Search

Objective: Current management of fibrous dysplasia (FD) involving the paranasal sinuses and the adjacent skull base is currently controversial. This study aims to present our experience in the management strategy of FD that involves the paranasal sinuses and the adjacent skull base. Methods: Twenty three patients from 2006 to 2019 with monostotic fibrous dysplasia (MFD), polyostotic fibrous dysplasia (PFD), or McCune-Albright syndrome (MAS) involving the paranasal sinuses and the adjacent skull base were retrospectively reviewed. This study series was divided into 3 groups based on management strategies: the observation group, the surgery group, and the optic nerve decompression group. Results: The observation group included 9 patients with asymptomatic MFD with stable condition during the follow-up period of 15 to 164 months. The surgery group included 10 symptomatic patients with MFD who had personalized endoscopic endonasal surgery. The symptoms of the patients were relieved after surgery. The optic nerve decompression group included 4 patients with visual loss, who underwent endonasal endoscopic optic nerve decompression (EOND) with the aid of image-guided navigation. Their vision improved after surgery. Conclusions: Clinical observation and periodic computed tomography (CT) scan are adopted for asymptomatic patients. Surgery is indicated in symptomatic patients. Optic nerve decompression is recommended as soon as possible if the patient has visual loss, whereas prophylactic decompression is not recommended if the optic nerve is encroached by FD without visual loss. Navigation plays an important role in endoscopic surgery involving the paranasal sinuses and the adjacent skull base, especially in FD resection and optic nerve decompression. [ABSTRACT FROM AUTHOR]

The article "Augmented Reality in Skull Base Oncology: A Pilot Study Focused on Tumor Resection and Skull Base Reconstruction" discusses the use of augmented reality (AR) in skull base tumor surgery to aid in defining tumor borders and planning for reconstruction with patient-specific titanium implants. The study utilized Brainlab navigation software to create 3D virtual surgical plans, which were then transferred to the Microsoft HoloLens 2 for intraoperative use. AR was successfully used in three cases, assisting surgeons in defining tumor margins, differentiating lesions from normal bone, and achieving optimal cosmesis. The study concludes that AR has the potential to enhance or replace some forms of intraoperative surgical navigation in treating skull base bony tumors. [Extracted from the article]

The article discusses a case series of six pediatric patients with lateral skull base encephaloceles, a rare condition involving bone defects and herniation of intracranial contents. Different surgical approaches and materials were used to repair these defects, including the use of a sternocleidomastoid muscle graft in immunocompromised patients. The study highlights the importance of a multidisciplinary approach in tailoring treatment to individual patient needs and introduces the use of a vascularized sternocleidomastoid graft as a potential option for encephalocele repair in pediatric cases. [Extracted from the article]

Background: Benign bone lesions are a common incidental finding in athletes during workup for musculoskeletal complaints, and athletes are frequently advised to halt participation in contact sports. There are no current guidelines to assist clinicians in referring patients with these lesions to a subspecialist or in advising athletes on the safety of returning to sport. Purpose: To assist sports medicine physicians in appropriate referral for patients with benign bone lesions through presentation of a literature review and the case of an adolescent athlete with a benign bone lesion in a location with a significant fracture risk. Study Design: Systematic review; Level of evidence, 4. Methods: A systematic literature review was performed using the PubMed database. Search terms included "enchondroma,""unicameral bone cyst,""UBC,""simple bone cyst,""SBC,""aneurysmal bone cysts, "ABC,""nonossifying fibroma,""NOF,""non-ossifying fibroma,""chondroblastoma,""osteochondroma,""exostosis,""chondromyxoid fibroma,""periosteal chondroma," and "fibrous dysplasia" combined with "fracture,""sports,""sport,""contact sport,""football," or "rugby." Randomized controlled trials, case series, and prospective and retrospective studies were all included. Abstracts were excluded. Results: In total, 42 separate articles were reviewed. The strength of evidence for each lesion was determined using the total number of patients described in the literature with the pathology. Unicameral bone cysts, aneurysmal bone cysts, and fibrous dysplasia, particularly in the spine, are associated with a high risk of fracture; therefore, subspecialist referral is warranted before returning to sport. Osteochondromas (exostosis), juxtacortical chondromas, nonossifying fibromas, chondromyxoid fibromas, and enchondromas were associated with low fracture risk, and decisions regarding referral can be made on a case-by-case basis. Conclusion: The presence of a benign bone lesion does not always necessitate immediate, absolute restriction from participation in contact sports. After appropriate workup and diagnosis, the risk of return to sport should be evaluated based on the pathology present, and the patient and clinician should engage in a shared decision-making process. The guidelines in this paper provide context for stratifying risk and the importance of specialist referral. For athletes with a confirmed diagnosis of an asymptomatic benign bone lesion with a low risk of fracture, return to play without restrictions or referral is often safe. [ABSTRACT FROM AUTHOR]

Fibrous dysplasia is an uncommon bone disorder affecting various parts of the skeleton, often affecting facial and cranial bones. In this case, a 10-year-old patient was diagnosed with fibrous dysplasia of the ethmoid sinus at an early age. The patient has experienced nasal congestion, snores, and worsening nasal patency since 2019. A CT scan revealed an expansive proliferative lesion, likely from the frontal or ethmoid bone, protruding into the nasal cavity, ethmoid sinus, and right orbit. The tumor causes bone defects in the area of the nasal bone, leading to fluid retention in the peripheral parts of the right maxillary sinus. The patient's parents decided not to undergo surgery to remove the diseased tissue and reconstruct the area, as it would be very extensive, risky, and disfiguring. The patient is being treated conservatively with an MRI, with a contrast performed approximately every six months and infusions of bisphosphonates. Despite the lesion's size, the patient does not experience pain characteristic of dysplasia, and functions typically. Fibrous dysplasia of bone is a rare condition that presents with the most visually apparent manifestations, often mistaken for other bone conditions. Advanced diagnostic tools, like CT and MRI, are used to identify conditions affecting the ethmoid sinus more frequently. However, diagnostic errors often occur in imaging studies, leading to confusion. The most common period for clinical manifestations and diagnosis is around 10 years of age. The preferred approach in managing fibrous dysplasia involves symptomatic treatment, which can alleviate airway obstruction, restore normal globe position and visual function, and address physical deformities. Surgical intervention is recommended only for patients with severe functional impairment, progressive deformities, or malignant transformation. [ABSTRACT FROM AUTHOR]

