Unusual Presentation of Juvenile Rhabdomyosarcoma in the Temporomandibular Area: A Case Report

Rhabdomyosarcoma frequently affects the craniofacial region with a rapid growing pattern that usually results in swelling of the interested area. The present paper describes a peculiar occurrence of rhabdomyosarcoma in a 12-year-old boy and the importance of a careful evaluation of clinical history to choose the correct diagnostic strategy. The patient was evaluated in the orthodontics section of the Dental School of the University of Bologna by an orofacial pain specialist, with a compliance of excruciating pain around the right ear that occurred 4 months earlier after a sports trauma. The patient had been previously evaluated by a pediatrician and an ENT specialist who requested a computerized tomography that did not show pathological conditions in the head/neck district. The drug therapy for pain control was not effective. The clinical examination showed a severe limitation of mouth opening, periauricular paresthesia and mandibular hyperalgesia on the right side. The patient reported intermittent, very intense stabs of pain occurring every 5 min, with a continuous dull pain in the temporal area. A nuclear magnetic resonance was requested and showed the presence of a solid expansive lesion in the right pterygoid area that eroded the cranial base and the medial portion of the mandibular condyle. An incisional biopsy led to the diagnosis of embryonal rhabdomyosarcoma. This case report emphasizes the importance of not underestimating the presence of pain in young subjects, suggesting a proper approach to apparently simple clinical cases

Early peripheral clearance of leukemia-associated immunophenotypes in AML: centralized analysis of a randomized trial

Although genetics is a relevant risk factor in acute myeloid leukemia (AML), it can be minimally informative and/or not readily available for the early identification of patients at risk for treatment failure. In a randomized trial comparing standard vs high-dose induction (ClinicalTrials.gov 64NCT00495287), we studied early peripheral blast cell clearance (PBC) as a rapid predictive assay of chemotherapy response to determine whether it correlates with the achievement of complete remission (CR), as well as postremission outcome, according to induction intensity. Individual leukemia-associated immunophenotypes (LAIPs) identified pretherapy by flow cytometry were validated and quantified centrally after 3 days of treatment, expressing PBC on a logarithmic scale as the ratio of absolute LAIP1 cells on day 1 and day 4. Of 178 patients, 151 (84.8%) were evaluable. Patients in CR exhibited significantly higher median PBC (2.3 log) compared with chemoresistant patients (1.0 log; P<.0001). PBC<1.0 predicted the worst outcome (CR, 28%). With 1.5 log established as the most accurate cutoff predicting CR, 87.5% of patients with PBC .1.5 (PBChigh, n = 96) and 43.6% of patients with PBC 641.5 (PBClow, n = 55) achieved CR after single-course induction (P<.0001). CR and PBChigh rates were increased in patients randomized to the high-dose induction arm (P 5 .04) and correlated strongly with genetic/cytogenetic risk. In multivariate analysis, PBC retained significant predictive power for CR, relapse risk, and survival. Thus, PBC analysis can provide a very early prediction of outcome, correlates with treatment intensity and disease subset, and may support studies of customized AML therapy

The Effects of Mandibular Advancement Device on Pressure Pain Threshold of Masticatory Muscles: A Prospective Controlled Cohort Study

AIMS: To determine if pressure pain thresholds (PPTs) of masticatory and neck muscles change after the application of a mandibular advancement device (MAD) in patients with obstructive sleep apnea (OSA). METHODS: A prospective study was conducted in a sample of 27 OSA patients (24 males and 3 females; mean age ± standard deviation [SD]: 54.8 ± 11.8, mean apnea-hypopnea index ± SD: 23.5 ± 13.3) and 27 age- and sex-matched healthy controls. Exclusion criteria were signs and symptoms of temporomandibular disorders (TMD), metabolic diseases, and use of antidepressants, analgesics, or anti-inflammatory drugs. A calibrated examiner evaluated PPTs of seven head and neck muscles bilaterally by using a Fischer algometer. In the OSA group, PPTs were recorded immediately before the MAD application (T₀), after 15 days (T₁), and after 6 months (T₂) of therapy; in the control group, PPTs were recorded at the same time intervals. PPT differences at baseline and over time within each group and between OSA and control groups were analyzed by Friedman and Mann-Whitney tests. RESULTS: There were no PPT differences between groups at baseline. In the OSA group, PPTs of temporalis and masseter muscles decreased significantly at T₁ compared with T₀ (P < .05), but no differences were found at T₂. No significant PPT differences were found in the neck muscles or over time in the control group. CONCLUSION: MAD application induces a decrease of PPTs of masticatory muscles at the beginning of the therapy, but a physiologic adaptation occurs by 6 months

Effects of experimental occlusal interference on body posture: an optoelectronic stereophotogrammetric analysis

In recent years, there has been increasing interest in the relationship between dental occlusion and body posture both among people and in scientific literature. The aim of the present longitudinal study is to investigate the effects of an experimental occlusal interference on body posture by means of a force platform and an optoelectronic stereophotogrammetric analysis. An occlusal interference of a 0- to 2-mm-thick glass composite was prepared to disturb the intercuspal position while not creating interference during lateral or protrusive mandibular excursions. Frontal and sagittal kinematic parameters, dynamic gait measurements and superficial electromyographic (SEMG) activity of head and neck muscles were performed on 12 healthy subjects. Measurements were taken 10 days before the application of the occlusal interference, and then immediately before the application, the day after it, and at a distance of 7 and 14 days under four different exteroceptive conditions. The outcomes of this study show that an occlusal interference does not modify significantly over time static and dynamic parameters of body posture under different exteroceptive conditions. It has a minimal influence only on the frontal kinematic parameters related to mandibular position, and it induces a transient increase of the activity of masticatory muscles. In this study, the experimental occlusal interference did not significantly influence the body posture during a 14-day follow-up period

Rosiglitazone promotes the differentiation of Langerhans cells and inhibits that of other dendritic cell types from CD133 positive hematopoietic precursors

Dendritic cells and their precursors express PPAR-gamma, whose stimulation has inhibitory effects on the maturation and function of dendritic cells in vivo. Dendritic cells can differentiate in vitro from CD133+ progenitors; the influence of PPAR-gamma stimulation on this process is unknown. We have addressed the effect of PPAR-gamma agonist rosiglitazone, at a concentration as used in clinics, on the differentiation of dendritic cells from human CD133+ progenitors. Cells were harvested from cord blood by density gradient and immunomagnetic separation, and cultured for 18 days with fetal calf serum, cytokines and 1 µmol/L rosiglitazone. Analyses included flow cytometry, electron microscopy and mixed lymphocyte reaction. As expected, control cells generated without rosiglitazone were dendritic, expressed MHC-II, CD80, CD83 and CD86 and stimulated mixed reaction potently. A minority of cells expressed the Langerhans cell marker CD207/langerin, but none contained Birbeck granules. With rosiglitazone much fewer cells were generated; they were all dendritic, expressed differentiation and maturation-related antigens in higher percentage and were better stimulators of lymphocytes than those generated without the drug. The vast majority of cells expressed CD207/langerin and many contained Birbeck granules, i.e. were full-fledged Langerhans cells. We conclude that stimulation of PPAR-gamma, while negatively affecting the number of generated cells, promotes the maturation of human cord blood CD133 positive precursors into efficient, immunostimulating dendritic cells with a Langerhans cell phenotype