Extraosseous Ewing Sarcoma in Children: A Systematic Review and Meta-Analysis of Clinicodemographic Characteristics.

Background: We conducted this systematic review to provide comprehensive evidence on the prevalence, clinical features and outcomes of young extraosseous Ewing sarcoma (EES) cases. Methods: PubMed, Scopus, Web of Science, and Google Scholar were searched for articles reporting the occurrence of EES among children and adolescents (<21 years). The primary outcome included the rate of occurrence of EES among children and adolescents, while the secondary outcomes included the descriptive analyses of the demographic characteristics, tumor characteristics, and clinical outcomes of the affected cases. The data are reported as the effect size (ES) and its corresponding 95% confidence interval (CI). Results: A total of 29 studies were included. Twenty-four reported instances of childhood disease among all the EES cases [ES = 30%; 95%CI: 29−31%], while five studies reported extraosseous cases among the pediatric EES cases [ES = 22%; 95%CI: 13−31%]. The thorax is the most common location of childhood EES [33%; 95%CI: 20−46%] followed by the extremities [31%; 95%CI: 22−40%]. Concurrent chemotherapy and radiotherapy [57%; 95%CI: 25−84%] was the most commonly implemented management protocol in the pediatric EES cases. The rate of no evidence of disease and 5-year overall survival was 69% for both outcomes. Mortality occurred in 29% of cases, while recurrence and secondary metastasis occurred in 35% and 16% of cases, respectively. Conclusions: Our findings provide insight into the clinical features and outcomes of EES among children and adolescents.

Size of ischial fibro-ostosis is associated with heterotopic ossification after total hip arthroplasty.

PURPOSE: The hypothesis of the present study was that degenerative fibro-ostosis (FO) of the ischial hamstring tendon insertion is a risk factor for heterotopic ossification (HO) following THA. METHODS: We followed 103 consecutive patients (43 males, 60 females, mean age 61 years) who underwent unilateral cementless THA for primary hip osteoarthritis and investigated the incidence of HO within the first 12 months after surgery. On pre-operative radiographs, a standardized evaluation for FO of the ischial hamstring tendon insertion concerning horizontal, vertical, and square dimensions was performed. HO was classified according to Brooker on radiographs at 12 months post-operatively. RESULTS: At follow-up, 56 patients (54%) had no radiographic evidence of HO, 23 (22%) were classified as Brooker I, 17 (17%) as II, 6 (6%) as III, and 1 (1%) as IV, respectively. Patients with post-operative HO had significantly greater vertical (3.0 mm vs. 2.3 mm, p = 0.001) and horizontal (47.9 mm vs. 39.1 mm, p = 0.025) dimensions of FO than patients without HO. Patients with FO and a vertical dimension of ≥ 2.5 mm were more likely to develop HO (55.6%) than patients with a vertical FO dimension of less than 2.5 mm (34.7%, OR = 2.35 p = 0.047). A weak correlation between the vertical and horizontal size of FO and the severity of HO was observed. CONCLUSION: Radiographic evidence of asymptomatic FO is a potential risk factor for the development of HO following THA and may be used as a simple diagnostic tool to pre-operatively identify patients at risk for post-operative HO. This association has not been previously described and further research to confirm the present findings and to justify additional prophylactic treatment in these patients is warranted.

What Sport Activity Levels Are Achieved in Patients After Resection and Endoprosthetic Reconstruction for a Proximal Femur Bone Sarcoma?

BACKGROUND: Limited information is available about sports activities of survivors after resection and reconstruction of primary malignant bone tumors with megaprostheses. Because patients often ask what activities are possible after treatment, objective knowledge about sports activities is needed to help assess the risks of sports participation and to help guide patients' expectations. QUESTIONS/PURPOSES: The aims of this study were to evaluate (1) what proportion of patients with proximal-femoral megaprostheses placed as part of tumor reconstructions can perform sports; (2) what activity levels they achieved; and (3) whether sports activity levels are associated with an increased likelihood of revision. METHODS: This retrospective study considered all 27 living patients in our institutional tumor registry with enduring proximal-femoral reconstructions performed more than 5 years ago who were between the ages of 11 and 49 years at the time of the reconstruction; seven were lost to followup and one was excluded because of paraplegia as a result of a car accident and another because of senile dementia; another two were excluded from statistics because of growing prostheses and skeletal immaturity at the time of followup, leaving 16 (11 male, five female) for analysis. Their mean age was 26 ± 12 years (range, 11-49 years) at surgery, and the mean followup was 18 ± 7 years (range, 5-27 years). Types of sports, frequency per week, duration of each sports session as well as the UCLA and modified Weighted Activity Score were assessed retrospectively by an independent assessor a median of 18 years (range, 5.3-27 years) after surgery. RESULTS: Patients recalled that preoperatively 14 were practicing sports 5 (± 4) hours/week. At followup, 11 of the patients were practicing one or more sports activities 2 (± 3) hours/week on a regular basis. The preoperative UCLA and modified Weighted Activity Score levels of 9 and 6 fell to levels of 6 (p = 0.005) and 3 (p = 0.025), respectively, at followup. With the numbers of patients available for study, we could not determine that prosthetic failures were associated with sport activity levels. CONCLUSIONS: Patients who survive primary malignant bone tumors in the proximal femur reconstructed by megaprostheses are able to perform some sports activities. The estimates of activity levels made in this study probably are best-case estimates, given that some patients were lost to followup; patients unaccounted for might not be doing as well as those represented here. Also, the degree to which sports participation influences implant durability remains, for the most part, unanswered; studies with more patients and longer followup will be needed to determine to what degree prosthesis survivorship relates to sporting activity levels. Most patients perform low-impact sports and at a lower level than they had preoperatively. Because this is a preliminary study of a select group of patients, further information is necessary to weight the benefits of higher sports activity levels against potential risks. If this can be confirmed in a larger number of patients, the information may guide surgeons in their discussion with patients preoperatively and give them some objective assessment of what to expect regarding sports activities. LEVEL OF EVIDENCE: Level IV, therapeutic study.