Background: Fibrous dysplasia (FD) is a self-limiting benign disease with slow progression in which the normal bone is replaced by dysplastic fibrous tissue. The craniofacial skeleton is one of the most commonly affected areas, and it can create unique challenges in dental implant therapy. This case aims to report an unusual presentation of FD localized in the alveolar crest bone of the edentulous site, causing special obstacles to implant placement, and provide a diagnostic and treatment process that may be referenced. Case presentation: A 32-year-old female patient presented with an abnormal expansion of the alveolar crest on the edentulous site of the mandibular first molar, which caused insufficient inter-arch space and pseudo-pocket in the adjacent teeth, previous medical history of residual root that existed for many years before tooth extraction was identified retrospectively. FD was diagnosed by the clinical, radiographic, and pathological examination, and the curettage was performed to remove abnormal bone tissue and obtain adequate restorative space for further implant therapy. This intervention was followed by histologically confirmed healing and successfully integrated implant placement. Conclusion: We described an unreported pathogenic site of FD that occurred at the alveolar ridge of the edentulous site, which ended up with satisfactory oral implant rehabilitation through 5-year sequential diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Fibrous dysplasia is a slow-progressing benign condition characterized by abnormal bone formation that leads to some skeletal disorders. Although some of the fibrous dysplasia have unusual clinical and radiographic features that can lead to a challenging diagnosis, most lesions reveal an expansile bone defect due to cortex thinning. This report presented a case of monostotic fibrous dysplasia of a 43-year-old woman with involvement of the right maxillary jaw and sinuses, which indicated unusual histopathological features. The patient was referred to the Department of Oral and Maxillofacial Surgery of Tehran University of Medical Sciences (Tehran, Iran) in 2022. Radiological and histological findings were discussed. The unusual histomorphic pattern of the lesion caused uncertainty and resulted in a missed definitive diagnosis in the primary biopsy. It was highlighted that awareness of rare histologic variants in fibro-osseous lesions especially fibrous dysplasia was required to improve diagnostic confidence.

ABSTRACT Objective Subjects and Methods Results Conclusions To assess the sclerostin, β‐catenin, and tryptase expression in fibro‐osseous lesions (FOL) of the jaws.Immunohistochemistry analysis was performed for these proteins on FOL and non‐lesional bone. The sclerostin‐positive cells were scored from 0 (no expression) to 3 (high expression).We analyzed 46 FOL biopsies and selected 38 patients. Categorization showed 15 fibrous dysplasia (FD), eight juvenile trabecular ossifying fibroma (JTOF), two psammomatoid ossifying fibroma (PsOF), and 13 FOL. We found more sclerostin‐positive cells in fibrous tissue than in bone, showing a phenotype like mast cells with strong dot‐cytoplasmic positivity. The analysis of sclerostin‐positive cell lesions (scored as 2 and 3) showed also tryptase positivity in 80.9% of 21 biopsies. β‐catenin was diffusely expressed on the fibrous component, mostly with cytosol staining. Non‐lesional bone showed sclerostin expression in medullary spaces and a few osteocytes.Sclerostin‐positive cells are mostly found in the fibrous tissue of FOL, and the tryptase mast cell marker was present in most of the lesions that were positive for sclerostin. [ABSTRACT FROM AUTHOR]

Background Methods Results Conclusion Plain Language Summary Key points Fibrous dysplasia is generally rare, and even rarer in older adults. Special care is needed when altering the alveolar bone in these cases, especially if an implant is involved. This case study highlights such a scenario.This case study details the experience of a 63‐year‐old African American female who presented with a bump on her jaw, ultimately leading to a diagnosis of fibrous dysplasia. The patient underwent surgery for osseous recontouring to address the bone overgrowth associated with the condition.The study documents the successful surgical recontouring of a fibrous dysplasia lesion in the upper right maxilla that developed 5 years after dental implant placement. Additionally, it highlights the preservation of peri‐implant hard and soft tissues over a 6‐month follow‐up period post‐surgery, ensuring proper tissue healing and bone shaping for optimal patient outcomes.The successful surgical recontouring of fibrous dysplasia in the upper right maxilla, along with the preservation of peri‐implant hard and soft tissues, underscores the potential for effective oral rehabilitation in similar cases involving dental implants.In this study, we managed a rare bone disorder called fibrous dysplasia in a 63‐year‐old woman, 5 years after she had dental implants in her upper jaw. This condition leads to bone overgrowth and deformities, making oral rehabilitation challenging. The patient presented with a bump in her upper jaw, which was diagnosed through various tests. We performed surgery to reshape the overgrown bone while preserving her dental implants and surrounding tissues. The surgery was successful, and 6 months later, the implants remained stable with no signs of recurring issues.Why is this case new information?This case is rare because it presents a successful management strategy for fibrous dysplasia in the maxilla with dental implants, a combination that is both uncommon and challenging.What are the keys to the successful management of this case?Successful management relies on accurate diagnosis, careful surgical recontouring to preserve peri‐implant bone while providing a maintainable environment for the patient, and thorough postoperative care to ensure healing and stability.What are the primary limitations to success in this case?Primary limitations include the risk of implant compromise, potential recurrence of fibrous dysplasia, and challenges related to bone quality and healing, particularly in an older patient. [ABSTRACT FROM AUTHOR]

