A family case report of parathyroid carcinoma associated with CDC73 mutation in hyperparathyroidism-jaw tumor syndrome.

Background: Hereditary primary hyperparathyroidism (PHPT) accounts for 5-10% of all PHPT cases, necessitating genetic testing for diagnosis and management. Among these, hyperparathyroidism-jaw tumor syndrome (HPT-JT) is an autosomal dominant disorder caused by CDC73 mutations with variable clinical presentations and incomplete symptoms. Case summary: The proband, diagnosed with PHPT, underwent parathyroidectomy at the age of 41 with pathological examination of parathyroid carcinoma (PC). Hereditary PHPT was initially suspected due to the early-onset PHPT and family history. Genetic testing identified a heterozygous CDC73 mutation, NM_024529.4: c. 687_688delAG (p. Arg229Serfs*37). Even in the absence of jaw tumors, the diagnosis of HPT-JT was confirmed based on the discovery of renal cysts. A secondary thyroidectomy was performed to reduce the risk of recurrence. Conclusion: Genetic testing is strongly recommended in cases of early-onset PHPT, family history, jaw tumors, renal and uterine involvement, atypical parathyroid tumors, and PC. This testing provides valuable information for personalized management, and counseling is available for affected families.

Current concepts in targeted therapies for benign tumors of the jaw - A review of the literature.

The aim of our study was to review current concepts in targeted therapies for benign tumors of the jaw. Benign odontogenic and maxillofacial bone tumors often require radical surgery, with consequent morbidity that impacts patients' postsurgical quality of life. Currently, targeted therapies and novel nonsurgical therapeutics are being explored for management of non-resectable tumors, with the aim of avoiding surgery or minimizing surgical scope. However, data on clinical applications of targeted therapies for benign tumors of the jaw remain sparse. Therefore, a literature review was conducted, based on the PubMed database, which included in vivo human clinical studies describing clinical application of targeted therapy for benign tumor of the jaw. The review assessed the outcomes of BRAF and MEK inhibitors for treatment of ameloblastoma, RANKL monoclonal antibody for treatment of giant cell tumor, cherubism, aneurysmal bone cyst, and fibrous dysplasia, and tyrosine kinase inhibitor for treatment of odontogenic myxoma and cherubism. Targeted therapies decreased tumor size, slowed down tumor progression, and reduced bone pain. Surgery remains the gold standard, but targeted therapies are promising adjuvant or alternative treatment options for reducing tumor progression and morbidity of tumor surgery.

Clinical Characterization of Oral and Maxillofacial Tumors and Tumor-Like Lesions in Children and Adolescents.

PURPOSE: To investigate the clinical characteristics of oral and maxillofacial tumors in children and adolescents. METHODS: This is a retrospective study of patients who had oral and maxillofacial tumors under the age of 18 years and were treated at the Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology from January 1990 to July 2021 (31 y). Their general conditions, pathological diagnosis, gender, age, and anatomical location were counted to analyze their morbidity and composition characteristics. RESULTS: This study contained 5405 cases, including 2903 male patients and 2502 female patients, with a median age of 9 years. Peak incidence was observed in the 14 to 18 years age group. The mandible (22.15%), maxilla (11.75%), and tongue (9.25%) were the most common sites of incidence. Malignant and intermediate type tumors accounted for 13.04%, benign tumors and tumor-like lesions for 55.67%, most often occurs in the maxillofacial bone, of which fibro-osseous lesions constitute an important part. Cysts accounted for 31.29%. Among the tumors occurring in the jaws, the most common malignant type was sarcoma, and ameloblastoma was the most common benign tumor. Malignant jaw tumors were mostly treated by resection, 10.64% by fibular flap reconstruction. While benign jaw tumors and tumor-like lesions were mostly treated by resection or curettage. CONCLUSIONS: The distribution of anatomical location and pathological types of oral and maxillofacial tumors in children has certain characteristics, so that the selection of their treatment options is different from that of adults due to the consideration of the growth and developmental characteristics of children.

The role of folded fibular flap in patients' reconstruction of mandibular defects: a retrospective clinical study.

