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Medicine Dec 2017Familial gigantiform cementoma (FGC) is a rare benign autosomal dominant fibrocemento-osseous lesion generally limited to the facial bones, typically in the anterior... (Review)
Review
RATIONALE
Familial gigantiform cementoma (FGC) is a rare benign autosomal dominant fibrocemento-osseous lesion generally limited to the facial bones, typically in the anterior portion of the mandible; it is often associated with abnormalities of the long bones and prepubertal pathologic fractures. Owing to the small number of such patients, a uniform treatment criterion has not been established. This paper presents a patient with FGC who was treated in our department, and offers a systematic review of the patients reported in the literature. Our aim was to explore the treatment strategy for patients with FGC.
PATIENT CONCERNS
Our patient, a 13-year-old boy, presented with a painless enlargement of the mandible first noted 2 years earlier. It had grown rapidly over the preceding 8 months, affecting both his appearance and ability to chew.
DIAGNOSIS
Based on the pathologic, clinical, and radiographic features, FGC was diagnosed.
INTERVENTIONS
Mandibuloectomy was performed. The mandibular defect was immediately reconstructed with his right vascularized iliac crest flap. At the same time, a PubMed search was conducted to identify studies reporting on other patients with FGC.
OUTCOMES
A 3-dimensional computed tomography (3D-CT) scan demonstrated appropriate height of the new alveolar bone. Follow-up results showed recovery of the patient's appearance and mandibular function. He was free of recurrence at 4-year follow-up.
LESSONS
FGC is a rare benign fibrocemento-osseous lesion of the jaws that can cause severe facial deformity. Incomplete removal leads to more rapid growth of the residual lesion. Therefore, extensive resection is a suitable strategy to avoid recurrence. Defects of the facial bones found intraoperatively should be repaired with resort to an appropriate donor site. However, it is important to be aware that patients with FGC always have concomitant abnormalities of skeletal metabolism and structure, as well as a vulnerability to fractures of the long bones of the lower extremity. Therefore, the optimal management strategy should include a review of treatment options for other patients as reported in the literature. An optimal protocol can not only provide sufficient high-quality bone suitable for the reconstruction of bone defects, but also minimize complications and maximize quality of life.
Topics: Adolescent; Bone Transplantation; Cementoma; Diagnosis, Differential; Humans; Ilium; Imaging, Three-Dimensional; Jaw Neoplasms; Male; Mandibular Neoplasms; Mandibular Reconstruction; Surgical Flaps; Tomography, X-Ray Computed
PubMed: 29390315
DOI: 10.1097/MD.0000000000009138 -
Dento Maxillo Facial Radiology Feb 2018Therapeutic assessment of odontogenic myxoma (OM) is poorly standardized. Unidimensional size criteria have shown to be unreliable in therapeutic decision-making. We... (Review)
Review
OBJECTIVES
Therapeutic assessment of odontogenic myxoma (OM) is poorly standardized. Unidimensional size criteria have shown to be unreliable in therapeutic decision-making. We evaluate the size distribution of OM and scan for associated clinicoradiological signs of aggressiveness. Additionally, we evaluate three-dimensional size delineation of OM aiming to improve future therapeutic assessment of this destructive neoplasm.
METHODS
Primarily, we reviewed the database "PubMed" for data concerning the size of OMs as radiologically determined. Afterwards, the impact of age, sex, locularity and location on the size was investigated by χ² test, Student's t-test and regression analysis. Furthermore, we statistically evaluated the impact of size on the occurrence of clinicoradiological signs of aggressiveness. Secondly, we approximated the volume of five unpublished cases of OM by semi-automatic image segmentation of cone-beam CT images.
RESULTS
Multilocular OMs were significantly larger than unilocular ones (p < 0.002). Age (0.042) and multilocularity (<0.002) significantly impacted size. Size was significantly associated with cortical perforation (0.032) and multilocularity (<0.002), further regression analysis revealed tooth resorption (0.019), cortical perforation (0.005) and multilocularity (<0.002) as significant predictors of size. Employing the volume as a mean of comparison, we found that the biggest OM (38.42 ml; multilocular) was 124 times larger than the smallest (0.31 ml; unilocular). However, using the maximum diameter (cm) as a surrogate for size, the biggest lesion (6.3) was only 5.25 times larger than the smallest (1.2).