Fibrous dysplasia is benign bone tumor, localized predominantly in diaphyses of long bones of extremities. In majority of cases the lesions are monoostotic, polyostotic form is uncommon, possibility of malignant transformation is very low (0.5%). [ABSTRACT FROM AUTHOR]

Chronic osteomyelitis of the skull base is a commonly reported pathology in existing scientific literature, but chronic osteomyelitis of the skull vault (COSV) is a rarely documented disease. We report the case of a 38 years old Afghan male with a presenting complaint of irregular swelling on the skull vault for six months. The patient had a history of head trauma one year back with a compound depressed fracture which had been surgically managed then. Physical examination revealed a 15 x 15 cm hard, immobile swelling with an old scar mark over the scalp. Neuroimaging was consistent with diffuse, bilateral frontoparietal swelling of bony origin, sparing underlying brain parenchyma. Surgically excision of the lesion was done through a bicoronal skin flap and cranioplasty done at the same time. Histological findings of the specimen reported chronic osteomyelitis. However, microbiology revealed no growth in culture. Patient was discharged on the second postoperative day uneventfully. This case turns minds into keeping skull vault osteomyelitis as differential diagnosis besides other spontaneous bony lesions; e.g. fibrous dysplasia, osteoma and giant cell tumours. [ABSTRACT FROM AUTHOR]

Background: Giant cell reparative granulomas are nonneoplastic, benign lesions that can expand and dissolve bone. Fibrous dysplasia is a benign condition in which normal bone tissue is replaced by abnormally proliferating immature reticular bone and fibrous tissue. The combination of giant cell reparative granuloma and fibrous dysplasia is extremely rare and can pose diagnostic and therapeutic challenges because of the complexity of clinical presentation. Case Presentation: We here present a patient who had a combination of fibrous dysplasia of bone and a giant cell reparative granuloma. An elderly male was admitted to the hospital with a blood‐streaked nasal discharge, blurry vision in his right eye, and an enlarged mass under the chin. A CT scan revealed that the lesion had infiltrated the head and face extensively, including the right maxillary sinus, sieve sinus, and the right nasal cavity, contraindicating surgery. The patient received a total dose of 30 Gy of 6 MV x‐ray radiotherapy delivered through helical tomotherapy over 15 sessions, with a single dose of 2 Gy being administered five times a week. Concurrently, The dose is 4 mg of zoledronic acid administered intravenously once every 21 days. After treatment, the patient's nasal congestion was significantly relieved, the vision of the right eye improved, and the mandibular lesion was significantly reduced. Conclusions: Treatment with radiotherapy combined with zoledronic acid for our patient's inoperable osteolytic giant cell reparative granuloma adjacent to vital nerves and blood vessels was extremely effective and safe. This case report provides a reference for the management of this rare combination. [ABSTRACT FROM AUTHOR]

The present review aimed to explore the treatment of metabolic bone diseases using anti-sclerostin antibodies, including Osteoporosis, Osteogenesis Imperfecta, Hypophosphatemia, Paget's disease, Fibrous Dysplasia, Multiple Myeloma, Fluorosis, and McCune-Albright Syndrome. These disorders representing metabolic bone diseases are elaborated in the present work. Literature was searched from publicly available databases including PubMed, Scopus, and the Web of Science. Most studies, showed that bone mineral density rose and the risk of fracture was decreased in individuals after being treated with anti-sclerostin antibodies. However, the possibility that these antibodies may trigger cardiovascular events or venous thromboembolism was mentioned as a reason for alarm. Even though anti-sclerostin antibodies have shown promise in treating metabolic bone disorders, additional study is required to determine whether or not they pose any safety concerns. [ABSTRACT FROM AUTHOR]

Objective: In this study, our aim was to develop and validate the effectiveness of diverse radiomic models for distinguishing between gnathic fibrous dysplasia (FD) and ossifying fibroma (OF) before surgery. Materials and Methods: We enrolled 220 patients with confirmed FD or OF. We extracted radiomic features from nonenhanced CT images. Following dimensionality reduction and feature selection, we constructed radiomic models using logistic regression, support vector machine, random forest, light gradient boosting machine, and eXtreme gradient boosting. We then identified the best radiomic model using receiver operating characteristic (ROC) curve analysis. After combining radiomics features with clinical features, we developed a comprehensive model. ROC curve and decision curve analysis (DCA) demonstrated the models' robustness and clinical value. Results: We extracted 1834 radiomic features from CT images, reduced them to eight valuable features, and achieved high predictive efficiency, with area under curves (AUC) exceeding 0.95 for all the models. Ultimately, our combined model, which integrates radiomic and clinical data, displayed superior discriminatory ability (AUC: training cohort 0.970; test cohort 0.967). DCA highlighted its optimal clinical efficacy. Conclusion: Our combined model effectively differentiates between FD and OF, offering a noninvasive and efficient approach to clinical decision‐making. [ABSTRACT FROM AUTHOR]

Background: Osteosarcoma of the jaws is a rare primary malignant tumor of bone. The clinical, radiological and histopathological features of a case associated with cemento-osseous dysplasia is presented. Case Report: A 57-year-old mixed-race female presented with a large, progressive, swelling of the right mandible. Radiographic examination revealed two associated lesions. Partially defined irregular radiopacities were noted in the left mandible, extending from the premolar to the molar region. The lesion had a cotton-wool appearance and resembled a fibro-osseous lesion; i.e. cemento-osseous dysplasia. A second large, expansive and irregular, radiopaque lesion was noted on the right angle of the mandible, extending beyond the inferior cortex of the mandible. The internal structure was heterogeneous and resembled irregular bone formation. The classic "sunburst" appearance of radiating bony spicules can be seen in the posterior–anterior view and the CBCT 3D reconstruction, indicating the outgrowth of the tumor matrix. Histopathological exam confirmed a final diagnosis of osteosarcoma closely associated with cemento-osseous dysplasia. The patient underwent a fludeoxyglucose-18 (FDG) positron emission tomography (PET) scan which indicated metastasis in the left lung and increased uptake in the right mandible. Chemotherapy was initially administered with a plan to resect the tumor, however, the patient demised as a result of medical complications. Conclusion: The question in the literature remains whether these two entities are coincidentally found or arise from each other. Nevertheless, it is important for clinicians to closely monitor patients with cemento-osseous dysplasia and biopsy any suspicious lesions that may develop into osteosarcoma. [ABSTRACT FROM AUTHOR]

