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Journal of Pediatric Neurosciences 2021Skull base tumors are varied in children and are particularly challenging to pediatric neurosurgeons, with few papers in the literature describing the evolution,...
CONTEXT
Skull base tumors are varied in children and are particularly challenging to pediatric neurosurgeons, with few papers in the literature describing the evolution, complications, and outcome. The authors evaluated long-term outcomes in children submitted to skull base tumor surgery and performed a literature review.
AIMS
The aim of this study was to analyze surgical results, complications, and outcomes, on comparison with previous publications.
MATERIALS AND METHODS
A retrospective analysis of children undergoing surgery at a single institution between 2000 and 2018 for lesions of the cranial base was carried out. In addition, a literature review was carried out describing a total of 115 children operated on for skull base tumors.
STATISTICAL ANALYSIS
Chi-squared and Fisher's exact tests were performed to compare the distribution of categorical variables and a nonparametric Mann-Whitney test was used to perform intergroup comparisons of continuous variables.
RESULTS
Seventeen children ranging in age from 8 months to 17 years (mean, 10.9 years) underwent skull base approaches. Tumor types included schwannoma, meningioma, chondroid chordoma, mature teratoma, epidermoid cyst, hemangiopericytoma, rhabdomyosarcoma, myofibroblastic inflammatory tumor, fibromyxoid sarcoma, Crooke's cell adenoma, ossifying fibroma, osteoblastoma, nasopharyngeal angiofibroma and Ewing's sarcoma. Gross total resection was achieved in 6 patients (35.3%), 12 patients (70.6%) had benign histology, and 5 patients (29.4%) had a malignant tumor. Transient postoperative cerebrospinal fluid leak affected only one patient. Thirteen children (76.4%) had a residual neurological deficit at last follow-up evaluation. Three (17.6%) surviving patients received adjuvant therapy. The rate of recurrence or lesion progression was 17.6%.
CONCLUSIONS
Skull base tumors in children present a therapeutic challenge because of their unique pathological composition and can lead to considerable morbidity and mortality in pediatric age.
PubMed: 34316306
DOI: 10.4103/jpn.JPN_56_20 -
Molecular Imaging and Radionuclide... Jun 2023Fibrous dysplasia (FD) is a rare congenital benign bone disease that manifests as a defect in the bone remodeling process, affecting the function, differentiation, and...
Fibrous dysplasia (FD) is a rare congenital benign bone disease that manifests as a defect in the bone remodeling process, affecting the function, differentiation, and maturation of osteoblasts. This process is located in the bone marrow, where the normal marrow tissue is replaced with immature bone islands and fibrous stroma. The etiology is unclear so far, but it is known to be connected with a point mutation of the gene that encodes Gs α protein at the time of embryogenesis, and because of that, all of the affected somatic cells become dysplastic. It is important to determine whether the mutation occurred earlier in the process of embryogenesis so that there will be more mutant cells and the disease will appear in a more severe form. The clinical presentation of FD is variable, so there are plenty of potential differential diagnoses. The most common include Paget disease, non-ossifying fibroma, osteofibrous dysplasia, aneurysmal bone cyst, adamantinoma, giant cell tumor, fracture callus, and low-grade central osteosarcoma.
PubMed: 37337879
DOI: 10.4274/mirt.galenos.2023.79095 -
International Journal of Surgery Case... Jan 2022Cemento ossifying fibroma (COF) is an encapsulated lesion that is often found in the mandible of middle-aged females. Cystic degeneration may be associated to several...
INTRODUCTION AND IMPORTANCE
Cemento ossifying fibroma (COF) is an encapsulated lesion that is often found in the mandible of middle-aged females. Cystic degeneration may be associated to several osseous lesion. This particular presentation is not well documented in the jaws.
CASE PRESENTATION
Patient presented was referred to oral surgery department for abnormal radiolucency in the right mandible on panoramic X-ray. The patient did not have any specific medical history and reported painful areas in right mandibular region. During diagnosis assessment, ameloblastoma was first considered due to the presence of cystic lesion clusters. But the mixed image in previous X-ray pointed to a fibro-osseous with a change in pathogenesis pathway. Diagnosis buildup was based on previous radiographs, incisional biopsy was proposed to identify the histopathological feature of the lesion before proceeding to further investigation (CBCT) or treatments (decompression or enucleation). COF of the jaws associated to non-specific cystic changes was the histopathological diagnosis. Delay in treatment showed an unexpected outcome with good clinical and bone healing.
CLINICAL DISCUSSION AND CONCLUSION
This case shows the importance of previous radiographs, when available, in diagnosis buildup. The fibro-osseous lesion with cystic change may show a good biological response with conservative management. A clinical and radiological surveillance after a conservative treatment may be a better option in treatment of these lesions.