CONCLUSIONS
Locularity and volumetric size characterization might help in therapeutic decision-making and could help to improve our understanding of OM.
Topics: Cone-Beam Computed Tomography; Humans; Imaging, Three-Dimensional; Myxoma; Odontogenic Tumors; Tumor Burden
PubMed: 29082773
DOI: 10.1259/dmfr.20170262 -
Brazilian Dental Journal 2017Here is described a case of ameloblastic fibrosarcoma (AFS) affecting the posterior mandible of a woman who was treated surgically and recovered without signs of... (Review)
Review
Here is described a case of ameloblastic fibrosarcoma (AFS) affecting the posterior mandible of a woman who was treated surgically and recovered without signs of recurrence or metastasis after 12 years of follow-up. Tumor sections were immunostained for cell cycle, epithelial and mesenchymal markers. Immunohistochemical analysis evidenced high Ki-67 positivity in stromal cells (mean of 20.9 cells/High power field). Epithelial cells displayed strong positivity for p53, p63 and cytokeratin 19. In addition to the case report, a systematic review of current knowledge is presented on the AFS's clinical-demographic features and prognostic factors. Based on the review, 88/99 cases were diagnosed as AFS, 9/99 as ameloblastic fibro-odontosarcoma and 2/99 as ameloblastic fibrodentinosarcoma. All these lesions displayed very similar clinical-demographic and prognostic features. Moreover, the review provided evidence that first treatment, regional metastasis, distant metastasis and local recurrence were significant prognostic values for malignant odontogenic mesenchymal lesions. Based on the findings, segregation among ameloblastic fibrosarcoma, ameloblastic fibrodentinosarcoma and ameloblastic fibro-odontosarcoma seems illogical, considering all these lesions have similar predilections and outcomes.
Topics: Adult; Female; Fibrosarcoma; Humans; Immunohistochemistry; Mandibular Neoplasms; Odontogenic Tumors
PubMed: 28492759
DOI: 10.1590/0103-6440201701050 -
Medicina Oral, Patologia Oral Y Cirugia... Nov 2016The keratocystic odontogenic tumor is a benign but aggressive neoplasm. As enucleation alone obtains high recurrence rates, some adjuvant treatments such as Carnoy's... (Meta-Analysis)
Meta-Analysis
INTRODUCTION AND OBJECTIVE
The keratocystic odontogenic tumor is a benign but aggressive neoplasm. As enucleation alone obtains high recurrence rates, some adjuvant treatments such as Carnoy's solution have been proposed. The aim of this study is to evaluate the reduction of recurrences with the use of Carnoy's solution as adjuvant in the treatment of keratocystic odontogenic tumors.
MATERIAL AND METHODS
An electronic search in Pubmed (MEDLINE), ScienceDirect and Cochrane databases was conducted with the key words "odontogenic keratocyst", "keratocystic odontogenic tumor", "carnoy's solution", "treatment" and "enucleation". The inclusion criteria were clinical studies using Carnoy's solution as adjuvant for the treatment of keratocystic odontogenic tumors, published in English, including at least 10 patients. Articles with an unclear reporting of the treatment applied, nonhuman studies, case reports and lesions associated to Gorlin-Goltz syndrome were excluded.
RESULTS
All the studies included were case series. The recurrence rate of enucleation ranged from 0% to 58.8%. With the only use of Carnoy's solution as adjuvant treatment to the enucleation, recurrences varied from 0% to 100%. The use of ≥ 2 adjuvant treatments reduced the range between 0% and 7.9%.
CONCLUSIONS
The use of Carnoy's solution as adjuvant therapy for the treatment of keratocystic odontogenic tumor has a grade C recommendation.