This study has analyzed 41 patients with mandibular ameloblastoma who underwent a partial mandibulectomy and reconstruction by folding the free fibular flap. In the preoperative and postoperative (6 months and 24 months after surgery), the Quality of Life (QOL) of these patients was assessed by using the University of Washington Quality of Life Questionnaire (UW-QOL) and the medical outcome study short form-36 (SF-36) questionnaires. SPSS 20.0 statistical software was used to conduct statistical analysis on the base data of the two groups of patients. Independent sample t test was conducted for sf-36 and UW-QOL scores at two time points in each group. The SF-36 survey showed that body pain (54.54 ± 8.10), general health (55.27 ± 7.54), and health changes (58.29 ± 9.60) decreased significantly at 6 months after surgery, but the mean score at 24 months after surgery all exceeded the preoperational level. At 24 months after the surgery, the vitality (80.41 ± 3.74), social function (81.61 ± 4.07), emotional role (82.39 ± 4.07), psychological health (81.66 ± 4.37) and total score (704.00 ± 31.53) all returned to the preoperative level, which was statistically significant compared with 6 months after surgery. However, there was no significant difference compared with the preoperative level. The UW-QOL survey showed that chewing (56.68 ± 7.23), speech (54.54 ± 7.7) and taste (62.29 ± 10.15) have significantly changed at 6 months after the surgery, and the difference was statistically significant at 24 months after surgery. Saliva generation decreased slightly (80.76 ± 3.35) at 6 months after surgery, but quickly returned to the preoperative level (81.59 ± 4.06). The total score of the patients almost recovered to the preoperative level at 24 months after surgery. The folded the fibular flap can not only repair the defects of soft tissue and bone tissue, but also restore the height of the alveolar ridge to, avoid the imbalance of crown and root ratio after implantation and reduce the occurrence of peri-implant inflammation, so that a true functional reconstruction can be realized.

A Retrospective Study of 158 Cases on the Risk Factors for Recurrence in Ameloblastoma.

Background: Ameloblastoma is an odontogenic tumor occurring in jaws, with local aggressiveness and postoperative recurrence. This study was aim to investigate the clinical and radiographic risk factors for recurrence in ameloblastoma. Methods: Patients diagnosed with ameloblastoma between March 2009 and March 2019 were retrospectively analyzed. Clinical and Radiological data and follow-up records were collected. Survival analyses were performed by Kaplan-Meier and log-rank tests, as well as Cox proportional hazards model. Results: One hundred and fifty-eight patients (104 males and 54 females were enrolled. The overall recurrence rate for ameloblastoma was 13.29%, and 10.76% recurred within 5 years. Most of the tumors were located in mandible (86.71%), while the rest 21 cases were in maxilla (13.29%). More than half cases (55.06%) showed multilocular radiolucency, 61 cases (38.61%) showed unilocular radiolucency. Significant differences were found with amelobastoma recurrence rate related to treatment modality, impacted tooth and root resorption (P =0.002, 0.022 and 0.007 respectively). Conclusions: Treatment modality, impacted tooth and root resorption all showed statistically significant associations with the recurrence rate in ameloblastoma. However, due to the limitation of this study, further studies are needed to reveal the true mechanism of ameloblastoma recurrence.

Dental Topics for Plastic Surgeons, Part Four: Common Cysts and Tumors of the Jaw.

ABSTRACT: Conditions that affect dental and periodontal structures receive sparse coverage in the plastic surgery literature, yet a working knowledge of this subject matter is important in certain areas of clinical practice and a fundamental understanding is part of plastic surgery competency tested on the in-service and written board examinations. This 4-part series was written to provide plastic surgeons with a working knowledge of dental topics that may be relevant to their clinical practice. This section, Part IV, covers common dental cyst and tumors of the jaw.

A clinicopathologic analysis of 135 cases of cemento-osseous dysplasia: To operate or not to operate?