PubMed: 34954627
DOI: 10.1016/j.ijscr.2021.106676 -
Head and Neck Pathology Dec 2022Ossifying fibroma of the craniofacial bones is a fibro-osseous lesion characterized by varied patterns of bone formation in a fibroblastic stroma. Ossifying fibroma is a...
Ossifying fibroma of the craniofacial bones is a fibro-osseous lesion characterized by varied patterns of bone formation in a fibroblastic stroma. Ossifying fibroma is a putatively benign lesion with no reports of malignant transformation or metastasis. Differentiation from other fibro-osseous lesions can be challenging necessitating synthesis of clinical, radiological and pathological findings. The molecular pathogenesis of ossifying fibroma is poorly understood but recent studies have reported MDM2 gene amplification and chromosomal copy number changes in a subset of ossifying fibromas. MDM2 amplification in ossifying fibroma, if true, presents a diagnostic problem because this genetic event, at least among craniofacial fibro-osseous lesions, was previously considered specific for low-grade osteosarcoma. In the present study, we investigated the utility of MDM2 and CDK4 immunohistochemistry, and fluorescence in situ hybridization for MDM2 gene amplification, in the diagnosis of 44 craniofacial bone ossifying fibromas. Focal MDM2 and CDK4 nuclear immunoreactivity was found in 11 and 1 ossifying fibromas, respectively, but none demonstrated MDM2 amplification by fluorescence in situ hybridization. A single tumor displayed MDM2 amplification without nuclear immunoreactivity to either MDM2 or CDK4. Our data suggest that while focal MDM2 and CDK4 nuclear expression may be detected in a minority of ossifying fibromas, this expression does not correlate with MDM2 amplification. In addition, MDM2 amplification is extremely rare in ossifying fibroma so the detection of this genetic abnormality should continue to raise concern for osteosarcoma.
Topics: Humans; In Situ Hybridization, Fluorescence; Gene Amplification; Proto-Oncogene Proteins c-mdm2; Cyclin-Dependent Kinase 4
PubMed: 35546651
DOI: 10.1007/s12105-022-01454-5 -
Dentistry Journal Jan 2022Peripheral ossifying fibroma (POF) is a benign localized lesion originating from gingival and alveolar oral mucosa. Its origin can be cells of periodontal ligament. The...
Peripheral ossifying fibroma (POF) is a benign localized lesion originating from gingival and alveolar oral mucosa. Its origin can be cells of periodontal ligament. The lesions usually develop in women in their twenties. POF is a complex clinical and histological diagnosis due to its shared characteristics with many other conditions. In this paper, we presented a case of an atypical peripheral ossifying fibroma (POF) in the left lateral part of the mandible in a 70-year-old male patient who had two semicircular bridges supported on four implants in the upper and lower jaws. A review of CBCT and orthopedic imaging showed no visible intraosseous changes. Histological analysis revealed the diagnosis of POF. The case in question is interesting, as elaborated on in the discussion section of this paper because POF is usually found in female patients aged between 20 and 30 years.
PubMed: 35049607
DOI: 10.3390/dj10010009 -
Heliyon May 2023The occurrence of hybrid odontogenic lesions with two or more morphologically distinct components is a rare phenomenon and poses a diagnostic challenge. We aimed to...
BACKGROUND
The occurrence of hybrid odontogenic lesions with two or more morphologically distinct components is a rare phenomenon and poses a diagnostic challenge. We aimed to study the clinical, radiological, and pathological features and behavior of hybrid odontogenic lesions, to enhance awareness about these rare lesions.
METHOD
Hematoxylin and Eosin slides of hybrid odontogenic lesions diagnosed between January 01, 2012 and December 31, 2020, were reviewed. Demographic and radiological information were obtained from the patient's medical records.
RESULTS
8 cases were diagnosed with a mean age of 19.1 years and male to female ratio of 1:1.7. Involvement of mandible was more common (n = 5) as compared to maxilla (n = 3). All patients presented with swelling for an average of 9.75 months (3-25 months) duration. Bleeding, loose teeth, pain and facial asymmetry were reported in 5,3, 3, and 2 cases, respectively. Radiologically, 7 cases were well demarcated, 75% cases (n = 6) were radiolucent, and average radiological size was 4.8 cm. All patients were managed with surgery alone. 5 cases (62.5%) underwent enucleation and curettage, while local excision, en-block resection and segmental mandibulectomy were performed in 1 case each. Histologically, ossifying fibroma/cemento-ossifyiong fibroma were the most lesion, occurring in 5 cases (62%), followed by giant cell granuloma like lesions (GCG) i.e., central and peripheral giant cell granuloma (n = 3), Adenomatoid Odontogenic tumor (AOT) (n = 2), and DC (n = 2), ameloblastic fibroma (AF) (n = 1), Ameloblastoma (n = 1), calcifying odontogenic cyst (COC) (n = 1), and complex odontoma (n = 1). No evidence of recurrence was noted after 4-99 months of surgery (mean: 32.9) in cases with available data (n = 7). Long-term complaints included facial asymmetry (n = 2) and pain (n = 1).
