Ano5 modulates calcium signaling during bone homeostasis in gnathodiaphyseal dysplasia.

ANO5 encodes transmembrane protein 16E (TMEM16E), an intracellular calcium-activated chloride channel in the endoplasmic reticulum. Mutations in ANO5 are associated with gnathodiaphyseal dysplasia (GDD), a skeletal disorder causing the jaw deformity and long bone fractures. However, the coordinated mechanism by which ANO5 mediates bone homeostasis in GDD remains poorly defined. Here, we show that ablation of Ano5 reduced intracellular calcium transients, leading to defects in osteogenesis and osteoclastogenesis and thus bone dysplasia. We found a causative de novo ANO5 frameshift insertion mutation (p.L370_A371insDYWRLNSTCL) in a GDD family with osteopenia, accompanied by a decrease in TMEM16E expression and impaired RANKL-induced intracellular calcium ([Ca2+]i) oscillations in osteoclasts. Moreover, using Ano5 knockout (KO) mice, we found that they exhibited low bone volume, abnormal calcium deposits, and defective osteoblast and osteoclast differentiation. We also showed that Ano5 deletion in mice significantly diminished [Ca2+]i oscillations in both osteoblasts and osteoclasts, which resulted in reduced WNT/ß-Catenin and RANKL-NFATc1 signaling, respectively. Osteoanabolic treatment of parathyroid hormone was effective in enhancing bone strength in Ano5 KO mice. Consequently, these data demonstrate that Ano5 positively modulates bone homeostasis via calcium signaling in GDD.

Excision margin of T3 basal cell carcinoma in maxillofacial area: A retrospective cohort study of Asians.

OBJECTIVES: Basal cell carcinoma is the most common skin cancer worldwide. Surgical excision is considered as the mainstay of treatment, while the evidence of excision margin in advanced stage is lacking, especially in maxillofacial area. MATERIALS AND METHODS: We conducted a 2-center retrospective cohort study. Disease-free survival rate was estimated for 116 Asian patients with T3 basal cell carcinoma in maxillofacial area who received stand surgical excision with margin of 3-5 mm (Group A), 6-9 mm (Group B), and 10-15 mm (Group C). RESULTS: For the entire cohort, five-year disease-free survival rates of Groups A, B, and C were 82.1%, 93.5%, and 92.4%, respectively. When compared with Group B, Group A was correlated with lower disease-free survival rate (HR 5.48, p = .04), and Group C was not associated with different disease-free survival rate (HR 0.85, p = .62). Perineural invasion (p = .006) and pathologic subtypes of infiltrative basal cell carcinoma (p = .01) were independent prognosticator for disease-free survival rate. CONCLUSIONS: This multicenter cohort study validated that T3 basal cell carcinoma Asian patients of maxillofacial area treated with excision margin of 6-9 mm had a substantial benefit of disease-free survival rate and skin conservation.

Correlation Between Body Mass Index and Anterolateral Thigh Flap Thickness: A Retrospective Study From a Single Center in China.

Background: During repair of oral and maxillofacial soft tissue defects, organ function is largely related to the amount of thickness of the flap. However, there are few studies on the influencing factors of the thickness of the flap. In this retrospective study, we aim to explore the correlation between body mass index (BMI) and anterolateral thigh (ALT) flap thickness by computed tomography (CT) and ultrasound and provide guidance for evaluating the ALT flap thickness before surgery. Methods: We selected three points A, B, and C on ALT flap and two skilled clinicians measured the thickness of these points. Age and gender as covariates and evaluated by the Chi-square analysis. Inter-group differences between the two BMI groups were examined by the student t test. Intra-group differences within each BMI group were tested by ANOVA. Linear regression analysis was performed to examine the relationship between BMI and ALT flap thickness. Results: One hundred sixty patients measured by CT were included in this study, and the ALT flap thickness measured by CT were 8.96 mm and 11.00 mm (P < 0.0001, t test) at point B in groups with BMI<24.0 and BMI≥24.0, respectively. The thicknesses at points A, B, and C were significantly correlated with the BMI (P < 0.001, correlation analysis, r = 0.462, 0.372, and 0.349 at the points A, B, and C, retrospectively, Pearson test). Conclusion: There was a significant correlation between the ALT flap thickness and BMI. A higher BMI was correlated with a thicker ALT flap.

lncRNA APOC1P1-3 promoting anoikis-resistance of breast cancer cells.