PURPOSE: The aim of this retrospective study was to investigate the characteristics of cemento-osseous dysplasia (COD) lesions for devising a clinical algorithm to help management decisions, specifically discussing the need for a possible surgical intervention versus solely follow-up. MATERIALS AND METHODS: One hundred thirty-five subjects were thus identified having previously diagnosed and treated for COD from the archives of the pathology in between 2005-2015. These were then re-evaluated. Demographic features, radiographic and histopathologic findings were assessed. RESULTS: The most frequent variant was the focal type (n=83), and the mandible (n=131) was predominantly affected jaw bone. Female predilection was evident for all subtypes. Almost half of the lesions (n=72) presented with mixed radiolucent and radiopaque appearances in radiography at the time of the biopsy. Cementicles/psammoma body-like calcifications and trabecular pattern were the most common hard tissue appearances. CONCLUSION: COD lesions are non-neoplastic fibro-osseous lesions which can exhibit similar histopathologic and radiologic features with other bone lesions. Ideally, these lesions should be clinically identified and followed-up. Considering the possible complications, a thorough knowledge of these lesions and their differentials is essential in order to avoid unnecessary surgical interventions.

Fibula osteal flap with proximal peroneal perforator skin paddle for composite oromandibular reconstruction: A case report.

RATIONALE: Cutaneous perforators of peroneal vessels are divided into proximal and distal perforators on the basis of perforator distributions and musculocutaneous or septocutaneous properties. The traditional fibular osteocutaneous free flap is raised over the distal two-thirds of the fibula with a skin paddle based on distal perforators, which is affixed to the posterior crural septum. However, the skin pedicle may not be available due to anatomic variations or intraoperative injuries. Herein, because of the absence of distal perforators, we reserved and expropriated proximal perforators originating from the musculocutaneous branch of the superior part of the peroneal artery before it divided into nutrient and arcuate arteries and successfully harvested a separate osteal fibula and proximal perforator skin paddle with a single vascular pedicle-peroneal vessel. PATIENT CONCERNS: A 62-year-old man with a 6-month history of mandibular swelling and soft tissue invasion was referred to us. DIAGNOSIS: Panoramic radiography and computed tomography showed an irregular radiolucent lesion of the mandibular body, and histopathological analysis confirmed a follicular-pattern ameloblastoma. INTERVENTIONS: The diseased mandible and soft tissue were resected and reconstructed with a vascularized fibular osteal flap with the proximal perforator skin paddle. OUTCOMES: The mandibular contour was successfully restored; the skin paddle in the mouth was in good condition after 8 months of follow-up. LESSONS: The proximal perforator is reliable and practical for supplying a skin paddle and has significant potential for future applications. We recommend reserving the proximal perforator skin paddle as a backup flap when planning to raise a fibula flap, since unavailability or injury of the traditional fibular skin island based on distal perforators occurs frequently. This approach can avoid the exploration for a second donor site, save surgical time, and reduce surgical complexity. Moreover, we anticipate more frequent use of the proximal perforator flap in the future because of its flexibility and large volume, and since it can be combined with the osteal fibula or fibular osteocutaneous flap. However, an understanding of the traits of the proximal perforator and determination of its peroneal origin by computed tomography angiography is crucial for predesigning fibular osteal flaps with a proximal perforator skin paddle.

Ameloblastoma of the jaws in children: an evaluation of cases seen in a tertiary hospital in South-Eastern Nigeria.

BACKGROUND: Ameloblastoma is one of the most common benign odontogenic tumours in Nigeria. It is considered uncommon in children. MATERIALS AND METHODS: This is a retrospective study of pediatric patients with histopathological diagnosis of ameloblastoma seen over seven years at the Oral and Maxillofacial Surgery Department of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Relevant information was retrieved from patients' records and data obtained were analysed using SPSS version 23, the chi-square test was used to compare qualitative variables, a p-value of <0.05 was considered as significant. RESULTS: One hundred and thirty-six cases of ameloblastoma in all age groups were seen within the period. Thirty of the cases met the requirement. The mean age of the patients was 14.4 STD 2.03 (range from 10-17) years. Fourteen (46.7%) patients were male while 16 (53.3%) were female giving a ratio of 1:1.1. The duration of the lesion ranged from 3 months to 72 months (mean 15.07 months). Histologically, the follicular type (n=20, 66.7%) constitute the majority, while the clinical types were solid-multicystic (n=18, 60%) and unicystic (n=12, 40%). Enucleation was the treatment of choice in most (n=18, (60%)) of the patients. CONCLUSION: Ameloblastoma is relatively uncommon in children, especially those less than ten years of age. The solid-multicystic variety was the predominant type in the children studied. Most patients presented long after the onset of the tumour and enucleation with mechanical curettage produced satisfactory results in these patients. FUNDING: None declared.