Thoracic fibrous dysplasia (FD) is a benign, osseous chest wall tumor. It originates from bone marrow and accounts for 30–50% of all benign osseous neoplasms in the chest wall. In FD, normal bone marrow is replaced by fibrous stroma and immature bone. We present a rare case in which massive intrathoracic polyostotic FD originating from the rib was diagnosed and treated. The extrathoracic part of the tumor appeared stable and unalarming for decades; however, in hindsight, the intrathoracic part significantly progressed, eventually leading to symptoms. The tumor was removed through a hemi-clamshell approach, which allowed adequate visualization and control of mediastinal structures. After establishing the diagnosis of FD, regular follow-up imaging is crucial for timing of a surgical intervention to prevent symptoms, impairment of quality of life, and unnecessarily complex resections. [ABSTRACT FROM AUTHOR]

Cemento-osseous dysplasia (COD) is classified, by the World Health Organization as a benign fibro-osseous lesion related to the tooth and periapical area of the jaws and is considered as a benign reactive process appearing from the apical periodontium in close relation with the apices of teeth. Usually, it is asymptomatic, discovered accidentally, and affecting particularly middle-aged African women. There are four subtypes distinguished of the lesion: periapical (PCOD), focal (FCOD), florid (FLCOD) and familial florid cementoosseous dysplasia (FFLCOD). Pseudocysts found in the jaws go by various names, including solitary bone cyst, traumatic bone cyst, or simple bone cyst (SBC). These two pathologies have been reported separately; however, their co-occurrence remains rare and the first case of FLCOD with co-occurrence of SBC was reported by Melrose et al. in 1976 and later a few cases been reported in the literature. The aim of this report is to describe a case of a 46- year-old oriental female diagnosed with FLCOD with co-occurrence of SBC. Under local analgesia, a surgical exploration of the cyst was performed. In addition, a biopsy with a trephine was done in the region of missing right first mandibular molar. Based on the patient clinical, radiographic, and histological findings, a diagnosis of FLCOD was made in cooccurrence with a mandibular SBC. An examination of another female family member unveils a distinctive case, and the familial factor has been ruled out. No further treatment was planned and only follow-up was suggested. [ABSTRACT FROM AUTHOR]

Craniosynostosis refers to the premature fusion of one or more of the fibrous cranial sutures connecting the bones of the skull. Machine learning (ML) is an emerging technology and its application to craniosynostosis detection and management is underexplored. This systematic review aims to evaluate the application of ML techniques in the diagnosis, severity assessment, and predictive modeling of craniosynostosis. A comprehensive search was conducted on the PubMed and Google Scholar databases using predefined keywords related to craniosynostosis and ML. Inclusion criteria encompassed peer-reviewed studies in English that investigated ML algorithms in craniosynostosis diagnosis, severity assessment, or treatment outcome prediction. Three independent reviewers screened the search results, performed full-text assessments, and extracted data from selected studies using a standardized form. Thirteen studies met the inclusion criteria and were included in the review. Of the thirteen papers examined on the application of ML to the identification and treatment of craniosynostosis, two papers were dedicated to sagittal craniosynostosis, five papers utilized several different types of craniosynostosis in the training and testing of their ML models, and six papers were dedicated to metopic craniosynostosis. ML models demonstrated high accuracy in identifying different types of craniosynostosis and objectively quantifying severity using innovative metrics such as metopic severity score and cranial morphology deviation. The findings highlight the significant strides made in utilizing ML techniques for craniosynostosis diagnosis, severity assessment, and predictive modeling. Predictive modeling of treatment outcomes following surgical interventions showed promising results, aiding in personalized treatment strategies. Despite methodological diversities among studies, the collective evidence underscores ML's transformative potential in revolutionizing craniosynostosis management. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumor (SFT) is a rare spindle cell tumor originating from mesenchymal tissue, and even rarer when it occurs intracranially. This case report described a 42-year-old man who presented with headache and limb weakness for more than 10 days. Magnetic resonance imaging (MRI) showed a well-defined multicompartmental cystic space-occupying lesion in the left occipital region, with surrounding edema and a compressed left lateral ventricle, the mass growing across the cerebellar vermis, which was initially diagnosed as hemangioblastoma. Neurosurgery was utilized to successfully remove the mass, and intracranial solitary fibrous tumor (ISFT) was identified by postoperative pathological analysis. Here, this article describes the imaging manifestations and pathologic features of a case of cystic intracranial solitary fibrous tumor, aiming to improve the understanding and diagnosis of this disease in order to provide an accurate therapy plan. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Cryptogenic multifocal ulcerating stenosing enteropathy (CMUSE) is a rare noninfectious chronic inflammatory disease of the digestive tract confined to the small bowel. Chronic inflammatory wasting leads to protein loss and weight reduction, and some patients eventually develop small bowel stenosis. The etiopathogenesis of CMUSE remains unknown. Case Report: A thin 62-year-old man was admitted to the hospital with abdominal pain and distension accompanied by bilateral lower-extremity edema for 2 months. After a series of medical tests, rheumatic or immune-related diseases, hyperthyroidism, and tuberculosis were excluded, and common digestive system diseases were also excluded. Abdominal CT showed incomplete obstruction of the small bowel. Enteroscopy showed small-bowel luminal narrowing. The patient subsequently underwent partial resection of the small bowel with end-toside anastomosis. The small-bowel stricture was about 120 cm from the ileocecal junction, and about 12 cm of small bowel was resected. Postoperative pathology of the resected material revealed multifocal ulceration of the mucosa with massive inflammatory cell infiltration and extensive hyperplastic fibrous tissue, consistent with the characteristics of CMUSE disease. At follow-up 6 months after surgery, he had no abdominal pain or distension, and his anemia and lower-extremity edema were improved. Conclusions: CMUSE diagnosis requires a combination of patient history, imaging, endoscopy, pathology, and exclusion of other digestive disorders, such as Crohn's disease. It is a chronic wasting disease, often accompanied by weight loss, abdominal pain, melena, and hypoproteinemia. Surgery is an important treatment for intestinal strictures caused by CMUSE. [ABSTRACT FROM AUTHOR]