BACKGROUND: Anoikis resistance plays a critical role in the tumor metastasis by allowing survival of cancer cells in the systemic circulation. We previously showed that long non-coding RNAs APOC1P1-3 (lncRNA APOC1P1-3) inhibit apoptosis of breast cancer cells. In this study, we explored its role in anoikis resistance. METHODS: We induced anoikis resistance in two breast cancer cell lines (MCF-7 and MDA-MB-231) under anchorage-independent culture conditions and studied lncRNA APOC1P1-3 effects on apoptosis. Using Dual-Luciferase activity assay, we determined whether it specifically binds to miRNA-188-3P. We further explored its role in lung metastasis by injecting MDA-MB-231 and MDA-MB-231-APOC1P1-3-knock-down cells in female BALB/c nude mice. RESULTS: We found that lncRNA APOC1P1-3 suppressed early apoptosis of these cells (demonstrated by gain or loss of their function, respectively) and promoted anoikis resistance via reducing activated- Caspase 3, 8, 9 and PARP. Moreover, it specifically binds to the target miRNA-188-3p acting as a "sponge" to block the inhibition of Bcl-2 (an anti-apoptosis protein). CONCLUSIONS: Our study supports a theory that lncRNA APOC1P1-3 can promote development of breast cancer metastasis via anoikis resistance by specifically binding to miRNA-188-3p to block the inhibition of Bcl-2.

Standardize routine angiography assessment of leg vasculatures before fibular flap harvest: lessons of congenital and acquired vascular anomalies undetected by color Doppler and physical examinations.

BACKGROUND: Fibular flaps have been widely used for mandibular and maxillary reconstructions. On occasion, anatomical variants of fibular arteries (FA) will be encountered. PURPOSE: Although anatomical variants of FA during fibular harvest have been reported, controversy exists regarding whether simple color Doppler ultrasonography (CDU) and physical examinations (PE) are sufficient for early preoperative detection. MATERIAL AND METHODS: A 10-year retrospective analysis in our department was performed to find the patients with various FA anomalies confirmed by computed tomography angiography (CTA) or intraoperative findings. RESULTS: A total number of 19 FA anomalies were found either pre- or intraoperatively in 16 patients, in whom three cases were with bilateral FA variants. Type IIIC variants, also called arteria peronea magna (great peroneal artery), were confirmed in two legs, while the majority (13 legs) had type IIIA hypoplastic/aplastic posterior tibialis arteries (PTA). Four legs had new type IIID (low FA and PTA bifurcations). Preoperative CDU and PE only suspected anomalies in two legs. Six cases proceeded with using the affected fibulas, within whom vascular grafts were used in half of them for lengthening the FA pedicle. Local ischemia, partial soleus muscle necrosis, and claudication were reported in one. CONCLUSIONS: Routine CTA before every fibular harvest, rather than simple PE and CDU, should be added for screening contraindications and ensuring safety for fibular flap harvest.

Subchronic exposure to concentrated ambient PM2.5 perturbs gut and lung microbiota as well as metabolic profiles in mice.

Exposure to ambient fine particular matter (PM2.5) are linked to an increased risk of metabolic disorders, leading to enhanced rate of many diseases, such as inflammatory bowel disease (IBD), cardiovascular diseases, and pulmonary diseases; nevertheless, the underlying mechanisms remain poorly understood. In this study, BALB/c mice were exposed to filtered air (FA) or concentrated ambient PM2.5 (CPM) for 2 months using a versatile aerosol concentration enrichment system(VACES). We found subchronic CPM exposure caused significant lung and intestinal damage, as well as systemic inflammatory reactions. In addition, serum and BALFs (bronchoalveolar lavage fluids) metabolites involved in many metabolic pathways in the CPM exposed mice were markedly disrupted upon PM2.5 exposure. Five metabolites (glutamate, glutamine, formate, pyruvate and lactate) with excellent discriminatory power (AUC = 1, p < 0.001) were identified to predict PM2.5 exposure related toxicities. Furthermore, subchronic exposure to CPM not only significantly decreased the richness and composition of the gut microbiota, but also the lung microbiota. Strong associations were found between several gut and lung bacterial flora changes and systemic metabolic abnormalities. Our study showed exposure to ambient PM2.5 not only caused dysbiosis in the gut and lung, but also significant systemic and local metabolic alterations. Alterations in gut and lung microbiota were strongly correlated with metabolic abnormalities. Our study suggests potential roles of gut and lung microbiota in PM2.5 caused metabolic disorders.