Patient specific implants for jawbone reconstruction after tumor resection.

In case of benign and malignant tumours affecting the maxillofacial region, the resection of jawbone reflects the standard therapy in more than 5.000 cases per year within the European Union. The resulting large bone defects lead to scarred, mangled facial appearance, loss of mastication and probably speech, requiring aesthetic and functional surgery as a basis for physical and physiological rehabilitation. Although autologous vascularized bone autografts reflect the current golden standard, the portion of bone available for the procedure is limited and subsequent high-dose anti-cancer chemo-/radiotherapy can lead to local tissue necrosis. Autologous vascularized bone from fibular or iliac-crest autografts is current golden standard in jawbone resection post-treatment, however, the portion of transplantable bone is limited and subsequent high-dose anti-cancer chemo-/radiotherapy often results in tissue necrosis Our research focuses on alternative treatment techniques: tissue reconstruction via novel patient-specifically manufactured maxillofacial implant that stimulates bone tissue growth. The planned neoformation of vascularized bone in such implants within the patient's own body as "bioreactor" is the safest approach in tissue engineering. The works described herein included the design of the metallic substrate of the implant with the use of computed tomography basing on real patients scans and then 3D-printing the substrates from the Ti6Al7Nb powder. The metal core was then evaluated in terms of structural characteristic, cytotoxicity and gene expression through the in vitro tests. Further experiments were focused on fabrication of the biocompatible coating for outer surface of the bone implant that would enhance the healing process and accelerate the tissue growth. Functional polymeric granulate dedicated for osteoconductive, osteoinductive and osteogenesis properties were elaborated. Another approach including the coating for the implant surface with two-phase biocompatible layer including polymeric microspheres and hydrogel carrier, which would provide long-time release of bone and cartilage growth factors around the implant were also done. The polymeric granulate containing ßTCP improved bone cells growth, but it some modification has to be done in order to improve structural pores to ensure for better osteoconductivity. The biocompatible coating including PVP hydrogel and polymeric microspheres is still in the development process.

Full-Digital Workflow for Fabricating a Custom-Made Direct Metal Laser Sintering (DMLS) Mandibular Implant: A Case Report.

Direct Laser Metal Sintering (DLMS) is an additive manufacturing (AM) technique that is capable of manufacturing metal parts according to a three-dimensional (3D) design made using computer-assisted-design (CAD) software, thanks to a powerful laser beam that melts selectively micro-powder layers, one on top of the other, until the desired object is generated. With DMLS, it is now possible to fabricate custom-made titanium implants for oral and maxillofacial applications. We present the case of a 67-year-old woman diagnosed with a squamous cell carcinoma of the mandible. The patient underwent subtotal mandibular resection; conventional reconstruction procedures failed to rehabilitate the function of the mandible. A prosthesis replacing the resected mandible was designed and fabricated using a digital workflow. The extensive bone defect was rehabilitated with a prosthesis replacing the mandibular bone and supporting a morse-taper dental prosthesis. The masticatory function was reestablished.

Odontogenic Myxoma: Systematic review and bias analysis.

BACKGROUND: Odontogenic myxoma (OM) is a rare neoplasm, which originates from odontogenic ectomesenchyme. There is no study in the literature that analyses the best standards for OM diagnosis and how the treatment modalities may influence the recurrence rates. OBJECTIVE: To evaluate the best standards for odontogenic myxoma (OM) diagnosis and treatment, and how these may influence the recurrence rates. STUDY DESIGN: Two independent researchers performed a systematic review in many databases. Fifty-two eligible studies were included for qualitative analysis. Bias analysis was conducted according to Oxford Centre for Evidence-Based Medicine. RESULTS: A total of 1363 OM cases were reported on, and female gender with average age of 27 years is the most common patient profile. Conventional microscopic findings were observed in 93.43% of the reported cases. In 57.49% of the cases, multilocular radiographic appearance was present, followed by unilocular appearance (32.87%). Posterior mandible was the site with the major prevalence, while surgical resection was the most common treatment modality, followed by enucleation. Recurrence rates for both treatment modalities were approximately close (13.04% and 25.0%, respectively). CONCLUSION: The correct diagnosis of OM relies on the association of clinical, radiographic and microscopic findings. About imaging examinations, panoramic radiography and computed tomography are sufficient for the evaluation of OM. Recurrence rates were closely among the two most used surgery treatments. So according to some clinical-radiological aspects, conservative surgery may be preferred than aggressive surgery modalities.

