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A 27-yearold female patient with swelling on her head since childhood. A) Axial CT image shows histopathologically verified fibrous dysplasia in groundglass density on right parietal bone. Axial plane B) T2-weighted and C) T1-weighted MR images show slightly hypointense lesion. D-F) Focal contrast-enhancement on MR images in early phase (D), increasing contrast-enhancement in later phases (E) and homogenous intense contrast-enhancement in late phase (F) are shown in axial, sagittal and axial planes, respectively.
Source publication
Aim:
Fibrous dysplasia (FD) is relatively rare in craniofacial region compared to other regions of body. In the present study, radiological findings of FD detected in patients who had computed tomography (CT) and/or magnetic resonance imaging (MRI) were evaluated.
Material and methods:
This study included a total of 25 patients (17 female and 8...
Contexts in source publication
Context 1
... 16 patients (64.0%), one bone was involved while multiple bones were involved in 9 patients (36.0%). Involved bones of 16 patients with single bone involvement were maxillary bone in six patients (Figures 1A-C; 2A, B; 3A-D), parietal bone in five patients ( Figure 4A-F), frontal bone in two patients and sphenoid, mandibular and temporal bones in one patient each. Multiple bones of up to four were involved, sphenoid bone being the most common one (Figures 5A-D; 6A-F). ...
Context 2
... cystic pattern was not observed in any patient. Intense heterogeneous contrast-enhancement was observed in four of the five patients who had contrast-enhanced MRI examination ( Figure 3A-D), while a contrasting increasing towards the later phases were observed in the fifth patient ( Figure 4A-F). Demographic and clinical features of FD patients were presented in Table I, while their radiological findings were given in Table II. ...
Context 3
... diffusion restriction was observed on diffusion-weighted MRI series. Dense heterogeneous contrasting was observed in lesions of four patients who had contrast-enhanced MRI, while in another patient contrastenhancement started as focal contrast-enhancement areas in early phases and turned to intense contrast-enhancement in late phase ( Figure 4A-F). ...
Context 4
... 16 patients (64.0%), one bone was involved while multiple bones were involved in 9 patients (36.0%). Involved bones of 16 patients with single bone involvement were maxillary bone in six patients (Figures 1A-C; 2A, B; 3A-D), parietal bone in five patients ( Figure 4A-F), frontal bone in two patients and sphenoid, mandibular and temporal bones in one patient each. Multiple bones of up to four were involved, sphenoid bone being the most common one (Figures 5A-D; 6A-F). ...
Context 5
... cystic pattern was not observed in any patient. Intense heterogeneous contrast-enhancement was observed in four of the five patients who had contrast-enhanced MRI examination ( Figure 3A-D), while a contrasting increasing towards the later phases were observed in the fifth patient ( Figure 4A-F). Demographic and clinical features of FD patients were presented in Table I, while their radiological findings were given in Table II. ...
Context 6
... diffusion restriction was observed on diffusion-weighted MRI series. Dense heterogeneous contrasting was observed in lesions of four patients who had contrast-enhanced MRI, while in another patient contrastenhancement started as focal contrast-enhancement areas in early phases and turned to intense contrast-enhancement in late phase ( Figure 4A-F). ...
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Citations
... Lesions are intermediate to low on T1-weighted imaging, variable on T2-weighted imaging and demonstrate heterogenous moderate to avid enhancement. 8,9 The case of MAS displayed hypointense signal with MRI on all sequences consistent with sclerosis as seen on radiographs. A postcontrast study was not performed in this instance of MAS. ...
... On MRI, they are hypo-to slightly hyperintense on T1WI and hyperintense on T2WI with a varied pattern of enhancement. Four different patterns of enhancement have been documented, which include 8,9 peripheral, peripheral and patchy internal (as in the presented case), peripheral and linear internal, and heterogeneous internal. The presence and amount of myxoid tissue together with tumoural cellularity and vascularity gives rise to the different enhancement patterns. ...
... 11 The most common complication of surgical excision of the myxomas is recurrence. 9 Mazabraud syndrome is associated with a higher incidence of malignant transformation to osteosarcoma, 8 and thus clinical and radiological surveillance is essential. ...
Fibrous dysplasia (FD) is a rare, non-inherited, congenital bone disorder which may be monostotic or polyostotic. The polyostotic form may rarely present in syndromic forms when associated with extra-skeletal manifestations. Mazabraud syndrome is a rare syndrome consisting of polyostotic FD presenting with intramuscular myxomas. McCune–Albright syndrome is recognised by polyostotic FD, precocious puberty and ‘café au lait’ spots. This report describes an adult patient with Mazabraud syndrome and a child with McCune–Albright syndrome.Contribution: Radiographic findings are typical with bowing deformities, sclerotic, lucent or mixed lesions and bony expansion, often with endosteal scalloping. MRI is often non-contributory and may actually mimic a more aggressive process. Early detection and correct diagnosis allow for early preventative treatment and rehabilitation to prevent devastating neurological sequelae and disability.