Title: Developmental validation of Y-SNP pedigree tagging system: A panel via quick ARMS PCR.

The Y chromosomal short tandem repeats (Y-STRs) have been used widely to establish paternal relatedness and examine sub-structures in different geographical regions. However, the applications of Y-STRs showed their limitations when it comes to resolving the complicated relationships within close relatives or among unrelated individuals from different geographic areas. Here, we overcome these limitations by introducing a new strategy for Y-SNP multiplex typing using rapid ARMS (amplification-refractory mutation system) PCR. Newly developed Y-SNP Pedigree Tagging System is able to profile 24 Y-SNPs in a single reaction while the whole process takes 4-5 hours. The panel precisely defines the 11 haplogroups (E-M96, D-JST021355, N-M231, C-M130, O-P186, I-M170, IJ-M429, K-M9, QR-M45, G-M201, and IJK-M522) and 13 sub-haplogroups (D1a1a1-N1, D1a2a-P47, C2-M217, N1a1-M46, O1a-M119, O1b-M268, O1b2-M176, O2-M122, O2a1-KL1, O2a2-P201, O2a2b-P164, O2a2a1a2-M7 and O2a2b1a1-M117). This system could contribute to providing the haplogroup affiliation of unknown pedigree and resolving the sub-structures of East Asian populations. In this study, the multiplex system was validated for: ability to detect degraded DNA, sensitivity, species specificity, reproducibility/repeatability, stability, performance in different scenarios, mixture studies, PCR amplification conditions, and population surveys. The Y-SNP information showed a consistent pattern within 40 father-son or brother-brother pairs. The results of this multiplex system showed the different distribution patterns of male donors from two Chinese Han populations. In this study, we try to discriminate the suspect's pedigree on the level of Y-SNP haplogroups. These results show that Y-SNP Pedigree Tagging System is a robust and reliable amplification kit which can be used for male haplogroup determination.

Comparison of wall removal type versus wall retaining type of decompression for treating large mandibular odontogenic cysts.

OBJECTIVE: To evaluate the differences between two types of decompression for treating large mandibular odontogenic cysts. MATERIALS AND METHODS: This retrospective, cohort study included patients who were diagnosed for large mandibular odontogenic cysts from January 2015 to April 2018 and underwent two different types of decompression based on removal or retention of the cyst wall. The primary outcome was the percentage of the residual cyst area within 1 year after surgery. We used the propensity score matching (PSM) to balance the covariates of the two groups, and the primary outcome was analyzed by the non-inferiority test. RESULTS: A total of 93 cases were included in our study. After 1 year, the wall removal group was non-inferior to the wall retaining group in terms of cyst area, cyst volume, and the difference between the HU values of the original cyst region and the normal mandible. In subgroup analysis, we found there were no differences between two groups in the percentage of patients in whom the cystic area was reduced by 90% after 1 year. CONCLUSION: Our findings suggest that the wall removal group showed better results than the cyst wall retaining group in large mandibular odontogenic cysts.

Jumonji domain-containing protein 6 functions as a marker of head and neck squamous cell carcinoma at advanced stage with no effect on prognosis.

A number of studies have reported the upregulation and functional roles of Jumonji domain-containing protein 6 (JMJD6) in various types of cancer. However, little is known regarding the clinical significance of JMJD6 in head and neck squamous cell carcinoma (HNSCC), particularly in terms of large-cohort data. In the present study, bioinformatics analysis was performed, using the University of California Santa Cruz Xena Browser and the Gene Expression Profiling Interactive Analysis 2 server, based on the Cancer Genome Atlas HNSCC cohort. In addition, a validation cohort was constructed based on 98 HNSCC cases. JMJD6 overexpression and knockdown, colony-formation, Transwell and cell viability assays were performed. JMJD6 was highly expressed in HNSCC samples and was associated with advanced pathological stage. However, no significant association was observed between JMJD6 expression levels and overall survival or disease-free survival times of patients with HNSCC. Subsequent in vitro assays indicated that overexpression of JMJD6 promoted malignant progression of HNSCC, by regulating epithelial-mesenchymal transition. Nevertheless, JMJD6 overexpression had no significant effects on the viability of HNSCC cells treated with 5-fluorouracil or cisplatin. Thus, it can be concluded that JMJD6 may function as a marker of HNSCC at advanced stage, however with no effect on drug resistance or prognosis. For patients with advanced HNSCC and high JMJD6 expression, rational chemotherapy may be more beneficial than radical surgery, considering their quality of life.