Aneurysmal bone cyst (ABC) is a rare and usually painful condition, representing about 1% of all bone tumors. A geographical lytic, expansile, and septated radiological pattern, with fluid–fluid levels on MRI, is classically displayed. ABC can be a primary bone lesion (70% of patients) or can arise in an underlying condition and is subsequently named "ABC-like changes" (30%). ABC-like changes are more frequently encountered in skeletal segments affected by chondroblastoma, fibrous dysplasia, giant cell tumor, osteoblastoma, non-ossifying fibroma, and osteosarcoma. In this article, we describe the first case of ABC-like changes developed in association with an ultra-rare sclerosing bone disease: melorheostosis. Melorheostosis is characterized by recognizable patterns on radiological studies with a pathological increased bone density and a cortical thickening within the periosteal or endosteal space, usually with a "dripping candle wax" appearance. More rarely, other different radiological patterns can be observed, such as "osteopatia striata-like," "osteoma-like," "myositis ossificans-like," and mixed patterns. Pain and limb hypotrophy are the most common clinical manifestations. We report the case of a Caucasian male with a clinic-radiological diagnosis of melorheostosis (with epiphyseal osteopoikilosis) since the age of twelve. At the age of nineteen, he suffered from increased pain in the proximal right thigh, and the radiological control revealed an expansive septated lesion at the right proximal femoral bone. The diagnosis of ABC-like changes developed in melorheostosis was obtained after CT-guided bone biopsy and confirmed by open-incisional biopsy. [ABSTRACT FROM AUTHOR]

Abstract Background Focal and florid cemento-osseous dysplasia are benign fibro-osseous lesions affecting the quality and quantity of the jawbones. This study aimed to determine the viability of implant-based approaches in the affected patients. Main text Different scientific databases, including PubMed/MEDLINE, Scopus, Web of Science, Embase, the Cochrane Library, and Google Scholar, were searched until October 8, 2023, using a pre-determined search strategy. Two reviewers screened the retrieved reports and extracted the required information from the included studies. The eligibility criteria included English-language case reports/series or clinical trials. The JBI critical appraisal checklist for case reports was used to assess the methodological quality of the included studies. Three studies were deemed eligible to be included in this study out of the initial 202 records found. Five implants were placed in three patients, positioned in the proximity of the lesion area, without any additional treatment to remove the pathology. The mandibular posterior area was the affected site in all patients. Only one implant failed in one patient after 16 years, which was attributed to peri-implantitis and not the lesion. Other implants demonstrated successful maintenance over follow-up periods. Conclusions Although the number of the included records was relatively low to draw firm conclusions, it seems that implant-based treatments in patients with focal/florid cemento-osseous dysplasia could be viable, considering a conservative and well-planned approach.

The article discusses a case of a 32-year-old man experiencing upper back pain following a minor fall, which was diagnosed as fibrous dysplasia through CT, MRI, and histopathological examination. Fibrous dysplasia is a non-cancerous bone abnormality that can affect any bone, with the monostotic form being more common. Treatment options include orthopedic procedures and medical treatments like bisphosphonates, with the patient in this case opting for non-surgical treatment with alendronate. The article provides insights into the diagnosis, management, and treatment of fibrous dysplasia, emphasizing the importance of individualized care for patients with this condition. [Extracted from the article]

The objective of this systematic review was to evaluate the risks of medication-related osteonecrosis of the jaw (MRONJ) in fibrous dysplasia (FD) and McCune-Albright syndrome (MAS) patients treated with bisphosphonates. Method and materials: A systematic review of the literature was performed by searching PubMed and Embase databases using MeSH terms (fibrous dysplasia of bone, “fibrous dysplasia, polyostotic,” osteonecrosis, jaw, therapeutics, diphosphonates, denosumab, teriparatide, estrogens, hormones, raloxifene hydrochloride, calcitonin, cathepsin K) and non-MeSH terms (antiresorptive therapy, antiresorptives, bisphosphonate, estrogen therapy, hormone therapy, bazedoxifene, cathepsin K inhibitor). Articles were limited to human studies, in English language, in which patients were on antiresorptives for at least 1 year. PRISMA statement guidelines were used to eliminate non-relevant studies. The PICOT question asked was, “Does exposure to bisphosphonates and other antiresorptives cause occurrence of MRONJ in fibrous dysplasia and fibrous dysplasia/McCune-Albright syndrome patients followed up for at least 1 year?” Results: Eight eligible articles were included in the quantitative synthesis after articles were screened using a PRISMA flowchart. There were 12 reported occurrences of MRONJ among a combined total of 312 fibrous dysplasia and fibrous dysplasia/McCune-Albright syndrome patients (3.85%). Conclusion: Patients with fibrous dysplasia or fibrous dysplasia/McCune-Albright syndrome have a low incidence of MRONJ and may apparently have low susceptibility to spontaneous development of MRONJ. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Monostotic fibrous dysplasia is a benign proliferation of fibrous and osseous tissues that expand medullary bone to cause symptoms due to compression of adjacent organs and anatomical structures. Focal seizures are rarely the first sign of this kind of lesion. This report describes a young female patient with left-sided focal motor seizures associated with fibrous dysplasia presenting as a mass in the right parietal bone. Case Report: An 18-year-old female student with left-sided focal motor seizures presented with a mass in the right parietal bone. Computed tomography revealed an expansile mixed-density lesion on the right parietal bone, a relatively homogeneous ground-glass appearance in the outer circumferential portion, and a lucent eccentric area with thinned but sclerotic borders. Magnetic resonance imaging revealed a homogeneously hypointense signal on T1WI, a small hyperintense signal on T2WI, and avid enhancement signal intensity on post-contrast T1. Electroencephalogram showed inter-ictal epileptiform activities derived from the right fronto-central lobe. Surgical en bloc resection with a margin of normal bone and cranioplasty were performed. Histopathology showed features indicative of fibrous dysplasia, including osteoid trabeculae arranged haphazardly in a dense fibroblastic stroma, irregular trabeculae lacking conspicuous osteoblastic rimming, and intervening fibrous stroma containing cytologically bland spindle cells. The patient achieved seizure control and has remained neurologically intact. Conclusions: This report has highlighted the importance of early diagnosis of fibrous dysplasia of bone to exclude primary bone malignancy or bone metastasis, to ensure rapid management and symptom control. [ABSTRACT FROM AUTHOR]

