-
Medical Oncology (Northwood, London,... Apr 2023Ameloblastoma in 66% of the cases harbor a somatic mutation of the "mitogen-activated protein kinase" signaling pathway (BRAF V600E). In V600E mutations, BRAF is in the... (Review)
Review
BACKGROUND
Ameloblastoma in 66% of the cases harbor a somatic mutation of the "mitogen-activated protein kinase" signaling pathway (BRAF V600E). In V600E mutations, BRAF is in the permanent "on" state and relays the growth-promoting signals independently of the EGFR pathway. Therefore, mutant BRAF represents a target for handful of new drugs.
METHODS
We conducted a literature search, with the search terms "Vemurafenib, Dabrafenib, Ameloblastoma, and BRAF." These included seven case reports with nine patients who underwent monotherapy with Dabrafenib or Vemurafenib or combination therapy with Dabrafenib and Trametinib.
RESULTS
The patients age ranges from 10 years up to 86 years. The distribution of women and men is 4:5. Patients with an initial diagnosis of ameloblastoma, as well as recurrences or metastasized ameloblastoma were treated. Indications cover neoadjuvant therapy up to the use in metastasized patients in an irresectable state. Results ranging from "only" tumor size reduction to restitutio ad integrum.
CONCLUSION
We see the use of BRAF Inhibitors to reduce tumor size with consecutive surgical treatment as a reasonable option for therapy. However, we are aware that at present the data are based only on case reports with the longest follow-up of just 38 months. We encourage further clinical trials in the use of BRAF Inhibitors for selecting ameloblastoma patients in a multi-center setting.
Topics: Male; Humans; Female; Child; Vemurafenib; Proto-Oncogene Proteins B-raf; Ameloblastoma; Imidazoles; Protein Kinase Inhibitors; Mutation; Antineoplastic Combined Chemotherapy Protocols
PubMed: 37115331
DOI: 10.1007/s12032-023-01993-z -
Dento Maxillo Facial Radiology Mar 2020This study aimed to search for scientific evidence concerning the accuracy of computer-assisted analysis for diagnosing maxillofacial radiolucent lesions. (Meta-Analysis)
Meta-Analysis
OBJECTIVES
This study aimed to search for scientific evidence concerning the accuracy of computer-assisted analysis for diagnosing maxillofacial radiolucent lesions.
METHODS
A systematic review was conducted according to the statements of Preferred Reporting Items for Systematic Reviews and Meta-analyses Protocols and considering 10 databases, including the gray literature. Protocol was registered at the International Prospective Register of Systematic Reviews (CRD42018089945). The population, intervention, comparison and outcome strategy was used to define the eligibility criteria and only diagnostic test studies were included. Their risk of bias was assessed by the Joanna Briggs Institute Critical Appraisal tool. Random-effects model meta-analysis was performed and heterogeneity among the included studies was estimated using the I statistic. The grade of recommendation, assessment, development, and evaluation (GRADE) tool assessed the quality of evidence and strength of recommendation across included studies.
RESULTS
Out of 715 identified citations, four papers, published between 2009 and 2017, fulfilled the criteria and were included in this systematic review. A total of 191 lesions, classified as periapical granuloma and cyst, dentigerous cyst or keratocystic odontogenic tumor, were analyzed. All selected articles scored low risk of bias. The pooled accuracy estimation, regardless of the classification method used, was 88.75% (95% CI = 85.19-92.30). Heterogeneity test reached moderate values (I = 57.89%). According to the GRADE tool, the analyzed outcome was classified as having low level of certainty.
CONCLUSIONS
The overall evaluation showed all studies presented high accuracy rates of computer-aided diagnosis systems in classifying radiolucent maxillofacial lesions compared to histopathological biopsy. However, due to the moderate heterogeneity found among the studies included in this meta-analysis, a pragmatic recommendation about the use of computer-assisted analysis is not possible.
Topics: Biopsy; Dentigerous Cyst; Diagnosis, Computer-Assisted; Humans; Image Processing, Computer-Assisted; Odontogenic Tumors
PubMed: 31709811
DOI: 10.1259/dmfr.20190204 -
World Journal of Clinical Cases Mar 2022Bone grafts have been applied for many years in orthopedic surgery to assist with bone repair for defects or bone discontinuity caused by trauma and tumors as well as...
BACKGROUND
Bone grafts have been applied for many years in orthopedic surgery to assist with bone repair for defects or bone discontinuity caused by trauma and tumors as well as periodontal defects. Jaw cysts are another common benign disease of the maxillofacial region which may lead to pathological bone fracture, loss of teeth, and infection. However, whether bone grafts are beneficial for bone regeneration in jaw cystic lesions and when bone grafts should be used remains unclear.