The necessity of IIb dissection in T1-T2N0M0 oral squamous cell carcinoma: protocol for a randomized controlled trial.

BACKGROUND: There is a growing debate on the relationship between health-related quality of life (HRQoL) and patient survival which has been going on for the last few decades. The greatest wish of clinicians is to extend the latter while improving the former. Following neck dissection of early-stage oral carcinoma, "shoulder syndrome" appears due to traction of the accessory nerve during removal of level IIb, which greatly affects patient quality of life. Since occult metastasis in level IIb of early-stage oral carcinoma is extremely low, some surgeons suggest that level IIb can be exempt from dissection to improve the HRQoL. However, other surgeons take the opposite view, and thus there is no consensus on the necessity of IIb dissection in T1-2N0M0 oral squamous cell carcinoma (OSCC). METHODS: We designed a parallel-group, randomized, non-inferiority trial that is supported by Shanghai Ninth People's Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, China. We will enroll 522 patients with early oral carcinoma who match the inclusion criteria, and compare differences in 3-year overall survival, progression-free survival (PFS) and HRQoL under different interventions (retention or dissection of level IIb). The primary endpoints will be tested by means of two-sided log-rank tests. Analysis of overall and progression-free survival will be performed in subgroups that were defined according to stratification factors with the use of univariate Cox analysis. In addition, we will use post-hoc subgroup analyses on the basis of histological factors that were known to have effects on survival, such as death of invasion of the primary tumor. To evaluate HRQoL, we will choose the Constant-Murley scale to measure shoulder function. DISCUSSION: Currently, there are no randomized controlled trials with large sample sizes on the necessity of IIB dissection in T1-T2N0M0 OSCC. We designed this noninferiority RCT that combines survival rate and HRQoL to assess the feasibility of IIb neck dissection. The result of this trial may guide clinical practice and change the criteria of how early-stage oral cancer is managed. The balance between survival and HRQoL in this trial is based on early-stage breast cancer treatment and may provide new ideas for other malignancies. TRIAL REGISTRATION: Chinese Clinical Trial Registry, ChiCTR1800019128 . Registered on 26 October 2018.

The rare entity of cystadenocarcinoma (CAC) in parotid gland: A single-center experience.

PURPOSE: Cystadenocarcinoma (CAC) is an extremely rare disease in parotid gland. This study aimed to identify the clinical characteristics of CAC, and the therapeutic options for its treatment. An attempt was also made to identify postoperative recurrence-related risk factors. MATERIAL AND METHODS: A retrospective study was conducted of CAC patients treated between 2008 and 2018. Predictive factors for postoperative recurrence (5-year RFS rate) were preliminarily filtered by Kaplan-Meier analysis and then further confirmed by a Cox regression model. Postoperative recurrence was defined as the primary outcome variable and was measured using both univariate and multivariate analysis. RESULTS: A total of 27 patients were analyzed, and the total incidence of postoperative recurrence was 33.3% (9/27). In the Cox regression analysis, patients who received a superficial parotidectomy were 0.046 times more likely to develop tumor relapse than those who only underwent enucleation (p = 0.032; 95% CI: 0.003-0.070). The chances of tumor recurrence in patients with the cribriform subtype were 9.701 times that for cases with a papillary pattern (p = 0.016; 95% CI: 1.517-62.030). The risk of postoperative recurrence increased abruptly, with an OR of 6.373 (p = 0.042; 95% CI: 1.070-37.965), when LN metastasis was found in patients. CONCLUSION: Preoperative diagnosis of CAC in parotid gland is extremely important for allowing surgeons to apply appropriate therapeutic strategies (enucleation or superficial parotidectomy). Patients with LN metastasis, cribriform pattern, and Ki-67 positivity should be treated further to avoid tumor relapse.

Preadsorption of Serum Proteins Regulates Bacterial Infections and Subsequent Macrophage Phagocytosis on Biomaterial Surfaces.