CONCLUSION
Most hybrid odontogenic lesions affect young females in the second decade of life and commonly show COF and OF as hybrid components. A conservative approach to management appears adequate.
PubMed: 37251860
DOI: 10.1016/j.heliyon.2023.e16221 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Aug 2021To analyze the clinical features of juvenile ossifying fibroma in nasal root and to compare different surgical methods. Seven cases of juvenile ossifying fibroma...
To analyze the clinical features of juvenile ossifying fibroma in nasal root and to compare different surgical methods. Seven cases of juvenile ossifying fibroma occurring in the nasal root were treated via surgery, 5 cases were resected under nasal endoscopy guided by Image Guidance System-based electromagnetic navigation, and 2 cases were resected by lateral nasal butterfly incision. Postoperative follow-up included endoscopy and CT scan of the sinuses. The pathological results of 7 patients were consistent with juvenile ossifying fibroma. Follow-up period ranged from 6 to 45 months, and there were no recurrence or surgical complications. Surgery is the only effective treatment. Endoscopic sinus surgery with image navigation and lateral nasal butterfly incision resection has been deemed available. The external nasal butterfly incision has less bleeding and shorter operation time, but with mild nasal face swelling after surgery, and nasal endoscopy is a surgical method with less damage.
Topics: Endoscopy; Fibroma, Ossifying; Humans; Neoplasm Recurrence, Local; Paranasal Sinus Neoplasms; Paranasal Sinuses
PubMed: 34304524
DOI: 10.13201/j.issn.2096-7993.2021.08.001 -
Heliyon Jul 2021Cemento-ossifying fibroma is a benign fibro-osseous lesion of the jaws. Cemento-ossifying fibroma develops from the periodontal ligament and contains multipotent stem...
Cemento-ossifying fibroma is a benign fibro-osseous lesion of the jaws. Cemento-ossifying fibroma develops from the periodontal ligament and contains multipotent stem cells that can form cementum, lamellar bone, and/or fibrous tissue. These tumours occur in the third and fourth decades of life with higher predilection of occurrence in the female population and seldom attain a large size. We report a rare case of cemento-ossifying fibroma in a 45-year-old man involving the body of the mandible and extending into the para-pharyngeal and infratemporal region. This article describes the clinical, radiographic, and histological features of a large cemento-ossifying fibroma of the mandible.
PubMed: 34337187
DOI: 10.1016/j.heliyon.2021.e07594 -
Journal of Surgical Case Reports Jul 2021The ossifying fibroma is a benign fibro-osseous tumor rarely affecting the skull base. The incidence of ossifying fibroma itself is uncommon. It is considered to be an...
The ossifying fibroma is a benign fibro-osseous tumor rarely affecting the skull base. The incidence of ossifying fibroma itself is uncommon. It is considered to be an aggressive and fast-growing bone lesion. Early detection and complete surgical removal are essential to deal with its aggressive nature and recurrence. We report a case of a 20-year-old man admitted for the management of ossifying fibroma of the orbital roof extending inside the orbit mimicking meningioma and revealed by a progressive proptosis and headache. The patient underwent surgery for the subtotal removal of the tumor with its frontal infiltration with a good outcome. All meningiomas like tumors are not meningiomas and other tumors such as ossifying fibroma might be mistaken for meningioma and even get confirmation from the pathological anatomy study. Need be for these tumors to be looked at more closely for better therapeutic decision-making.
PubMed: 34276961
DOI: 10.1093/jscr/rjab304 -
Journal of Cancer 2017: Ossifying fibroma in the jaws is a benign tumor and easily recurs in children, of which the treatment methods and prognosis still remain controversial. In this study,...
: Ossifying fibroma in the jaws is a benign tumor and easily recurs in children, of which the treatment methods and prognosis still remain controversial. In this study, we aimed to review the clinicopathological characteristics, treatment, and prognosis of ossifying fibroma in the jaws of children, and offer recommendations for clinical decision-making. A retrospective study was carried out on patients below the age of 18 years with ossifying fibroma in the jaws. Patients with complete clinical, pathological, and radiological records were included and followed-up. Sixty-three cases were collected with a preliminary search. After screening, fifty patients were included for general information analysis, of which forty-two patients were included in the recurrence analysis. Twelve patients showed a relapse, with a recurrence rate of 28.6% (12/42). The recurrence rates in cases with different surgical approaches and different X-ray boundaries were statistically different. Besides, twenty-three patients underwent reconstruction by free tissue grafting and the success rate was 96% (22/23). There was significant difference in the recurrence rates among different X-ray manifestations and surgical methods. An extended resection and reconstruction with free tissue grafting was a reliable method with a 96% success rate.
PubMed: 29151945
DOI: 10.7150/jca.21556