontist found that the teeth responded positively to a sensitivity test. The initial diagnosis could have resulted in unnecessary root canal treatment, but after careful clinical, radiographic, and tomographic assessments by different professionals, FCOD was diagnosed, conservatively treated, and regularly monitored. It is important that dentists have a basic knowledge of the various injuries that affect the jaw bones to prevent errors in diagnosis and treatment and to promote oral health.

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El tumor fibromixoma lipoesclerosante óseo (TFMLE) fue descrito inicialmente por Ragsdale en 1986, como una lesión fibroósea polimórfica de hueso con una mezcla de elementos histológicos incluyendo lipoma, fibroxantoma, mixoma, mixofibroma, necrosis grasa, osificación isquémica, áreas de displasia fibrosa y rara vez cartílago o cambios quísticos. La localización más frecuentemente descrita es en la región intertrocantérica del fémur. Es un hallazgo en pacientes asintomáticos o bien estar asociado a fractura. Radiológicamente es una lesión lítica, geográfica, de margen bien definido y habitualmente esclerótico, en algunos casos puede observarse mineralización en el interior de la lesión o un cierto grado de expansión en el contorno. Se ha descrito la estrecha relación del TFMLE con la displasia fibrosa por las características histológicas y la presencia de una mutación Gsα, otra hipótesis de la etiología de la lesión incluye la reacción de la displasia fibrosa a la fatiga por estrés.

The bone liposclerosing myxofibrous tumor (LSMFT) was initially described by Ragsdale in 1986 as a polymorphic fibroosseous bone lesion with a mix of histologic elements that include lipoma, fibroxanthoma, myxoma, myxofibroma, fat necrosis, ischemic ossification, areas of fibrous dysplasia, and infrequent presence of cartilage or cystic changes. The most frequently reported location is the intertrochanteric area of the femur. Radiologically it is a lytic, geographic lesion, with well-defined margins and usually sclerotic. In some cases findings include mineralization inside the lesion or a certain degree of expansion to the contour. The close relationship between LSMFT and fibrous dysplasia has been described based on the histologic characteristics and the presence of the Gsα mutation. Another hypothesis of the etiology of the lesion is the reaction of fibrous dysplasia to stress.

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The bone liposclerosing myxofibrous tumor (LSMFT) was initially described by Ragsdale in 1986 as a polymorphic fibroosseous bone lesion with a mix of histologic elements that include lipoma, fibroxanthoma, myxoma, myxofibroma, fat necrosis, ischemic ossification, areas of fibrous dysplasia, and infrequent presence of cartilage or cystic changes. The most frequently reported location is the intertrochanteric area of the femur. Radiologically it is a lytic, geographic lesion, with well-defined margins and usually sclerotic. In some cases findings include mineralization inside the lesion or a certain degree of expansion to the contour. The close relationship between LSMFT and fibrous dysplasia has been described based on the histologic characteristics and the presence of the Gsα mutation. Another hypothesis of the etiology of the lesion is the reaction of fibrous dysplasia to stress.

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Central giant cell granuloma (CGCG) of the jaws represents a localized and benign neoplastic lesion sometimes characterized by aggressive osteolytic proliferation. The World Health Organization defines it as an intraosseous lesion composed of cellular and dense connective tissues that contain multiple hemorrhagic foci, an aggregation of multinucleated giant cells, and occasional bone tissue trabeculae. The origin of this lesion is uncertain; however, factors such as local trauma, inflammation, intraosseous hemorrhage, and genetic abnormalities have been identified as possible causes. CGCG generally affects those younger than 30 years and occurs more frequently in women (2:1). This lesion corresponds to approximately 7% of all benign tumors of the jaws, with prevalence in the anterior region of the jaw. Aggressive lesions are characterized by symptoms, such as pain, numbness, rapid growth, cortical perforation, root resorption, and a high recurrence rate after curettage. In contrast, nonaggressive CGCGs have a slow rate of growth, may contain sparse trabeculation, and are less likely to move teeth or cause root resorption or cortical perforation. Nonaggressive CGCGs are generally asymptomatic lesions and thus are frequently found on routine dental radiographs. Radiographically, the 2 forms of CGCG present as radiolucent, expansive, unilocular or multilocular masses with well-defined margins. The histopathology of CGCG is characterized by multinucleated giant cells, surrounded by round, oval, and spindle-shaped mononuclear cells, scattered in dense connective tissue with hemorrhagic and abundant vascularization foci. The final diagnosis is determined by histopathologic analysis of the biopsy specimen. The preferred treatment for CGCG consists of excisional biopsy, curettage with a safety margin, and partial or total resection of the affected bone. Conservative treatments include local injections of steroids, calcitonin, and antiangiogenic therapy. Drug treatment using antibiotics, painkillers, and corticosteroids and clinical and radiographic monitoring are necessary for approximately 10 days after surgery. There are only a few cases of spontaneous CGCG regression described in the literature; therefore, a detailed case report of CGCG regression in a 12-year-old boy with a 4-year follow-up is presented and compared with previous studies.