... MRI is an excellent method for assessing cases of complex fibrous dysplasia, reflecting the variable tissue components. CT complements and enhances the interpretation of MRI of bone lesions, especially in cases where MRI shows enhancement that is suspicious of a malignant neoplasm (19,23). However, the presentation of those features is often non-specific, and it is recommended to use multimodal imaging that can show the anatomy, blood supply, molecular components and metabolism of the lesion at the same time to improve the diagnostic accuracy of FDB and its malignant transformation. ...
Fibrous dysplasia of bone (FDB) is a rare benign condition in which fibrous tissue replaces normal bone architecture. FDB rarely undergoes malignant transformation, but there are reports of locally aggressive fibrous dysplasia with cortical destruction and soft tissue extension. Diagnosis of FDB malignant transformation is not easy, especially in monostotic form, because of the overlap in imaging features of locally aggressive fibrous dysplasia and fibrous dysplasia with malignant transformation. The present case study reports a rare case of FDB in a 23-year-old man with polyostotic fibrous dysplasia arising in the left side of the pelvis and lower limb bones with partial transformation to fibrosarcoma. This study explored the multimodal imaging features of FDB malignant transformation, to achieve early detection and improve diagnostic accuracy of local FDB aggressiveness and its malignant transformation.
... La dysplasie fibreuse (DF) osseuse est une pathologie bénigne non néoplasique caractérisée par l'apposition d'un tissu fibreux et osseux immature d'architecture atypique à la place de l'os médullaire sain (Gokce & Beyhan 2020). Son expansion est lente et peut entraîner des déformations et une fragilité osseuse (Gokce & Beyhan 2020). ...
... La dysplasie fibreuse (DF) osseuse est une pathologie bénigne non néoplasique caractérisée par l'apposition d'un tissu fibreux et osseux immature d'architecture atypique à la place de l'os médullaire sain (Gokce & Beyhan 2020). Son expansion est lente et peut entraîner des déformations et une fragilité osseuse (Gokce & Beyhan 2020). Elle est non héréditaire, due à une mutation somatique de type gain de fonction du gène GNAS, codant la sous-unité alpha de la protéine Gs. ...
... 2019 Les examens radiographiques de routine sont généralement à l'origine de la découverte fortuite de la pathologie. L'imagerie tomographique à faisceau conique (CBCT) est l'examen de choix pour l'évaluation des caractéristiques radiologiques des lésions maxillaires (Gokce & Beyhan 2020, Atalar et coll. 2015. ...
Fibrous dysplasia (FD) is a rare, congenital, benign bone disorder in which healthy bone tissue is replaced by abnormal scar-like (fibrous) connective tissue. The lesions may affect one or more bones, including the jawbones. A 13-year-old girl visited the dentist for failed eruption of her left maxillary permanent teeth and facial asymmetry. Radiological analysis revealed impactions of the permanent teeth in hypertrophic, hyperdense, weakly trabeculated bone tissue. To restore the aesthetics of the smile, a treatment combining decoronation and bonded restorations was performed. FD enlargement would slow down after puberty, allowing for long-term therapies. But there is no information about implantology in this type of bone lesion. The aesthetic and functional consequences of a maxillary FD should be managed as soon as possible. Treatment involves a multidisciplinary team and follow-up care into adulthood.
... La dysplasie fibreuse (DF) osseuse est une pathologie bénigne non néoplasique caractérisée par l'apposition d'un tissu fibreux et osseux immature d'architecture atypique à la place de l'os médullaire sain (Gokce & Beyhan 2020). Son expansion est lente et peut entraîner des déformations et une fragilité osseuse (Gokce & Beyhan 2020). ...
... La dysplasie fibreuse (DF) osseuse est une pathologie bénigne non néoplasique caractérisée par l'apposition d'un tissu fibreux et osseux immature d'architecture atypique à la place de l'os médullaire sain (Gokce & Beyhan 2020). Son expansion est lente et peut entraîner des déformations et une fragilité osseuse (Gokce & Beyhan 2020). Elle est non héréditaire, due à une mutation somatique de type gain de fonction du gène GNAS, codant la sous-unité alpha de la protéine Gs. ...