Background: Pleural solitary fibrous tumors (pSFTs) are rare mesenchymal pleural tumors with rich vascularity. Surgical resection is the cornerstone of pSFTs treatment, requiring careful preoperative imaging to delineate lesion extent and vascular supply including contrast-enhanced computed tomography and other examinations depending on its size and characteristics. Case presentation: The patient was a 34-year-old female with a mass measuring approximately 67 × 42 × 65 mm in the left posterior mediastinum. Intraoperatively, the mass demonstrated rich vascularity. Two veins originating from the abdominal cavity entered the lower pole, one converged from the superior pole, draining into the brachiocephalic vein. Additionally, two arteries arose directly from the descending aorta, while several veins drained into the intercostal veins. In response to unexpected intraoperative vascular findings, vascular clips and silk threads were used to ligate them. Subsequently, the tumor was successfully dissected, with approximately 600 ml of blood loss recorded during the 4-hour surgery. The patient exhibited a satisfactory postoperative recovery, and follow-up spanning over six months revealed no indications of recurrence or metastasis. Conclusions: We firstly present a case of successful resection of a pSFT in a 34-year-old woman with a distinct feeding vessel arising from the descending aorta and describe the related surgical procedures. This case highlights preoperative evaluation of mass vascularity based on contrast-enhanced computed tomography. When blood supply is challenging to clarify, angiography can offer additional details, especially for giant pSFTs. Despite this, thorough intraoperative exploration remains essential to detect unexpected vessels. Appropriate interventions should be customized based on the vascular origins and the surrounding anatomical structures. [ABSTRACT FROM AUTHOR]

Introduction: Fibrosing mediastinitis (FM) is a rare disease characterized by excessive proliferation of fibrous tissue in the mediastinum and can cause bronchial stenosis, superior vena cava obstruction, pulmonary artery and vein stenosis, etc. Case presentation: An aging patient with intermittent chest tightness and shortness of breath was diagnosed with FM associated pulmonary hypertension (FM-PH) by echocardiography and enhanced CT of the chest, and CT pulmonary artery (PA)/ pulmonary vein (PV) imaging revealed PA and PV stenosis. Selective angiography revealed complete occlusion of the right upper PV, and we performed endovascular intervention of the total occluded PV. After failure of the antegrade approach, the angiogram revealed well-developed collaterals of the occluded RSPV-V2b, so we chose to proceed via the retrograde approach. We successfully opened the occluded right upper PV and implanted a stent. Conclusions: This report may provide new management ideas for the interventional treatment of PV occlusion. [ABSTRACT FROM AUTHOR]

Background: Focal and florid cemento-osseous dysplasia are benign fibro-osseous lesions affecting the quality and quantity of the jawbones. This study aimed to determine the viability of implant-based approaches in the affected patients. Main text: Different scientific databases, including PubMed/MEDLINE, Scopus, Web of Science, Embase, the Cochrane Library, and Google Scholar, were searched until October 8, 2023, using a pre-determined search strategy. Two reviewers screened the retrieved reports and extracted the required information from the included studies. The eligibility criteria included English-language case reports/series or clinical trials. The JBI critical appraisal checklist for case reports was used to assess the methodological quality of the included studies. Three studies were deemed eligible to be included in this study out of the initial 202 records found. Five implants were placed in three patients, positioned in the proximity of the lesion area, without any additional treatment to remove the pathology. The mandibular posterior area was the affected site in all patients. Only one implant failed in one patient after 16 years, which was attributed to peri-implantitis and not the lesion. Other implants demonstrated successful maintenance over follow-up periods. Conclusions: Although the number of the included records was relatively low to draw firm conclusions, it seems that implant-based treatments in patients with focal/florid cemento-osseous dysplasia could be viable, considering a conservative and well-planned approach. [ABSTRACT FROM AUTHOR]

Pediatric cavernous sinus meningiomas are rare. Herein, we present the case of a 5-year-old boy who presented with progressive left oculomotor nerve palsy. A head magnetic resonance imaging revealed a mass lesion in the left cavernous sinus, following which tumor resection was performed. The tumor strongly adhered to the left oculomotor nerve without adherence to the walls of the cavernous sinus; however, its border with the nerve was unclear. Histopathological analysis led to the diagnosis of fibrous meningioma. To the best of our knowledge, this is the first case study to report the pediatric development of a cavernous sinus meningioma in the oculomotor nerve. [ABSTRACT FROM AUTHOR]