Topics: Acetic Acid; Chloroform; Ethanol; Humans; Neoplasm Recurrence, Local; Odontogenic Tumors
PubMed: 27475699
DOI: 10.4317/medoral.21250 -
The Cochrane Database of Systematic... Nov 2015The keratocystic odontogenic tumours (KCOTs) account for between about 2% and 11% of all jaw cysts and can occur at any age. They are more common in males than females... (Review)
Review
BACKGROUND
The keratocystic odontogenic tumours (KCOTs) account for between about 2% and 11% of all jaw cysts and can occur at any age. They are more common in males than females with a male:female ratio of approximately 2:1. Although they are benign, KCOTs are locally very aggressive and have a tendency to recur after treatment. Reported recurrence rates range from 3% to 60%. The traditional method for the treatment of most KCOTs is surgical enucleation. However, due to the lining of the cyst being delicate and the fact that they frequently recur, this method alone is not sufficient. Adjunctive surgical treatment has been proposed in addition to the surgical enucleation, such as removal of the peripheral bone (ostectomy) or resection of the cyst with surrounding bone (en-bloc) resection. Other adjunctive treatments proposed are: cryotherapy (freezing) with liquid nitrogen and the use of the fixative Carnoy's solution placed in the cyst cavity after enucleation; both of which attempt to address residual tissue to prevent recurrence.
OBJECTIVES
To assess the available evidence comparing the effectiveness of interventions for the treatment of KCOTs.
SEARCH METHODS
We searched the following electronic databases: the Cochrane Oral Health Group Trials Register (to 17 March 2015), the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, 2015, Issue 2), MEDLINE via Ovid (1946 to 17 March 2015) and EMBASE via Ovid (1980 to 17 March 2015). We searched the US National Institutes of Health Trials Register (http://clinicaltrials.gov) and the WHO Clinical Trials Registry Platform for ongoing trials. No restrictions were placed on the language or date of publication when searching the electronic databases.
SELECTION CRITERIA
Randomised controlled trials comparing one modality of intervention with another with or without adjunctive treatment for the treatment of KCOTs. Adults, over the age of 18 with a validated diagnosis of solitary KCOTs arising in the jaw bones of the maxilla or mandible. Patients with known Gorlin syndrome were to be excluded.
DATA COLLECTION AND ANALYSIS
Review authors screened trials for inclusion. Full papers were obtained for relevant and potentially relevant trials. If data had been extracted, it would have been synthesised using the fixed-effect model, if substantial clinical diversity were identified between studies we planned to use the random-effects model with studies grouped by action provided there were four or more studies included in the meta-analysis, and we would have explored the heterogeneity between the included studies.
MAIN RESULTS
No randomised controlled trials that met the inclusion criteria were identified.
AUTHORS' CONCLUSIONS
There are no published randomised controlled trials relevant to this review question, therefore no conclusions could be reached about the effectiveness or otherwise of the interventions considered in this review. There is a need for well designed and conducted randomised controlled trials to evaluate treatments for KCOTs.
Topics: Adult; Female; Humans; Male; Mandibular Diseases; Maxillary Diseases; Odontogenic Cysts; Odontogenic Tumors
PubMed: 26545201
DOI: 10.1002/14651858.CD008464.pub3 -
PloS One 2015Ameloblastoma is the second most common odontogenic tumor, known to be slow-growing, persistent, and locally aggressive. Recent data suggests that ameloblastoma is best... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Ameloblastoma is the second most common odontogenic tumor, known to be slow-growing, persistent, and locally aggressive. Recent data suggests that ameloblastoma is best treated with wide resection and adequate margins. Following primary excision, bony reconstruction is often necessary for a functional and aesthetically satisfactory outcome, making early diagnosis paramount. Despite earlier diagnosis potentially limiting the extent of resection and reconstruction, an understanding of the growth rate and natural history of ameloblastoma has been notably lacking from the literature.
METHOD
A systematic review of the literature was conducted by reviewing relevant articles from PubMed and Web of Science databases. Each article's level of evidence was formally appraised according to the Centre of Evidence Based Medicine (CEBM), with data from each utilized in a meta-analysis of growth rates for ameloblastoma.
RESULTS
Literature regarding the natural history of ameloblastoma is limited since the tumor is immediately acted upon at its initial detection, unless the patient voluntarily refuses a surgical intervention. From the limited data, it is derived that the highest estimated growth rate is associated with solid, multicystic type and the lowest rate with peripheral ameloblastomas. After meta-analysis, the calculated mean specific grow rate is 87.84% per year.
CONCLUSION
The growth rate of ameloblastoma has been demonstrated, offering prognostic and management information, particularly in cases where a delay in management is envisaged.
Topics: Adult; Aged; Ameloblastoma; Female; Humans; Jaw Neoplasms; Male; Middle Aged; Young Adult
PubMed: 25706407
DOI: 10.1371/journal.pone.0117241