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Chondrosarcoma (CHS) is a malignant neoplasm characterized by the formation of cartilaginous matrix by neoplastic cells, with a high propensity for local recurrences. Head and neck CHS is rare, accounting for less than 12% of all cases of CHS, usually affecting the maxilla. The majority of affected patients are in the fourth decade of life, with a slight predilection for male patients. A painless swelling is commonly the most frequent complaint. Surgery with wide en-bloc resection is the preferred treatment for CHS; radiotherapy and chemotherapy are usually palliative options. Owing to its rarity, there are few clinical series evaluating the biological behaviour of head and neck CHS. The aim of this study is to analyse the clinicopathological characteristics of head and neck CHS by reporting 3 new cases of this neoplasia affecting the jaw bones and reviewing the clinical series previously published in the English literature.

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A displasia fibrosa é uma alteração de desenvolvimento caracterizada pela substituição do osso normal por tecido conjuntivo denso e trabéculas ósseas imaturas, geralmente encontrada em adolescentes e adultos jovens. Uma alteração genética que envolve a proteína Gs-alfa parece ser a base do processo. A exata incidência e prevalência são difíceis de estabelecer, mas as lesões representam cerca de 5% a 7% dos tumores ósseos benignos. Nos ossos craniofaciais tem predileção pela maxila, podendo causar deformidade grave e assimetria, afetando igualmente ambos os sexos. Radiograficamente, pode apresentar diferentes padrões de imagem dependendo do grau de mineralização e maturação da lesão. .A avaliação da displasia fibrosa nas radiografias da região craniofacial pode ser difícil por causa das aparências variáveis e das estruturas que se sobrepõem, de modo que a tomografia computadorizada é um recurso relevante para o seu correto diagnóstico e planejamento de tratamento. O objetivo deste estudo foi caracterizar a displasia fibrosa através da análise da lacunaridade, um método multiescala para descrever padrões de dispersão espacial. Foram avaliados 10 pacientes (6 homens e 4 mulheres) comprometendo a maxila em sua grande maioria.

Para a análise da lacunaridade, empregou-se cortes tomográficos axiais e coronais e, posteriormente, selecionou-se as regiões de interesse das áreas displásicas e do osso normal contralateral por meio do software MATLAB®. Após testes e análises estatísticas, concluiu-se que os cortes coronais, com ampliação de 3x do seu tamanho original, mostraram superioridade em relação aos axiais e, que a lacunaridade foi menor nas áreas da região displásica em relação ao osso normal, ou seja, a primeira apresentou uma maior homogeneidade de textura que a segunda. Mediante isso, pela técnica da validação cruzada leave-one-out é possível separar os grupos com uma alta acurácia (94,75%) concluindo-se que a lacunaridade é um método de análise de imagens contributivo na caracterização da displasia fibrosa.

Fibrous dysplasia is an alteration of development characterized by replacing normal bone for dense connective tissue and immature trabecular bones, typically found in teenagers and young adults. Genetic modification which involves alpha-Gs protein appears to be the basis of the process. The exact incidence and prevalence are difficult to be established, but injuries represent about 5% to 7% of benign bone tumors. On the craniofacial bones, the tumors have a predilection for the maxilla and often can cause severe deformity and asymmetry affecting both sexes equally. Radiographically, it may have different patterns depending on the image degree of mineralization and maturation of the lesion. The evaluation of radiographs of fibrous dysplasia in the craniofacial region can be difficult because of the different appearances and structures that overlaps, however, CT is an important resource for proper diagnosis and treatment planning.

The aim of this study was to characterize the fibrous dysplasia by analyzing the lacunarity which is a multiscale method to describe patterns of spatial dispersion. We evaluated 10 patients (6 males and 4 females) and the maxillary was the most affected area. To the lacunarity analysis, we used an axial and coronal view and then were selected the regions of interest in the areas of dysplastic and contralateral normal bone by means of MATLAB® software. After tests and statistical analysis can be conclued that the coronal magnification 3x its original size showed superiority compared to thrust, and that the lacunarity was lower in the areas of dysplastic region in relation to normal bone, namely the first presented a more uniform texture than the second. Through this, the technique of cross-validation "leave-one-out" is possible to separate the groups with a high accuracy (94.75%) concluding that the lacunarity is a method of image analysis to characterize the contributory fibrous dysplasia.