This article, titled "Painful Benign Bone Tumors of the Hip: A Pictorial Review," aims to explore the role of imaging in diagnosing and treating painful benign bone tumors near the hip joint. The proximal femur is a common location for these tumors, including osteoid osteomas, osteoblastomas, solitary bone cysts, aneurysmal bone cysts, fibrous dysplasia, and cartilaginous tumors. Symptoms of these tumors are often nonspecific, leading to a delay in diagnosis. Imaging techniques such as plain films, ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) are used to evaluate these tumors and guide treatment. Minimally invasive percutaneous procedures have also become an alternative to open surgery for treating these tumors. [Extracted from the article]

Purpose: Fibrous dysplasia (FD) is a rare genetic disease with benign bone tumors. FD can affect one (monostotic FD) or multiple bones (polyostotic FD), with craniofacial lesions being common. Because of its rarity, there are only few clinical reports on FD in the head and neck region and its clinical characteristics remain incompletely defined. This study aimed to determine patient demographics, symptoms, diagnostics, and given treatment in patients with FD of the head and neck in a Finnish population. Methods: A retrospective review on all patients diagnosed with or treated for FD of the head and neck at the Helsinki University Hospital during 2005–2020. Results: In total 74 patients were identified; 54% were male and the mean age 45 years. Overall 95% had monostotic FD. Mandibula and maxilla were the most common anatomic sites. Majority of patients had symptoms, most commonly pain and lesion growth, and 49% had extra-skeletal symptoms. For all, diagnosis was primarily based on imaging findings, biopsies were obtained from 41%. Altogether 54 patients (73%) were managed by observation only, 20 patients (27%) received treatment; ten bisphosphonates, six surgery and four both. Conclusion: Although highly variable in its clinical manifestations, head and neck FD lesions are often symptomatic and impose risk for extra-skeletal complications. Treatment is often conservative but should be individually tailored. Future studies are encouraged to better define the disease characteristics and hopefully offer new treatment possibilities. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumors (SFTs) belonging to a distinct group of mesenchymal tumors, was originally described by Klemper and Rabin in the pleura in year 1931. However, it can also be extra-pleural in origin. With tongue being the most common site involved in this region, epiglottis, larynx, thyroid, external auditory canal, lacrimal sac, hypoglossal nerve, parotid gland, sublingual gland, Para pharyngeal space, nasopharynx, scalp, gingiva, orbit and infratemporal fossa as well as paranasal sinuses and nasal cavities can also get involved. But SFTs involving nasal cavities and nasopharynx are quite uncommon, accounting for < 0.1% of all Sino-nasal neoplasms. Until now there have only been 40 cases of nasal SFT reported in literature. We report the case of an extraserosa solitary fibrous tumor arising from the nasal cavity with extension to the sphenoid sinus, a much rarer presentation of its type. Our case report is one of its type, emphasizing the need conducting further studies on the nature and management of the disease. [ABSTRACT FROM AUTHOR]

A 77-year-old Asian female with a history of left orbit exenteration and lid-sparing reconstruction for recurrent sebaceous carcinoma presented with fluid-like sensation of the left orbit. Magnetic resonance imaging (MRI) demonstrated bright T2 signal and a cyst-like cavity within the exenterated orbit. Decision was made to proceed with surgical exploration and excision. A calcified, bone-like cavity was encountered intraoperatively and removed. Histopathology revealed dense fibrous connective tissue with areas of calcification without osseous metaplasia, suggestive of retained blood in the orbit that underwent dystrophic calcification. This case report illustrates a rare occurrence of a bone-like calcific cyst following exenteration. [ABSTRACT FROM AUTHOR]

A 70-year-old male presented with diplopia and painless proptosis of the left eye for 5 months. Examination showed 6 mm of axial proptosis and restriction of supraduction, abduction and adduction, and mild limitation of infraduction of the left eye. Magnetic resonance imaging demonstrated a large, moderately well-circumscribed intraconal mass in the left lateral orbit, and excisional biopsy was performed. Histopathologic features of mixed fibrous and myxoid areas in a whorl-like pattern and immunohistochemical staining for MUC4 confirmed the diagnosis of low-grade fibromyxoid sarcoma (LGFMS). Next-generation sequencing revealed genetic fusion of EWSR1-CREB3L1. LGFMS is an extremely rare neoplasm with only two prior documented cases of orbital involvement. Here, we report the third case of orbital LGFMS. [ABSTRACT FROM AUTHOR]

The World Health Organization's (WHO) 2022 update on the classification of odontogenic and maxillofacial bone tumors has revolutionized diagnostic and treatment paradigms by integrating novel molecular insights. Fibro-osseous lesions of the maxillo-facial bones constitute a heterogeneous group encompassing fibrous dysplasia, Psammomatoid Ossifying Fibroma (PSOF), Juvenile Trabecular Ossifying Fibroma (JTOF), and other variants. Despite histological similarities, their distinct clinical manifestations and prognostic implications mandate precise differentiation. The intricacies of diagnosing fibro-osseous lesions pose challenges for pathologists, maxillofacial surgeons, dentists and oral surgeons, underscoring the importance of a systematic approach to ensure optimal patient management. Herein, we present two cases, fibrous dysplasia and Cemento-Ossifying Fibroma, detailing their clinical encounters and management strategies. Both patients provided informed consent for publishing their data and images, adhering to ethical guidelines. [ABSTRACT FROM AUTHOR]

Purpose: We report a case of fibrous dysplasia (FD) with aneurysmal bone cyst (ABC)-like change in a child with orbital involvement, review the related cases, and discuss clinical features, therapy, and prognosis of this disease. Case presentation. A 10-year-old girl had right proptosis (degree of exophthalmos: OD 16 mm, OS 13 mm) and limited vision (visual acuity: OD 1.0, OS 0.8) without trauma. Preoperative CT showed a 5.0*4.3 cm right-sided crania-orbital communicating tumor. MRI indicated a well-defined multicystic mass with scattered fluid levels and soap bubble-like alterations. The child underwent total tumor resection and orbital parietal titanium mesh reconstruction. At 20 months of follow-up, the child has recovered from ocular problems, and the tumor has not recurred. Conclusion: FD combined with ABC rarely occurs in orbit and generally begins with ocular symptoms. The etiology is uncertain. Early diagnosis and surgery are essential. Complete resection is suggested whenever possible because residual lesions may recur. [ABSTRACT FROM AUTHOR]

