Aneurysmal bone cysts (ABCs) are benign bone lesions arising predominantly in the pediatric population that can cause local pain, swelling, and pathologic fracture. Primary lesions, which constitute roughly two thirds of all ABCs, are thought to be neoplastic in nature, with one third of ABCs arising secondary to other tumors. Diagnosis is made with various imaging modalities, which exhibit characteristic features such as Bfluid-fluid levels,^although biopsy is critical, as telangiectatic osteosarcoma cannot be excluded based on imaging alone. Currently, the standard of care and most widely employed treatment is intralesional curettage. However, tumor recurrence with curettage alone is common and has driven some to propose a multitude of adjuvants with varying efficacy and risk profiles. Historically, therapies such as en bloc resection or radiation therapy were utilized as an alternative to decrease the recurrence rate, but these therapies imposed high morbidity. As a result, modern techniques now seek to simultaneously reduce morbidity and recurrence, the pursuit of which has produced preliminary study into minimally invasive percutaneous treatments and medical management.
BACKGROUND CONTEXT Osteosarcoma (OGS) and Ewing sarcoma (EWS) are the two classic primary malignant bone tumors. Due to the rarity of these tumors, evidence on demographics, survival determinants, and treatment outcomes for primary disease of the spine are limited and derived from small case series. PURPOSE To use population-level data to determine the epidemiology and prognostic indicators in patients with OGS and EWS of the osseous spine. STUDY DESIGN/SETTING Large-scale retrospective study. PATIENT SAMPLE Patients diagnosed with OGS and EWS of the spine in the Surveillance, Epidemiology, and End Results (SEER) registry from 1973 to 2012. OUTCOME MEASURES Overall survival (OS) and disease-specific survival (DSS). METHODS Two separate queries of the SEER registry were performed to identify patients with OGS and EWS of the osseous spine from 1973–2012. Study variables included age, sex, race, year of diagnosis, tumor size, extent of disease (EOD), and treatment with surgery and/or radiation therapy. Primary outcome was defined as OS and DSS in months. Univariate survival analysis was performed using the Kaplan-Meier method and the log-rank test. Multivariate analysis was performed using Cox proportional hazards regression models. RESULTS The search identified 648 patients with primary OGS and 736 patients with primary EWS of the spine from 1973 to 2012. Mean age at diagnosis was 48.1 and 19.9 years for OGS and EWS, respectively, with OGS showing a bimodal distribution. The median OS and DSS were 1.3 and 1.7 years, respectively, for OGS, with OGS in Paget’s disease having worse OS (0.7 years) relative to the mean (log-rank p=.006). The median OS and DSS for EWS were 3.9 and 4.3 years, respectively. Multivariate cox regression analysis showed that age (OS p<.001, DSS p<.001), decade of diagnosis (OS p=.049), surgical resection (OS p<.001, DSS p<.001), and EOD (OS p<.001, DSS p<.001) were independent positive prognostic indicators for spinal OGS; radiation therapy predicted worse OS (hazard ratio [HR] 1.48, confidence interval [CI] 1.05–2.10, p=.027) and DSS (HR 1.74, CI 1.13–2.66, p=.012) for OGS. For EWS, age (OS p<.001, DSS p<.001), surgical resection (OS p=.030, DSS p=.046), tumor size (OS p<.001, DSS p<.001), and EOD (OS p<.001, DSS p<.001) were independent determinants of improved survival; radiation therapy trended toward improved survival but did not achieve statistical significance for both OS (HR 0.76, CI 0.54–1.07, p=.113) and DSS (0.76, CI 0.54, 1.08, p=.126). CONCLUSIONS Age, surgical resection, and EOD are key survival determinants for both OGS and EWS of the spine. Radiation therapy may be associated with worse outcomes in patients with OGS, and is of potential benefit in EWS. Overall prognosis has improved in patients with OGS of the spine over the last four decades.
Introduction The goal of early-onset scoliosis (EOS) treatment is to improve health-related quality of life (HRQoL) for patients and to reduce the burden on their parents or caregivers. The purpose of this study is to develop and finalize the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24), and examine the validity, reliability, and responsiveness of the EOSQ-24 in measuring patients’ HRQoL, the burden on their caregivers, and the burden on their caregiver’s finances. The study also established aged-matched normative values for the EOSQ-24. Methods The EOSQ-24 was administered to caregivers of male and female patients aged 0 to 18 years with EOS. Patients with EOS are diagnosed before 10 years of age. Criterion validity was investigated by measuring agreement between its scores and pulmonary function testing. Construct validity was established by comparing values across different etiology groups using the known-group method, and measuring internal consistency reliability. Content validity was confirmed by reviewing caregiver and health provider ratings for the relativity and clarity of the EOSQ-24 questions. Test-retest reliability was examined through intraclass correlation coefficients. Responsiveness of the EOSQ-24 before and after surgical interventions was also investigated. Age-matched, healthy patients, without spinal deformity, were enrolled to establish normative EOSQ-24 values. Results The pulmonary function subdomain score in the EOSQ-24 was positively correlated with pulmonary function testing values, establishing criterion validity. The EOSQ-24 scores for neuromuscular patients were significantly decreased compared with idiopathic or congenital/structural patients, demonstrating known-group validity. Internal consistency reliability of patients’ HRQoL was excellent (0.92), but Family Burden was questionable (0.64) indicating that Parental Burden and Financial Burden should be in separate domains. All 24 EOSQ items were rated as essential and clear, confirming content validity. All EOSQ-24 domains demonstrated good to excellent agreement (0.68 to 0.98) between test and retest scores. Neuromuscular patients improved their HRQoL postoperatively, whereas idiopathic patients worsened their HRQoL postoperatively, indicating the ability of the instrument to respond to different trajectories of HRQoL according to etiology in patients with EOS. Discussion The developed and finalized EOSQ-24 is a valid, reliable, and responsive instrument that is able to serve as a patient-reported outcome measure evaluating health status for patients with EOS and burden of their caregivers. This instrument will be able to serve as an outcome measure for future research including clinical trials evaluating the effectiveness of various treatments. In addition, the EOSQ-24 allows assessment of patients’ HRQoL, and the burden on their caregivers relative to age-matched healthy peers. Level of Evidence Level II—diagnostic study with consecutive patients enrolled in national registries.
Outpatient ACDF is increasing in frequency nationwide over the past several years. Nationwide data demonstrate a greater risk of perioperative surgical complications, including revision anterior and posterior fusion, as well as a higher risk of postoperative acute renal failure. Candidates for outpatient ACDF should be counseled and carefully selected to reduce these risks.
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