Clinical, histopathologic, subtype, and immunohistochemical analysis of jaw phosphaturic mesenchymal tumors.

Jaw phosphaturic mesenchymal tumors (PMTs) are a rare neoplasm with uncertain histogenesis. This study aimed to clarify the clinical and pathological features of jaw PMTs.We reviewed the clinical records of 39 patients diagnosed with PMTs in the jaws, and investigated clinical and morphologic characteristics, histologic subtypes, and immunophenotypes of all cases.Microscopic analyses revealed 2 major histologic tumor subtypes: "phosphaturic mesenchymal tumors of mixed epithelial and connective tissue" (PMTMECT), and "phosphaturic mesenchymal tumors of mixed connective tissue" (PMTMCT). PMTMECTs and PMTMCTs accounted for 29 and 10 cases of PMTs, respectively. Most PMTMECT diagnoses were made predominantly in males aged <45 years, and the incidence was similar in both the mandible and maxilla. In contrast, patients with PMTMCTs are predominantly females aged ≥45 years, and all tumors were in the mandible. Histologically, PMTMECT had lower cellularity and a more elongated and spindled mesenchymal component with less elaborate intrinsic microvasculature than PMTMCT. Immunohistochemically, the epithelia of all PMTMECTs was immunoreactive for AE1/AE3. Other immunohistochemical staining of PMTMECTs revealed positive expression of vimentin, SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34 in either one or both components. Immunohistochemical staining of PMTMCTs was diffusely positive for vimentin and a varied ratio of positivity for SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34, but negative for AE1/AE3. Most patients were cured by complete resection, except 2 patients who had repeated recurrences, one of which also had multiple metastasis.Jaw PMT can be divided into 2 major histological subtypes. PMTMECTs are more common than are PMTMCTs, and can transform into malignant PMTMCTs during the progression. PMTMECTs were more commonly observed in males and the incidence was similar in both the maxilla and mandible. PMTMCTs were almost always observed in the mandible of females. Compared with PMTMCTs, PMTMECTs have an admixture of epithelial components with less prominent vasculature and lower cellularity. There were no statistically significant differences in the expression of immunohistochemical markers except AE1/AE3 between PMTMECTs and PMTMCTs. However, immunohistochemical markers have great significance for differentiating other mesenchymal tumors.

Defining secure surgical bone margins in head and neck squamous cell carcinomas: The diagnostic impact of intraoperative cytological assessment of bone resection margins compared with preoperative imaging.

BACKGROUND: Imaging provides crucial staging information for treatment planning of head and neck squamous cell carcinomas (HNSCCs). Despite technical progress in imaging techniques, defining the extent of bone involvement preoperatively remains challenging and requires intraoperative information to control for adequate resection. The intraoperative cytological assessment of the bone resection margins (ICAB) provides information whether bone is infiltrated by carcinoma. The aim of this study was to assess the diagnostic value of preoperative imaging compared with ICAB in order to achieve carcinoma-free bone margins. MATERIALS AND METHODS: 108 HNSCC patients underwent preoperative computed tomography (CT), magnetic resonance imaging (MRI) and orthopantomogram (OPG) for staging and surgical planning. Curative resection was planned based on imaging. Intraoperatively, the resection margins were controlled by ICAB. The diagnostic value of preoperative imaging and ICAB was assessed with reference to the histological findings. RESULTS: CT showed a sensitivity of 89.7%, specificity of 63.0%, positive predictive value (PPV) of 85.9%, and negative predictive value (NPV) of 70.8%. MRI revealed a sensitivity of 45.5%, specificity of 66.7%, PPV of 71.4% and NPV of 40.0%. OPG-imaging had a sensitivity of 64.7%, specificity of 76.2%, PPV of 81.5%, NPV 57.1%. In comparison, ICAB provided a sensitivity of 78.6%, specificity of 95.7%, PPV 73.3%, and NPV 96.7%. The accuracy was 82.1%, 52.9%, 69.0%, and 93.5% for CT, MRI, OPG, and ICAB, respectively. CONCLUSION: Preoperative imaging lacks accuracy in defining adequate bone resection margins, compared with ICAB. ICAB supports preoperative imaging and intraoperative frozen sections to improve bone margin control.