... 2019 Les examens radiographiques de routine sont généralement à l'origine de la découverte fortuite de la pathologie. L'imagerie tomographique à faisceau conique (CBCT) est l'examen de choix pour l'évaluation des caractéristiques radiologiques des lésions maxillaires (Gokce & Beyhan 2020, Atalar et coll. 2015. ...
La dysplasie fibreuse (DF) osseuse est une patho- logie osseuse rare, congénitale et bénigne dans laquelle le tissu osseux sain est remplacé par du tissu fibro-osseux. Les lésions peuvent affecter un ou plusieurs os, notamment les os des mâ- choires. Une adolescente de 13 ans a consulté pour des échecs d’éruption des dents perma- nentes maxillaires gauches et une asymétrie du tiers moyen de l’hémiface gauche. L’analyse ra- diologique a mis en évidence des inclusions des dents permanentes dans un tissu osseux hyper- trophique, hyperdense et faiblement trabéculé. Afin de restaurer l’esthétique du sourire, une prise en charge associant décoronation et res- taurations collées a été privilégiée. L’évolution de la DF aurait tendance à ralentir après la puberté, permettant ainsi de planifier des thérapeutiques à long terme dès la fin de la croissance. Cepen- dant, il existe peu de données sur le recours à l’implantologie dans le cas de lésions osseuses sclérotiques. Les conséquences esthétiques et fonctionnelles d’une DF maxillaire peuvent être majeures sur le plan bucco-dentaire. Leur prise en charge au cas par cas fait intervenir une équipe pluridisciplinaire, nécessitant un suivi de l’enfance jusqu’à l’âge adulte.
... As a result, fibrous dysplasia typically appears on CT images as an expanded medullary bone with ground-glass opacity, causing endosteal scalloping and cortical thinning. Depending on the proportion of the osseous and fibrous components and the presence or absence of hemorrhage and/or cystic degeneration, there are three different patterns of fibrous dysplasia that have been described: homogeneously sclerotic (typically showing diffuse ground-glass opacity), lytic (or predominantly lytic), and mixed (ie, basipostsphenoid) fibrous dysplasia (Fig 10) (Table 3) (21,22). ...
... Although classic low signal intensity from the osseous components is seen at MRI, this is not always present and the appearance is commonly confusing, especially at T1-weighted gadolinium-enhanced MRI, with heterogeneous and avid enhancement from the fibrous component masquerading as a tumor (21). Correlation with CT images is always useful when the MRI appearance is confusing or inconclusive. ...
The greater wings of the sphenoid bone (GWS) comprise the components of the sphenoid bone that make up most of the posterior orbital wall and form the anterior and medial parts of the floor of the middle cranial fossa. Many important skull base foramina, which transmit vital neurovascular structures, are present in these paired wings on either side of the central body of the sphenoid bone. A wide variety of diseases can affect the GWS, ranging from benign osseus lesions to malignant primary and secondary bone abnormalities. The complex three-dimensional curved (winged) shape of the GWS and the wide array of pathologic entities that affect this bone can make it challenging for the radiologist to report the imaging findings accurately, especially in relation to the important skull base foramina. The authors describe a systematic approach to understanding the three-dimensional anatomy of the GWS and review important diseases, with the aid of imaging examples. Useful imaging "pearls" that can help in making specific diagnoses are provided throughout the article. ©RSNA, 2022.
... Four clinical presentations of FD have been described [65]: ...
Benign bone tumors in the pediatric population can have varied clinical presentations ranging from asymptomatic to nonspecific pain, swelling, or pathological fracture. A systemic imaging approach should be utilized to evaluate for focal bone abnormalities. Radiologists must be aware of salient imaging features of pediatric benign bone tumors, as it helps to guide clinicians for further management and help decreasing patient anxiety and unnecessary medical intervention.
Bone-related disorders of the jaw (BRDJ) include a spectrum of non-neoplastic and neoplastic lesions of the maxillofacial region that have been recently classified into fibro-osseous lesions, giant cell lesions and osseous tumours. The histopathological features of BRDJ can be similar and overlie each other. Imaging is important in order to reach a specific diagnosis. However, the appearance of BRDJ on imaging is non-specific in some cases. Computed tomography (CT) and magnetic resonance imaging (MRI) are used for accurate localisation, characterisation of the tumour matrix, delineation of the lesion extension and establishment of the relation of BRDJ to the surrounding structures. Imaging is usually done to detect the relationship with the adjacent surrounding vital structures and to diagnose aggressive forms, malignant transformation and associated syndromes. The correlation of the demographic findings, the location and the clinical presentations with the imaging features are important for the diagnosis of BRDJ. The proposed clinico-radiological diagnostic algorithm with CT and MRI helps a specific diagnosis to be reached in some cases.