Bacterial infection has been one of the main obstacles for extensive biomedical applications of biomaterial films. Understanding the interactions among macromolecules, cells, and bacteria in the microenvironment located on the film surface at the molecular level is essential for developing antibacterial films. Here we report the distinct influence of several key serum proteins adsorbed on diamond-like carbon (DLC) and traditional Ti films on initial bacterial adhesion, biofilm formation, and corresponding immune responses. Type I collagen, Fn, and IgG were selected as the typical serum proteins. Gram-positive bacterium Staphylococcus epidermidis and Gram-negative bacterium Escherichia coli were used as the model bacteria. Macrophage phagocytosis tests were carried out to examine the impact of adsorbed proteins on the ability of macrophages to clear the adhered pathogens. Results show that it was the specific molecular recognition between adsorbed proteins and bacteria, not the surface physiochemical properties such as surface wettability, surface roughness, and surfaces charge, that decisively affected bacterial adhesion and following biofilm formation. Collagen resisted bacterial adhesion on both DLC and Ti films, even though the molecules exhibited distinct conformations on the two surfaces, whereas for Fn and IgG, the specific molecular recognition was closely related to protein conformations. Fn molecules formed globular aggregates on Ti surfaces that greatly enhanced bacterial adhesion but exhibited a fibril conformation on DLC surfaces that inhibited bacterial adhesion. IgG showed an end-on orientation with free F(ab)2 domains on Ti surfaces, facilitating bacterial adhesion and biofilm formation, while the flattened orientation on DLC films showed little effect on bacterial behaviors. Furthermore, preadsorption of Fn and IgG significantly promoted the phagocytosis ability of macrophages against S. epidermidis and affected the corresponding secretion of inflammatory cytokine. These results would give insights into understanding protein-surface interactions for developing appropriate surface modification techniques for biomaterials with desired anti-inflammatory functions.

Chitosan-based liposomal thermogels for the controlled delivery of pingyangmycin: design, optimization and in vitro and in vivo studies.

Pingyangmycin (PYM) has been applied clinically for many years to treat vascular malformations (VM) in China. The major limitation of PYM injections is quick diffusion from the injection site, which increases side effects, especially the possibility of pulmonary injury. In this paper, chitosan/glycerophosphate disodium (CS/GP) thermogels containing liposomes for sustained and localized PYM delivery were prepared and optimized by a three-level three-factorial Box-Behnken experimental design to evaluate the effects of different variables (the PYM concentration, CS amount and GP content), on the selected responses (cumulative percentage PYM released in 1 day, 9 days and the rate constant k). The results revealed that the optimized PYM liposomal thermogels had a controlled PYM release for 14 days in vitro, which confirmed the validity of optimization. In vitro morphological observation, cell cycle and apoptosis analysis showed an effective anti-proliferation action of PYM liposomal thermogels on human vascular endothelial cells (EA.hy926). In vivo pharmacokinetics research in rabbits displayed that compared with PYM liposomes and PYM thermogels, PYM liposomal thermogels had a better controlled delivery of PYM. Histological examination of rabbit ear veins showed that after local application with PYM lipsomal thermogels for 21 days, obvious vein thrombosis and inflammatory reaction could be observed. The above results indicated that PYM-loaded lipsomal CS/GP thermogels might have a good prospect for the treatment of VM.

Proper Choice of Donor Site Veins for Patients Undergoing Free Radial Forearm Flap Reconstruction for the Defects of Head and Neck.

PURPOSE: The purpose of this study was to analyze the reliability of the deep venous drainage system of the free radial forearm flap. MATERIALS AND METHODS: The authors implemented a retrospective cohort study and enrolled patients admitted for the evaluation and management of head and neck tumors with radial forearm flap reconstruction. The primary predictor variables were the donor site veins, including the deep, dual, and superficial venous drainage systems. The primary outcome measurement was the incidence of venous compromise. Potential confounders included patient demographics, history of chemotherapy with or without radiotherapy, defect sites, and the recipient artery and vein. The χ2 test and logistic regression analysis were used for statistical analysis. RESULTS: The final study population consisted of 520 patients (mean age, 54 years) who underwent head and neck soft tissue reconstruction using the free radial forearm flap. Patients with the deep system (odds ratio [OR] = 0.251; 95% confidence interval [CI], 0.108-0.581) or dual system (OR = 0.197; 95% CI, 0.064-0.605) had a markedly lower risk of venous compromise than those with the superficial system. The overall incidence of inadequate outflow was 2.5% (5 of 204) when the deep system was used alone and 8.9% (19 of 212) when the superficial system was used alone (P = .004). CONCLUSIONS: Patients with the deep venous drainage system had a statistically lower risk of venous compromise. This study recommends using the deep system as a drainage vein for the free radial forearm flap.