Long-Term Outcomes of Parathyroidectomy in Hyperparathyroidism-Jaw Tumor Syndrome: Analysis of Five Families with CDC73 Mutations.

BACKGROUND: Hyperparathyroidism-jaw tumor syndrome (HPT-JT) is a rare disease caused by CDC73 germline mutations, with familial primary hyperparathyroidism (pHPT), ossifying jaw tumors, genito-urinary neoplasms. The present study was aimed at determining the long-term postoperative outcome of parathyroidectomy in HPT-JT. METHODS: A retrospective analysis of a single-center series of 20 patients from five unrelated HPT-JT families undergoing parathyroid surgery was performed. RESULTS: Pathology confirmed a single-gland involvement in 95% of cases at onset. Parathyroid carcinoma occurred in three patients undergoing en-bloc parathyroidectomy and thyroid lobectomy: parathyroid benign lesions in 17 patients undergoing subtotal parathyroidectomy for evident multiglandular involvement (n = 1) or selective parathyroidectomy for single-gland involvement (n = 16), during bilateral (n = 13) or targeted unilateral neck exploration (n = 7). At a median overall follow-up of 16 years (range 2.5-42), patients with parathyroid carcinoma had a persistent/recurrent disease in 66.6%; patients with benign lesions had recurrent pHPT in 23.5% after a prolonged disease-free period; recurrent benign pHPT occurred slightly more often in cases of discordant preoperative localization (60% vs 9%; p = 0.06). CONCLUSION: pHPT in HPT-JT is generally characterized by a benign and single-gland involvement, with a relatively increased risk of malignancy (15%). Parathyroid carcinoma needs extensive surgery because of high risk of permanent/recurrent disease (66.6%). In benign involvement, targeted unilateral exploration with selective parathyroidectomy may be effective in cases of concordant single-gland localization at preoperative localization imaging techniques. Bilateral neck exploration with subtotal parathyroidectomy might be preferred in cases of negative or discordant preoperative localization, because of the increased risk of multiglandular involvement and long-term recurrences (23.5%).

Extra Nodal Rosai-Dorfman Disease Originating in the Nasal and Paranasal Complex and Gnathic Bones: A Systematic Analysis of Seven Cases and Review of Literature.

Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. The classic form of the condition includes a painless cervical lymphaenopathy accompanied by fever, weight loss and an elevated ESR. Extra nodal RDD (ENRDD) is most frequent in the head and neck. Thirty-eight cases of ENRDD have been described. Seven cases of ENRDD were identified in our pathology biopsy services. The demographic and clinical information was tabulated logically on the basis of age, gender, location and presence or absence of symptoms, treatment and follow-up. Radiographic and histopathological features were also examined. The findings in these cases were correlated with those available from the previously reported cases. Six cases affected women and one case was diagnosed in a male. The age ranged from 22-55 years. Three cases presented as a nasal mass. One of these lesions extended into the paranasal sinuses. One case was located in the maxilla and extended to involve the maxillary sinus. Three cases were diagnosed in the mandible. The maxillary and one mandibular lesion (Case 2) resulted in significant painful irregular bone destruction with a non-healing socket and tooth mobility respectively. One mandibular lesion was asymptomatic (Case 6). The third case affecting the mandible presented as a rapidly expansile mass following a tooth extraction (Case 7). Nasal masses presented with symptoms of obstruction. Nasal masses were excised with no recurrence from up to 2-3 years of follow-up. The mandibular lesions were curetted aggressively. The oral mass in Case 7 was excised synchronously. No recurrence up to 2 years was recorded in Case 2. Follow-up information is not available for Cases 6 and 7. The maxillary lesion was not intervened surgically. The patient has persistent but stable disease for a follow-up period of 2 years. ENRDD is rarely considered in the differential diagnosis in the absence of lymph node involvement. Lesions of ENRDD resemble many other histiocytic and histiocyte-rich lesions of the head and neck. This makes the diagnosis of ENRDD challenging with the potential for under diagnosis or misdiagnosis and delay in treatment.

