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Background Tumor-associated fibroblast growth factor 23 (FGF-23)-induced hypophosphatemic rickets is a rare but known pediatric entity first described in 1959. It results from local production of phosphatonins by benign and malignant mesenchymal tumors. Case-Diagnosis/Treatment We report an 8-year-old boy with tumor-associated hypophosphatemic rickets due to paraneoplastic FGF-23 secretion from a benign mesenchymal pelvic-bone tumor. Excessive FGF-23 production was visualized by immunohistochemistry in the resected tumor. Phosphate wasting stopped immediately after tumor resection. We reviewed 26 reports of pediatric patients with tumor-induced hypophosphatemic rickets; paraneoplastic FGF-23 secretion was documented in only three of them. All tumors developed inside bone, were benign in 21/26 cases, and were localized in femur/tibia (13/26), radius/ulna/humerus (7/26), pelvis (4/26), rib (1/26), and craniofacial (1/26) bones. Mean interval between onset of signs and/or symptoms and diagnosis was 34 months. Conclusions In patients with hypophosphatemic rickets acquired beyond infancy, radiologic investigations for bone tumors need to be performed rapidly. In contrast to biochemical screening for increased circulating FGF-23 levels, immunohistochemical confirmation of FGF-23 production in resected tumor tissue can be regarded as being well established. Keywords Children * Hypophosphatemic rickets * Fibroblast growth factor 23 * Tumor-induced rickets, Introduction Tumor-induced hypophosphatemic rickets (TIR) is a rare condition first described in 1959 [1]. In adults, the same pathogenetic mechanism results in tumor-induced osteomalacia (TIO), which has been reported in [...]

Osteosarcoma is the most common primary malignant bone tumour in children and adolescents. More than a third of patients do not respond to standard therapy and urgently require alternative treatment options. Due to a high degree of inter‐ and intra‐tumoural genomic heterogeneity and complexity, recurrent molecular alterations that could serve as prognostic predictors or therapeutic targets are still lacking in osteosarcoma. Copy number (CN) gains involving the IGF1R gene, however, have been suggested as a potential surrogate marker for treating a subset of patients with IGF1R inhibitors. In this study, we screened a large set of osteosarcomas and found specific CN gains of the IGF1R gene in 18 of 253 (7.1%) cases with corresponding IGF1R overexpression. Despite the discouraging results observed in clinical trials in other tumours so far, focusing only on selected patients with osteosarcoma that show evidence of IGF pathway activation might represent a promising new and innovative treatment approach. [ABSTRACT FROM AUTHOR]

Abstract Background Dynamic three-dimensional (3D) deformation of the pelvic bones is a crucial factor in the successful design and longevity of complex orthopaedic oncological implants. The current solutions are often not very promising for the patient; thus it would be interesting to measure the dynamic 3D-deformation of the whole pelvic bone in order to get a more realistic dataset for a better implant design. Therefore we hypothesis if it would be possible to combine a material testing machine with a 3D video motion capturing system, used in clinical gait analysis, to measure the sub millimetre deformation of a whole pelvis specimen. Method A pelvis specimen was placed in a standing position on a material testing machine. Passive reflective markers, traceable by the 3D video motion capturing system, were fixed to the bony surface of the pelvis specimen. While applying a dynamic sinusoidal load the 3D-movement of the markers was recorded by the cameras and afterwards the 3D-deformation of the pelvis specimen was computed. The accuracy of the 3D-movement of the markers was verified with 3D-displacement curve with a step function using a manual driven 3D micro-motion-stage. Results The resulting accuracy of the measurement system depended on the number of cameras tracking a marker. The noise level for a marker seen by two cameras was during the stationary phase of the calibration procedure ± 0.036 mm, and ± 0.022 mm if tracked by 6 cameras. The detectable 3D-movement performed by the 3D-micro-motion-stage was smaller than the noise level of the 3D-video motion capturing system. Therefore the limiting factor of the setup was the noise level, which resulted in a measurement accuracy for the dynamic test setup of ± 0.036 mm. Conclusion This 3D test setup opens new possibilities in dynamic testing of wide range materials, like anatomical specimens, biomaterials, and its combinations. The resulting 3D-deformation dataset can be used for a better estimation of material characteristics of the underlying structures. This is an important factor in a reliable biomechanical modelling and simulation as well as in a successful design of complex implants.

Introduction Simple bone cysts (SBCs) are common in children and adolescents. The risk of refracture and the probability of spontaneous healing in SBCs are mainly dependent on the activity of the cyst and can be quantified with the Cyst-Index. Avoiding pathological fractures is the primary goal. Our study presents a comparison of two different bioresorbable bone graft substitutes (BGSs) in the minimally-invasive treatment of SBC in the active stage by percutaneous cyst aspiration and injection. Methods Between 2006 and 2017, 38 patients (aged two to 37 years; mean age 12.4 (sd 5.6)) were treated with percutaneous cyst aspiration and refilled with bioresorbable BGSs in three hospitals. The cysts of 21 patients (11 humerus, five femur, four calcaneus, one fibula) were refilled with porous beta- tricalcium phosphate (PB-TP group) (ChronOS Inject) and of 17 patients (nine humerus, six femur, one calcaneus, one fibula) with hydroxyapatite/calcium sulphate (H/CS group) (CERAMENT|BONE VOID FILLER). There were 13 (62%) preceding fractures in the PB-TP group and eight (47%) in the H/CS group. The follow-up of all patients was at least two years. During follow-up, radiological healing (modified Neer classification), activity level, refracture rates, recurrence rates, resorption period and complications were analyzed. Results In all, 21 patients treated with PB-TP group experienced 27 operations (one refracture, three recurrences and one persistent cyst). A total of 17 patients treated with H/CS experienced 20 operations (one refracture and one 1 Department of Pediatric Orthopedics, University Children's Hospital Basel, Basel, Switzerland 2 Cantonal Hospital Aarau, Aarau, Switzerland 3 Cantonal Hospital Bellinzona, Bellinzona, Switzerland Correspondence should be sent to Andreas Krieg, University Children's Hospital, Switzerland, Spitalstrasse 33, CH-4053, Basel. E-mail: andreas.krieg@ukbb.ch recurrence). After six weeks, 95% in the PB-TP group returned to unrestricted activity with one refracture in the femur due to insufficient biomechanical stability and all returned to unrestricted activity in the H/CS group. Partial or complete radiological response was observed in 81% after 13 months (sd 3.4). Three recurrences (14%) occurred in the PB-TP group and one recurrence (6%) occurred in the H/CS group. The refracture rates were similar in both groups; one (5%) in the PBTP group and one (6%) in the H/CS group. All H/CS treated cysts showed completed resorption after two years, whilst in PB-TP treated cysts no resorption occurred in five cases (25%) (p = 0.031). Two (10%) wound infections occurred in the PBTP group and no infections occurred in the H/CS group. Conclusion Both PB-TP and H/CS can provide stability and prevent refracture in patients with single bone cysts at the upper extremity or the foot. For the proximal femur, additional stabilization is necessary, due to the weight-bearing and associated high refracture rate. The H/CS bone graft substitute has a better resorption rate than the PB-TP graft. [ABSTRACT FROM AUTHOR]