Fisetin suppresses malignant proliferation in human oral squamous cell carcinoma through inhibition of Met/Src signaling pathways.

Fisetin (3,7,3',4'-tetrahydroxyflavone) is a dietary flavonoid and has been indicated as a novel anti-cancer agent in several types of cancer cells. However, the mechanisms underlying the effect of fisetin in human oral squamous cell carcinoma (OSCC) remain unclear. Here, we report that fisetin significantly inhibits tumor cell proliferation and induces apoptosis in OSCC (UM-SCC-23 and Tca-8113) cancer cell lines. Further analysis demonstrates that fisetin also inhibits Met/Src signaling pathways using the PathScan® receptor tyrosine kinases (RTK) Signaling Antibody Array Kit. Fisetin resulted in decreased basal expression of Met and Src protein in UM-SCC-23 cancer cell lines, which validated by western blot. A student's t-test (two-tailed) was used to compare differences between groups. Furthermore, fisetin significantly inhibited the expression of a disintegrin and metalloproteinase 9 (ADAM9) protein in OSCC cells. Taken together, these results provide novel insights into the mechanism of fisetin and suggest potential therapeutic strategies for human OSCC by blocking the Met/Src signaling pathways.

Management strategy in patient with familial gigantiform cementoma: A case report and analysis of the literature.

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.

Familial Gigantiform Cementoma: Case Report of an Unusual Clinical Manifestation and Possible Mechanism Related To "Calcium Steal Disorder".

Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. We present an adolescent case with recurrent familial gigantiform cementoma who received surgical intervention in our hospital. Apart from typical multiquadrant and expansile abnormalies involving both jaws, he also suffered from several times of fractures in lower extremity. Furthermore, radiographic examinations of calvaria, pelvis, femoris, tibia, and fibula all revealed radiolucent areas signifying diffuse osteopenic bone losses. Some of his consanguineous relatives bore the same burden of fractures during pubertal period.Considering these polyostotic conditions, a correlation of congenital bone metabolism disorder in cases with familial gigantiform cementoma, named "calcium steal disorder," was thus proposed. Familial gigantiform cementoma is closely associated with "calcium steal disorder." Whole-body dual-energy absorptiometry should be considered as a routine examination for fracture-related risk prediction.

A modified technique for reconstruction of a total maxillary defect.

Total maxillary defects with orbital retention (Brown class 2b) are a challenge to reconstructive surgeons because of the variety of anatomical structures involved. Traditional techniques to reconstruct the orbital floor, zygoma, and maxilla using only a vascularised fibular flap are complicated, as the osteotomy and orientation of bone are difficult. Reconstruction of the orbital floor with titanium mesh may also cause palpable discomfort and increase the risk of secondary infection. We describe a modified technique using a vascularised fibular flap, together with a coronoid temporalis pedicle flap, which we used in two patients in whom we achieved satisfactory aesthetic and functional results. Our technique provides adequate tissue for infraorbital skin defects, provides pedicles of sufficient length, and requires only one fibular osteotomy. To our knowledge this is the first report of this technique.

Alveolar soft part sarcoma of the oral and maxillofacial region: clinical analysis in a series of 18 patients.

OBJECTIVES: To summarize the clinical features, diagnosis, treatment strategies, and prognosis of alveolar soft part sarcoma (ASPS) of the oral and maxillofacial region. STUDY DESIGN: We performed a retrospective study in a consecutive series of 18 patients with ASPS of the oral and maxillofacial region between 1995 and 2013. Demographic characteristics, tumor sizes, sites, tumor metastasis, diagnosis, treatments, and overall follow-ups were documented. RESULTS: The 18 patients were diagnosed pathologically with primary tumor developed on the tongue (10), the cheek (5), the pharynx (1), and the gingiva (2) with an average tumor size of 4 cm. At the latest follow-up, 1 patient with lung metastases survived for 23 months; 1 died 3 months after the confirmation of local recurrence and multiple pulmonary metastases; the rest of the patients were disease free and remained in good health. CONCLUSIONS: ASPS of the oral and maxillofacial region appears to have special clinical characteristics.