Adjunctive Strategies for Benign Maxillofacial Pathology.

Benign cysts and neoplasms of the maxillofacial region can vary in behavior, with some growing rapidly and resulting in destruction of surrounding structures. Despite their benign histology, many require often-morbid treatment to prevent recurrence of these lesions. Several less invasive and adjunctive medical treatments have been developed to lessen the morbidity of surgical treatment. As the molecular and genomic pathogenesis of these lesions is better understood, more directed treatments may lessen the burden for patients.

Reconstrucción microquirúrgica de cabeza y cuello en paciente pediátrico / Micro-surgical reconstruction of the head and neck in a pediatric patient

OBJETIVO: Realizar un análisis retrospectivo de pacientes pediátricos sometidos a reconstrucciones con injertos libres microvasculares del territorio maxilofacial en el Hospital Luis Calvo Mackenna entre los años 2014 y 2018. MATERIALES Y MÉTODO: Se realizó un análisis de los casos reconstruidos entre los años indicados. RESULTADOS: Un total de 11 pacientes fueron incluidos en la muestra, 7 hombres y 4 mujeres. El rango de edad de los pacientes fue entre 3 a 17 años (media 10,1). Siete colgajos de fíbula, 3 dorsales y 1 anterolateral de muslo fueron utilizados para reconstrucción. La tasa de éxito de los colgajos fue de un 100%. Discusión: Se realizó una discusión de los colgajos y sus principales indicaciones en pediatría y se compararon los resultados de la revisión con los casos reportados. CONCLUSIÓN: El estudio preoperatorio, una adecuada planificación, la rehabilitación de la oclusión dentaria, la reconstrucción simétrica y mantener el contorno facial deben ser objetivos de la reconstrucción.

AIM: Carry out a retrospective analysis of pediatric patients undergoing reconstructions with free micro-vascular grafts of the maxillofacial territory at the Luis Calvo Mackenna Hospital during the years 2014 and 2018. MATERIALS AND METHOD: Retrospective analysis of the cases reconstructed during the years 2014 and 2018 was made. RESULTS: A total of 11 patients were included in the sample, 7 men and 4 women. The age range of the patients was between 3 to 17 years (mean 10.1 years) . Seven Fibula flaps, 3 dorsal and 1 anterolateral thigh flaps were used for reconstruction. The success rate of the flaps was 100%. DISCUSSION: A discussion of the flaps and their main indications in pediatrics was made and the results of the review were compared with the cases reported. CONCLUSION: The pre-operative study, adequate planning, the rehabilitation of the dental occlusion, the symmetry reconstruction and maintaining the facial contour must be objective of the reconstruction.

Persistent response to vemurafenib in metastatic ameloblastoma with BRAF mutation: a case report.

BACKGROUND: Ameloblastomas are uncommon locally aggressive tumors of odontogenic epithelium that rarely metastasize. Currently, there is no standard of care for the metastatic forms. Several studies have shown that ameloblastomas frequently have a BRAF mutation. CASE PRESENTATION: We report a case of a 33-year-old Caucasian woman with ameloblastoma diagnosed 30 years ago who developed lung metastasis 19 years ago. Systemic oral treatment with vemurafenib, a BRAF inhibitor, was initiated 28 months ago within the AcSé French basket clinical trial of vemurafenib. CONCLUSIONS: The patient has shown a durable clinical, functional, and radiographic partial response with vemurafenib. These observations suggest the possibility of introducing neoadjuvant and/or adjuvant targeted therapy in locally advanced ameloblastoma to improve outcome. BRAF inhibition has proved to be an efficient strategy in patients with a BRAF-mutated ameloblastoma.