AIM
To study the efficacy of bone grafts compared to spontaneous healing in the treatment of jaw cystic lesions.
METHODS
A literature search was performed in Medline, Cochrane Library and Embase to identify related articles published in English in the last ten years. The following key words and MeSH terms were used: "jaw cyst", "cystic lesion", "odontogenic cyst", "periapical cyst", "dentigerous cyst", "follicular cyst", "keratocyst", "treatment", "surgery", "bone graft", "enucleation", "cystectomy", and "bone regeneration". Case reports, clinical trials, clinical studies, observational studies and randomized controlled trials were included. Study quality was evaluated.
RESULTS
Ten studies ( = 10) met the inclusion criteria. Five studies reported spontaneous bone healing after enucleation, three studies investigated the efficacy of various bone grafts, and two randomized comparative studies focused on the comparison between spontaneous healing and bone grafting. Over 90% of bone regeneration occurred within 6 mo after bone grafting. The bone regeneration rate after cystectomy showed great variation, ranging from 50% to 100% after 6 mo, but reaching over 90% after 12 mo.
CONCLUSION
While the long-term superiority of bone grafting compared with spontaneous healing after cystectomy is unclear, bone grafts accelerate the process of healing and significantly increase bone quality.
PubMed: 35434117
DOI: 10.12998/wjcc.v10.i9.2801 -
World Journal of Clinical Oncology Jan 2020Ameloblastomas are common benign epithelial odontogenic neoplasms that present an aggressive and unpredictable behavior that may modify treatment strategies. Different...
BACKGROUND
Ameloblastomas are common benign epithelial odontogenic neoplasms that present an aggressive and unpredictable behavior that may modify treatment strategies. Different signaling pathways that participate in the progression of these tumors have been identified. B-raf proto-oncogene serine/threonine kinase (BRAF) is a protein involved in the behavior of ameloblastomas, and it is related to many cell mechanisms. BRAF gene mutations have been identified in ameloblastomas, of which the BRAF V600E (valine substituted by glutamic acid at amino acid 600) mutation has been the most common and can be present concomitantly with other mutations that may be involved in its behavior. Targeted therapies have been used as an alternative in the case of resistance or contraindications to conventional treatments.
AIM
To document the presence of BRAF V600E and additional mutations, their behavior, and targeted therapies in these tumors.
METHODS
An electronic literature search was conducted according to PRISMA guidelines in PubMed/MEDLINE, Cochrane, EMBASE, and SpringerLink using the terms "ameloblastomas", "BRAF V600E", "additional mutations", and "targeted therapies". Ameloblastomas were classified according to WHO guidelines. Inclusion criteria were articles in English, published not more than 10 years ago, and studies with laboratory works related to BRAF V600E. Articles were evaluated by two independent reviewers and retrieved for full-text evaluation. The EBLIP Critical Appraisal Checklist was used to evaluate the quality of the eligible studies. Descriptive statistical analysis was performed.
RESULTS
Two independent reviewers, with a substantial concordance indicated by a kappa coefficient of = 0.76, evaluated a total of 19 articles that were included in this study. The analysis registered 521 conventional ameloblastomas (AM), 81 unicystic ameloblastomas (UA), 13 ameloblastic carcinomas (AC), three metastatic ameloblastomas (MA), and six peripheral ameloblastomas (PA), of which the histopathological type, anatomic location, laboratory tests, expression of BRAF mutation, and additional mutations were registered. The BRAF V600E mutation was found in 297 AM (57%), 63 UA (77.7%), 3 AC (23%), 1 MA (50%), and 5 PA (83.3%). Follicular type predominated with a total of 116 cases (40%), followed by plexiform type with 63 cases (22.1%). Furthermore, both types presented additional mutations, in which alterations in JAK3 P132T, SMARCB1, PIK3CA, CTNNB1, SMO, and BRAF G606E genes were found. Four case reports were found with targeted therapy to BRAF V600E.
CONCLUSION
The identification of BRAF V600E and additional mutations as an aid in targeted therapies has been a breakthrough in alternative treatments of ameloblastomas where surgical treatments are contraindicated.
PubMed: 31976308
DOI: 10.5306/wjco.v11.i1.31 -
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 -
Medicina Oral, Patologia Oral Y Cirugia... Jan 2021The aim of this study was to investigate the clinicopathologic features of primary intraosseous squamous cell carcinoma arising from an odontogenic keratocyst (PIOSCC ex...