Hedgehogs, as exotic species, are more susceptible to various neoplastic conditions affecting diverse bodily systems, particularly the tegumentary, hemolymphatic, and digestive systems. Among these conditions, epithelial tumors are the most prevalent, followed by round cell tumors and mesenchymal tumors. A striking characteristic is the malignant nature of over 8% of these tumors, leading to a generally unfavorable prognosis. This study aims to present a unique case involving a 2.5 year-old male African pygmy hedgehog in Concepción, Biobío District, Chile, diagnosed with a mesenchymal neoplasia originating from mesothelial cells. The hedgehog presented to the veterinary clinic with acute abdominal pain, prompting ultrasound imaging, and comprehensive cytological, histopathological, and immunohistochemical analyses. During abdominal ultrasound, a mass was observed, and its cytological examination revealed the presence of malignant cells. The histopathological examination unveiled a diffuse mesothelial cell tissue interwoven with abundant fibrous tissue and small cysts containing serous fluid, all enveloped by flattened or cuboidal cells of mesothelial origin. Immunohistochemistry further confirmed the diagnosis, demonstrating positive immunostaining for calretinin and mesothelin markers, corroborating the diagnosis of fibrous malignant peritoneal mesothelioma. This case highlights the complexity of neoplastic conditions in hedgehogs and emphasizes the importance of multimodal diagnostic approaches for accurate identification and understanding of these rare diseases. [ABSTRACT FROM AUTHOR]

Objectives: Cranioplasty is a complex craniofacial and neurosurgical procedure that aims to reinstate the architecture of the cranial vault and elevate both its aesthetic and neurological function. Several reconstructive materials have been thoroughly explored in the search for the optimal solution for cranioplasty. This study aimed to evaluate different material used for cranial reconstruction in Oman. Methods: This retrospective study included all patients who had had cranioplasty procedures performed at Khoula Hospital, Muscat, Oman, from 2012 to 2022. Demographic information, the characteristics of the cranial defect and any complications that occurred post-operatively were analysed. Results: A total of 47 patients were included in this study. The most common cause of cranial defects was craniectomy following traumatic head injury (70.2%) along with excision of fibrous dysplasia (10.6%). The most frequently utilised material for cranial repair was autologous bone grafts (n = 28), followed by polyetheretherketone (PEEK; n = 14). Interestingly, the replacement of bone grafts from previous craniectomy showed a notably high resorption rate (71.4%), in contrast to split calvarial grafts (0%) and other types of bone grafts (14.3%). Additionally, delayed graft infection was observed in 3.6% of the bone graft group and 7.1% of the PEEK group. Conclusion: Patient-specific alloplastic implants such as PEEK have gained popularity for large and complex cranioplasty, as they provide excellent aesthetic outcomes and leave no donor site morbidity. In contrast, bone grafts remain the gold standard for small to medium-sized cranial defects. [ABSTRACT FROM AUTHOR]

Key Clinical Message: We present a rare case of a female pelvic solitary fibrous tumor unsuccessfully resected using single‐port laparoscopy, requiring conversion to laparotomy. Although the resection was successful, the surgical approach could have been improved. For large tumors, minimally invasive results are possible with flexible choices of equipment and incision position. [ABSTRACT FROM AUTHOR]

This report documents the simultaneous occurrence of aneurysmal bone cysts and fibrous dysplasia in a neonatal calf's mandibular bone. The mandibular bones exhibited significant protrusion, cystic cavities, and thin woven bone spicules. Additionally, the bone trabeculae were underdeveloped with necrotic changes, and there was an absence of bone marrow and osteoblastic rimming in the woven bone. Aneurysmal bone cysts were characterized by blood-filled cystic spaces lacking epithelial or endothelial cells, separated by fibroblastic septa. In conclusion, a unique case of mandibular fibrous dysplasia displaying certain similarities to cherubism and ABC has been recorded in a calf. [ABSTRACT FROM AUTHOR]

Context Fibrous dysplasia (FD) results in fractures, pain, and deformities. Abnormal osteoprogenitor cells overproduce FGF23, leading to hyperphosphaturia in most patients and frank hypophosphatemia in a subset. Studies suggest hypophosphatemia is associated with increased FD-related morbidity. However, the relationship between phosphorus and skeletal complications has not been investigated, and the optimal therapeutic target has not been determined. Objective Characterize the impact of serum phosphorus on FD-related morbidity and identify levels associated with increased skeletal complications. Methods Natural history study with 240 subjects at a clinical research center who had ≥1 fasting phosphorus level, determined as age- and sex-adjusted Z-scores. Subjects were categorized based on frank hypophosphatemia (Z-score ≤ −2; n = 48); low-normophosphatemia (> −2 to ≤ −1; n = 66); and high-normophosphatemia (> −1 to ≤ 2; n = 125). Main outcomes were fractures, orthopedic surgeries, and scoliosis. Results Subjects with frank and low-normophosphatemia had increased fracture and surgery rates vs high-normophosphatemia. The prevalence of moderate to severe scoliosis was similarly higher in the frank and low-normophosphatemia groups. In a subanalysis of patients matched for Skeletal Burden Score ≥35, fracture and surgery rates remained higher in the frank hypophosphatemia group, suggesting association between phosphorus and skeletal complications is not explained by differences in FD burden alone. Conclusion Both frank hypophosphatemia and low-normophosphatemia are associated with increased FD-related complications. This supports FGF23-mediated hypophosphatemia as a driver of skeletal morbidity, which may impact a larger proportion of the FD/McCune-Albright syndrome population than previously recognized. These findings enable clinicians to identify at-risk patients and will inform development of prospective studies to determine optimal therapeutic targets. [ABSTRACT FROM AUTHOR]