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JUSTIFICATIVA E OBJETIVOS: O objetivo deste estudo foi descrever um caso de displasia fibrosa e discutir diagnósticos diferenciais,conduta e evolução. RELATO DO CASO: Paciente do sexo masculino, 51 anos, que apresentava abaulamento indolor na região anterior do tórax entre o 4º e 5º espaço intercostal esquerdo. Na radiografia de tórax observou-se massa em lobo superior do hemitórax esquerdo,sendo, portanto solicitado tomografia computadorizada de tórax que evidenciou lesão expansiva em 4º arco costal esquerdo. A cintilografia óssea demonstrou grande área de intenso aumento da remodelação óssea lateralmente entre os 4º e 5º arcos costais esquerdo. O paciente foi submetido à cirurgia para retirada dos arcos costais acometidos, sendo o material encaminhado para análise imuno-histoquímica que selou o diagnóstico de displasia fibrosa. O paciente evoluiu bem e mantém-se assintomático após seis meses de acompanhamento. CONCLUSÃO: A importância do caso descrito está em demonstrar que doenças raras acontecem e só consolida seu diagnóstico quando se pensam em hipóteses e diagnósticos diferenciais,quesitos esses indispensáveis para o exercício da clínica médica.

BACKGROUND AND OBJECTIVES: We describe a case of fibrous dysplasia with to discuss differential diagnosis, management and evolution.CASE REPORT: Male patient, 51 years old male patient presenting painless bulging in the anterior chest, between the 4th and 5th intercostals space. The x-ray demonstrated a tumor in upper lobe of left lung. Because of this abnormality we request a CT scan that demonstrated an expansive lesion of the same localization. The cintilography with computadorized tomography demonstrated bone remodeling in that area. Because of this, the patient was submitted a removalof ribs and, the immunohistochemical methods diagnosed fibrous dysplasia. The patient remains asymptomatic aftersix moths follow-up. CONCLUSION: The importance of this case is we always remember that rare diseases occur and we only made your diagnoses when think about hypothesis and differential diagnoses, indispensable characterizes in medical practice.

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Florid osseous dysplasia (FOD) is a benign condition of the jaws in which the normal architecture of bone is replaced by a fibrous tissue containing a variable amount of bone and cementum-like tissue. This lesion is most commonly seen in middle aged black women. FOD appears as dense, lobulated masses, often symmetrically located in the mandible, rarely in the maxilla. The lesion is usually asymptomatic and benign. However, a secondary infection may occur and its treatment can be difficult and complicated. This paper reports the case of two patients. The first one is a white woman aged 65 and the second one is a black woman aged 70, both diagnosed with FOD, revealed by secondary infections. The diagnosis was based on clinical and radiographic findings, as biopsy is contraindicated. Radiological and clinical features of FOD and its management will be also discussed on the basis of recent literature.

La displasia ósea florida (DOF) es una patología benigna del maxilar y mandíbula en la que se sustituye la arquitectura normal del hueso por un tejido fibroso que contiene una cantidad variable de tejido óseo y cementoide. Esta lesión es más frecuente en mujeres negras de edad media. La DOF aparece como una masa densa, lobulada, a menudo situada simétricamente en la mandíbula, rara vez en el maxilar. La lesión suele ser asintomática y benigna. Sin embargo, una infección secundaria puede ocurrir y su tratamiento puede ser difícil y complicarse. Este artículo reporta el caso de dos pacientes. La primera es una mujer blanca de 65 años y la segunda es una mujer negra de 70 años, ambas diagnosticadas con DOF, revelada por infecciones secundarias. El diagnóstico se basó en los hallazgos clínicos y radiológicos, ya que la biopsia está contraindicada. Las características clínicas y radiológicas de la DOF y su manejo son discutidos en base a la literatura reciente.

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Várias lesões podem acometer os ossos do complexo maxilo-facial, as quais apresentam comportamento e caracterísitcas clínico-radiográficas distintas dependendo da natureza de cada uma delas. Nesses ossos ocorrem lesões neoplásicas e não neoplásicas, como os cistos e tumores odontogênicos, cistos e neoplasias não-odontogências, lesões fibro-ósseas, reativas e do desnvolvimento. Nesta revisão, por reapresentarem lesões de relevância clínico-radiográfica, foram discutidas as caracterísitcsa clínico-epidemiológicas, radiográficas e diagnóstico diferencial das lesões fibro-ósseas (displasia fibrosa, displasias ósseas e fibroma ossificante) além de outras patologias não neoplásicas como lesão central de células gigantes, cisto ósseo aneurismático, cavidade óssea idiopática e defeito ósseo de Stafne.

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A displasia fibrosa é uma condição patológica benigna onde ocorre a proliferação e substituição do tecido ósseo por tecido fibroso. Existem duas categorias primárias da doença: displasia fibrosa monostótica, que envolve somente um osso, e displasia fibrosa poliostótica, que acomete múltiplos ossos, ocorrendo mais frequentemente no esqueleto crânio-facial. Geralmente esta condição patológica tem início na infância e progride até a puberdade e adolescência, podendo estar relacionada com a chamada Síndrome de McCune-Albright. Neste trabalho, realizou-se uma revisão de literatura a apresenta-se um caso clínico, enfocando os aaspectos etiológicos, clínicos, imaginológicos e o respectivo tratamento da displasia fibrosa crânio-facial.

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