BACKGROUND
The aim of this study was to investigate the clinicopathologic features of primary intraosseous squamous cell carcinoma arising from an odontogenic keratocyst (PIOSCC ex OKC) and comprehensively improve the understanding of this disease.
MATERIAL AND METHODS
We retrospectively investigated five cases of PIOSCC ex OKC at Peking University School and Hospital of Stomatology. We also conducted a systematic review of studies on PIOSCC ex OKC by using online databases from their inception until February 2020.
RESULTS
In our series of five cases, all lesions were located in the mandible. Three cases (60%) showed recurrent OKCs and two cases (40%) showed primary OKCs. During the follow-up period, one patient died of local relapse. No patients developed metastasis. On the basis of our literature survey, we selected 22 articles reporting 29 patients with PIOSCC ex OKC. Seven of these patients (24.1%) showed local recurrence, three patients (10.3%) developed cervical metastasis, three patients (10.3%) developed distant metastasis (in the pleura in one case and in the lung in two cases), and seven patients died from the disease during the follow-up period. The disease-specific 5-year survival rate in the study group was 53.2%. Through univariate and multivariate analysis, local recurrence was identified as the only significant independent prognostic factor for survival (P < 0.05).
CONCLUSIONS
The results suggest that PIOSCC ex OKC is a rare intermediate-grade malignancy. Although elective neck dissection is typically unnecessary, adequate therapy should be applied to achieve the lowest local recurrence rate possible to ensure a favorable survival rate.
Topics: Carcinoma, Squamous Cell; Head and Neck Neoplasms; Humans; Neoplasm Recurrence, Local; Odontogenic Cysts; Retrospective Studies
PubMed: 33037806
DOI: 10.4317/medoral.23947 -
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 -
PloS One 2012Ameloblastoma is a locally aggressive odontogenic neoplasm. With local recurrence rates reaching 90%, only completeness of excision can facilitate cure. Surgical... (Comparative Study)
Comparative Study Review
BACKGROUND
Ameloblastoma is a locally aggressive odontogenic neoplasm. With local recurrence rates reaching 90%, only completeness of excision can facilitate cure. Surgical clearance has widely been based on pre-operative imaging to guide operative excision margins, however use of intra-operative specimen x-ray or frozen-section has been sought to improve clearance rates, and advanced imaging technologies in this role have been proposed. This manuscript aims to quantify the evidence for evaluating intra-operative resection margins and present the current standard in this role.
METHOD
The current study comprises the first reported comparison of imaging modalities for assessing ameloblastoma margins. A case is presented in which margins are assessed with each of clinical assessment based on preoperative imaging, intra-operative specimen x-ray, intra-operative specimen computed tomography (CT) and definitive histology. Each modality is compared quantitatively. These results are compared to the literature through means of systematic review of current evidence.
RESULTS
A comparative study highlights the role for CT imaging over plain radiography. With no other comparative studies and a paucity of high level evidence establishing a role for intra-operative margin assessment in ameloblastoma in the literature, only level 4 evidence supporting the use of frozen section and specimen x-ray, and only one level 4 study assesses intra-operative CT.
CONCLUSION
The current study suggests that intra-operative specimen CT offers an improvement over existing techniques in this role. While establishing a gold-standard will require higher level comparative studies, the use of intra-operative CT can facilitate accurate single-stage resection.
Topics: Ameloblastoma; Female; Frozen Sections; Histocytochemistry; Humans; Intraoperative Period; Jaw Neoplasms; Neoplasm Recurrence, Local; Surgery, Computer-Assisted; Tomography, X-Ray Computed; Young Adult
PubMed: 23094099
DOI: 10.1371/journal.pone.0047897 -
Indian Journal of Dental Research :... 2018Ameloblastoma is a benign locally aggressive type of odontogenic tumor derived exclusively from the epithelium. Histologically, ameloblastoma is classified into many...
Ameloblastoma is a benign locally aggressive type of odontogenic tumor derived exclusively from the epithelium. Histologically, ameloblastoma is classified into many variants, of which granular cell ameloblastoma (GCA) is a rare type, characterized by nest of large eosinophilic granular cells. This article describes a case of GCA in a 50-year-old female patient with clinical, radiological, and histological features along with a systematic review of the literature.
Topics: Ameloblastoma; Diagnosis, Differential; Female; Granular Cell Tumor; Humans; Jaw Neoplasms; Mandibular Osteotomy; Middle Aged; Photomicrography; Plastic Surgery Procedures; Treatment Outcome
PubMed: 30589015
DOI: 10.4103/ijdr.IJDR_407_17