Metastatic adult-type non-rhabdomyosarcoma soft tissue sarcomas in children and adolescents: A cohort study from the European paediatric Soft tissue sarcoma Study Group

Background

Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined.

Methods

This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group. Treatment programs were originally designed for patients with metastatic rhabdomyosarcoma, i.e., nine courses of multidrug chemotherapy (with or without bevacizumab in the BERNIE study), followed by 12 cycles of maintenance therapy, whereas radiotherapy and/or surgery (on primary tumor and/or metastases) were delayed until after seven courses of chemotherapy had been administered.

Results

The study included 61 patients <21 years old treated from July 2008 to December 2016. The lung was the site of metastases in 75% of the cases. All patients received multi-agent chemotherapy, 44% had local therapy to primary tumor, and 18% had treatment of metastases. Median time to progression/relapse was 6 months. A high rate of tumor progression was observed during the initial part of the chemotherapy program. With a median follow-up of 41.5 months (range, 2–111 months), 3-year event-free survival and overall survival were 15.4% (95% confidence interval [CI], 7.6–25.7) and 34.9% (95% CI, 22.7–47.5), respectively. There were no statistically significant differences in outcome depending on the type of treatment administered.

Conclusions

The study confirmed the overall poor outcome for patients with metastatic NRSTS, whose treatment remains a challenge.

Plain Language Summary

• Pediatric non-rhabdomyosarcoma soft tissue sarcomas form a heterogeneous group of rare tumors.
• Although recent international studies have defined the standard of care for patients with localized disease, limited data are available on the clinical behavior of patients with distant metastases.
• This study on 61 metastatic cases treated on two prospective European protocols confirms that the chances of survival of such patients are often dismal and a standard treatment is still lacking.

     

Perinatal and familial risk factors for soft tissue sarcomas in childhood through young adulthood: A population-based assessment in 4 million live births

Perinatal factors have been associated with soft tissue sarcomas (STS) in case-control studies. However, (i) the contributions of factors including fetal growth remain unknown, ( ii ) these factors have not been examined in cohort studies and (iii) few assessments have evaluated risk in specific STS subtypes. We sought to identify the role of perinatal and familial factors on the risk of STS in a large population-based birth cohort. We identified 4,023,436 individuals in the Swedish Birth Registry born during 1973–2012. Subjects were linked to the Swedish Cancer Registry, where incident STS cases were identified. We evaluated perinatal and familial factors obtained from Statistics Sweden, including fetal growth, gestational age, and presence of a congenital malformation. Poisson regression was used to estimate incidence rate ratios (IRRs) and 95% confidence intervals (CIs) for associations between perinatal factors and STS overall, as well as by common subtypes. There were 673 individuals diagnosed with STS in 77.5 million person-years of follow-up. Having a congenital malformation was associated with STS (IRR = 1.70, 95% CI: 1.23–2.35). This association was stronger (IRR = 2.90, 95% CI: 1.25–6.71) in recent years (2000–2012). Low fetal growth was also associated with STS during the same time period (IRR = 1.86, 95% CI: 1.05–3.29). Being born preterm was associated with rhabdomyosarcoma (IRR = 1.74, 95% CI: 1.08–2.79). In our cohort study, those with congenital malformations and other adverse birth outcomes were more likely to develop a STS compared to their unaffected contemporaries. These associations may point to disrupted developmental pathways and genetic factors influencing the risk of STS.     

Liquid‐based cytology of pigmented phaeohyphomycotic lesion of the palm masquerading as a metastatic tumor

Phaeohyphomycosis is a chronic subcutaneous fungal infection caused by dematiaceous fungi, commonly involving the extremities, which present as single or multiple lesions mimicking a benign tumor. Fine‐needle aspiration cytology (FNAC) is an easy method for the diagnosis of the lesion. Morphology of the fungus is characteristic with no difficulty for cytopathologist for identification; with a close differential diagnosis of Candida and Aspergillosis. The special stains like periodic acid schiff (PAS) highlights the fungus and the Masson Fontana confirm the melanin pigment and dematiaceous nature of the fungi. Liquid‐based cytology (LBC) preparation of the aspirated material was attempted, which is not usually performed on day to day basis. However, in our case the conventional smears were better for appreciating the fungal morphology.     

Three-dimensional soft tissue prediction in orthognathic surgery: a clinical comparison of Dolphin,ProPlan CMF,and probabilistic finite element modelling

Three-dimensional surgical planning is used widely in orthognathic surgery. Although numerous computer programs exist, the accuracy of soft tissue prediction remains uncertain. The purpose of this study was to compare the prediction accuracy of Dolphin, ProPlan CMF, and a probabilistic finite element method (PFEM). Seven patients (mean age 18 years; five female) who had undergone Le Fort I osteotomy with preoperative and 1-year postoperative cone beam computed tomography (CBCT) were included. The three programs were used for soft tissue prediction using planned and postoperative maxillary position, and these were compared to postoperative CBCT. Accurate predictions were obtained with each program, indicated by root mean square distances: RMS_Dolphin = 1.8 ± 0.8 mm, RMS_ProPlan = 1.2 ± 0.4 mm, and RMS_PFEM = 1.3 ± 0.4 mm. Dolphin utilizes a landmark-based algorithm allowing for patient-specific bone-to-soft tissue ratios, which works well for cephalometric radiographs but has limited three-dimensional accuracy, whilst ProPlan and PFEM provide better three-dimensional predictions with continuous displacements. Patient or population-specific material properties can be defined in PFEM, while no soft tissue parameters are adjustable in ProPlan. Important clinical considerations are the topological differences between predictions due to the three algorithms, the non-negligible influence of the mismatch between planned and postoperative maxillary position, and the learning curve associated with sophisticated programs like PFEM.     

Percutaneous soft tissue release performed using a blunt cannula in rabbits with chronic collagenase-induced Achilles tendinopathy

Background

This study investigated the effects of percutaneous soft tissue release (PSTR) performed using a blunt cannula on (1) the inflammatory cells-count, (2) expressions of calcitonin gene-related peptide (CGRP) and (3) substance P (SP) in rabbits with chronic phase of collagenase-induced Achilles tendinopathy.

A review of suture anchors

Suture anchors are designed to allow soft tissue to bone healing in cases where inadequate soft tissue stock on bone makes it impossible to perform a direct soft tissue-to-soft tissue repair. Suture anchor fixation is one of the most important innovations in arthroscopic glenohumeral shoulder surgery, optimizing the link between bone and soft tissue at the rotator cuff footprint. Newer developments and techniques have expanded the use of suture anchors to most other musculoskeletal tissues including the hip, elbow, hand, knee foot and ankle.Their success depends on having an understanding of the biology and biomechanics that affect their use and knowing the factors that may affect the clinical outcome. This review concentrates on the design, insertion technique, composition and evolution of suture anchors in orthopaedic surgery and the ways in which they are utilised in different tissues.     

Assessment of gas chromatography methodology approach for the trace evaluation of carcinogenic impurity,methyl chloride,in trimetazidine dihydrochloride

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