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Objectives To evaluate the efficacy and toxicity profiles of everolimus in Japanese patients with renal angiomyolipoma associated with tuberous sclerosis complex. Methods Patients with a 4-cm or larger angiomyolipoma meeting the diagnostic criteria of tuberous sclerosis complex were selected as participants of our investigation. In each case, we assessed tuberous sclerosis complex-associated symptoms, the treatment effect and adverse events. The treatment effect was evaluated by measuring the tumor volume reduction rate using abdominal computed tomography or magneitc resonance imaging. Adverse events were investigated using CTCAE v4.0- JCOG. The dose of everolimus was set at 10 mg once a day for adults. For childhood angiomyolipoma, everolimus administration was initiated at a dose of 5 mg once a day. Blood everolimus level was measured, and the dose was adjusted to maintain this within a range of 5-15 ng/ mL. Results The angiomyolipoma volume decreased in 46 of 47 cases, and the mean reduction rate for all cases was 60% in 12 months. The angiomyolipoma volume markedly decreased in the first 3 months, and the size leveled off after 6 months. The main adverse events related to everolimus treatment were stomatitis (91%) and irregular menstruation (65%). Grade 3 or severer adverse events were noted in three cases (6%). All patients developed some adverse events in the first 6 months. The incidence markedly decreased to 40-50% after 13 months. Conclusion The tumor volume-reducing effect of everolimus in a Japanese population was equivalent to or higher than that in Western populations. A wide variety of everolimus treatment-related adverse events can be observed, but most cases are very mild. Special attention should be given to stomatitis, irregular menstruation and interstitial lung disease as adverse events. [ABSTRACT FROM AUTHOR]

ABSTRACT Cam deformity could lead to suboptimal articulation by causing secondary femoroacetabular impingement after periacetabular osteotomy; however, the inherent femoral head-neck morphology in dysplastic hips and the effect of an additional osteoarthritic deformity have not been well described. We compared femoral head-neck morphology using three-dimensional imaging of normal and dysplastic hips in pre/early (Tönnis grade 0 and 1) and advanced stage osteoarthritis (Tönnis grade 2). Using computed tomography, we measured the circumferential α-angle and head-neck offset ratio in 68 dysplastic hips and 24 normal hips. Locations of the head-neck junction were represented by the clock position. In the pre/early group, the α-angle was significantly larger at the anterosuperior and inferior aspects (1, 2, and 5-7 o'clock) and head-neck offset ratio was smaller at the anterosuperior aspect (2 o'clock) than in the control group. The α-angle was significantly larger at the anterior aspects (1-4 o'clock) in the advanced group than in the pre/early group. The maximum α-angle was most commonly found at 2 o'clock (60%, 41/68 hips) in dysplastic hips. The prevalence of cam deformity (maximum α-angle >55°) was 4.2% (1/24 hips) in the control group, 22% (11/50 hips) in the pre/early group, and 50% (9/18 hips) in the advanced group. Cam deformity, inherent in the pre/early group, was found with relatively high frequency. The higher prevalence in the advanced group reflected degeneration-modified changes. When performing periacetabular osteotomy, preoperative radiographic assessments should include the femoral head-neck junction to prevent secondary femoroacetabular impingement, especially in patients with advanced stage osteoarthritis. © 2015 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 34:1613-1619, 2016. [ABSTRACT FROM AUTHOR]

Background: We had previously identified the following risk factors for insufficient control of early T-stage head and neck cancer by transoral surgery (TOS): (1) tumor thickness > 7 mm on enhanced computed tomography (CT), and (2) poor differentiation in pathological examination. We subsequently used a different patient cohort to validate the usefulness of these factors in determining the need for adaptation of TOS. Study setting: A prospective observational study Methods: Patients who received TOS as a definitive treatment between April 1, 2016 and September 30, 2020 were included. Primary control rates (by single TOS and TOS alone) in relation to the above-mentioned risk factors were calculated. Overall (O), recurrence-free (RF), and disease-free (DF) survival (S) outcomes were evaluated. A combination analysis based on the number of risk factors was also performed. Results: Patients with tumor thickness > 7 mm had a 2.88-fold [95% confidence interval (CI) 1.01–8.51] higher risk of incomplete primary resection by single TOS, while patients who showed poor differentiation on pathological assessments had a 13.14-fold (95% CI 3.66–47.14) higher risk of insufficient primary control by TOS alone. The 3 year OS, RFS, and DFS rates were 99%, 83%, and 63%, respectively. Patients with both risk factors had a 93.00-fold (95% CI 4.99–1732.00) higher risk of incomplete primary control by TOS alone. Conclusions: Among patients with early-stage laryngeal, oropharyngeal, and hypopharyngeal squamous cell carcinoma, primary control by TOS alone may not be achieved in patients with both risk factors, that is, tumor thickness > 7 mm as measured by enhanced CT and poor differentiation on pathological examination. [ABSTRACT FROM AUTHOR]