Background
Primary sarcomas originating from the bones of hand and wrist are rare but carry a significant burden of morbidity.
Methods
National Cancer Institute's Surveillance, Epidemiology and End ...Result database from 1975 to 2017 was queried to report incidence and survival data in 237 patients in the United States. Kaplan–Meier and Cox regression were used to determine the prognostic factors affecting survival. χ2 test was used to assess the correlation.
Results
Incidence of hand and wrist sarcoma was 0.017 per 100 000 persons in 2017 and has not significantly changed since 1975 (p > 0.05). Disease‐specific 5‐year and 10‐year survival for the entire cohort was 90% and 84%, respectively. On multivariate analysis race “others,” histology other than “osteosarcoma,” “undifferentiated” grade, and size “≥6 cm” were predictors of worse disease‐specific survival. Cross‐tabulation of race with other significant prognostic factors on univariate analysis revealed a significant correlation of race with every other significant prognostic factor except for grade.
Conclusions
The current study is an analysis of a population‐based registry reporting incidence and survival data for patients with sarcoma of hand and wrist. Independent prognostic factors include race, histology, grade, and size. There is a lack of improvement in survival over the last four decades.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Introduction
Advances in diagnostic and treatment modalities for high grade bone sarcomas (HGBS) of lower extremity (LE) have enabled limb salvage resections as a feasible first-line surgical option. ...However, amputations are still performed. Impact of amputation on survival and predictive factors for amputation and the stage at presentation for HGBS of LE remain unknown.
Methods
National Cancer Database was used to extract 5781 cases of high-grade bone sarcoma of the LE from 2004 to 2017. Kaplan–Meier and Cox regression were used to determine the impact of amputation on survival. Chi square test and logistic regression were used to assess the correlation of predictive factors with amputation and stage at presentation.
Results
Amputation hazard ratio (HR) 1.516; 95% confidence interval (CI) 1.259–1.826;
p
< 0.001 and advanced stage (HR 0.248; 95% CI 0.176–0.351;
p
< 0.001) were independent predictors of poor overall survival. The impact of amputation on survival was most pronounced for pediatric and adolescents and young adults (AYA) age groups (18% decrease in 10-year survival). Amputation was more likely to be performed among those with nonprivate insurance (HR 1.736; 95% CI 1.191–2.531;
p
= 0.004), a finding that was mirrored for advanced stage at presentation (HR 0.611; 95% CI 0.414–0.902;
p
= 0.013).
Discussion
Amputation is an independent predictor of poor outcomes among patients with HGBS of LE. The impact of amputation on survival is the highest for the pediatric and AYA age group. Nonprivate insurance is associated with increased likelihood of amputation and an advanced stage at presentation among patients with high-grade bone sarcoma of the LE. This is the largest study highlighting insurance-related disparities in this cohort.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
The R2Pulm trial was conducted to evaluate the effect of busulfan-melphalan high-dose chemotherapy with autologous stem-cell rescue (BuMel) without whole-lung irradiation (WLI) on event-free survival ...(main end point) and overall survival, compared with standard chemotherapy with WLI in Ewing sarcoma (ES) presenting with pulmonary and/or pleural metastases.
From 2000 to 2015, we enrolled patients younger than 50 years of age with newly diagnosed ES and with only pulmonary or pleural metastases. Patients received chemotherapy with six courses of vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) and one course of vincristine, dactinomycin, and ifosfamide (VAI) before either BuMel or seven courses of VAI and WLI (VAI plus WLI) by randomized assignment. The analysis was conducted as intention to treat. The estimates of the hazard ratio (HR), 95% CI, and
value were corrected for the three previous interim analyses by the inverse normal method.
Of 543 potentially eligible patients, 287 were randomly assigned to VAI plus WLI (n = 143) or BuMel (n = 144). Selected patients requiring radiotherapy to an axial primary site were excluded from randomization to avoid excess organ toxicity from interaction between radiotherapy and busulfan. Median follow-up was 8.1 years. We did not observe any significant difference in survival outcomes between treatment groups. Event-free survival was 50.6% versus 56.6% at 3 years and 43.1% versus 52.9% at 8 years, for VAI plus WLI and BuMel patients, respectively, resulting in an HR of 0.79 (95% CI, 0.56 to 1.10;
= .16). For overall survival, the HR was 1.00 (95% CI, 0.70 to 1.44;
= .99). Four patients died as a result of BuMel-related toxicity, and none died after VAI plus WLI. Significantly more patients in the BuMel arm experienced severe acute toxicities than in the VAI plus WLI arm.
In ES with pulmonary or pleural metastases, there is no clear benefit from BuMel compared with conventional VAI plus WLI.
Background
There are several options for proximal humerus reconstruction in young children after resection of a malignant tumor and no one technique has been definitively shown to be superior to ...others, leaving the decision to surgeon and patient choice. Claviculo pro humeri (CPH) is a biologic reconstruction of the proximal humerus using the patient’s ipsilateral clavicle as a rotational osseous flap. CPH represents a potential option for this complicated clinical problem in very young children, but little is known about it because the indications for its use are so uncommon.
Questions/purposes
The purposes of this study were to (1) assess the oncologic outcomes of CPH at a minimum of 2 years in a small series of patients; (2) elicit the complications associated with this procedure; and (3) show the Musculoskeletal Tumor Society (MSTS) functional score of these patients.
Methods
Four patients (average age, 5 years 11 months; range, 4 years 5 months to 8 years 9 months at the time of surgery) were treated with CPH for reconstruction after resection of a proximal humerus sarcoma; this represented all of the patients treated with this approach for this problem between January 2008 and April 2011 at one institution. During this period, the general indications for using CPH were the need to reconstruct a proximal humerus defect in a child younger than 10 years of age. During this time, CPH was used for all patients treated for proximal humerus sarcomas meeting these criteria. Patient demographics, diagnosis, tumor size and extent, operative details, radiographs and MRIs, complications, and functional outcomes were assessed.
Results
All are alive with no evidence of disease at a minimum followup of 31 months (average, 43 months; range, 31–58 months). Two patients developed nonunion and underwent revision surgery. Osseous union and a stable neoshoulder articulation were ultimately obtained in all patients. Limited shoulder motion was the only functional deficit noted with forward elevation ranging between 30° and 90°. MSTS functional scores were excellent with a range of 87% to 90%.
Conclusions
This is a rarely used procedure in North America but we achieved functional limb salvage in all four patients. Consistent with prior literature, nonunion was the major complication in this series. The two nonunions were successfully treated without interruption of chemotherapy or significant bone graft donor site morbidity. Based on these results, the authors suggest that this procedure is a reasonable reconstruction option to consider after proximal humerus resection in patients younger than 10 years of age. Further followup will be required to assess long-term results and to determine how this procedure compares with the alternatives.
Level of Evidence
Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UL, UM, UPUK, VKSCE, ZAGLJ
Abstract Background Children living in poverty and those of marginalized race or ethnicity experience inferior disease outcomes across many cancers. Whether survival disparities exist in osteosarcoma ...is poorly defined. We investigated the association between race, ethnicity, and proxied poverty exposures and event-free and overall survival for children with nonmetastatic osteosarcoma receiving care on a cooperative group trial. Methods We conducted a retrospective cohort study of US patients with nonmetastatic, osteosarcoma aged 5-21 years enrolled on the Children’s Oncology Group trial AOST0331. Race and ethnicity were categorized to reflect historically marginalized populations, as Hispanic, non-Hispanic Black, non-Hispanic Other, and non-Hispanic White. Poverty was proxied at the household and neighborhood levels. Overall survival and event-free survival functions of time from trial enrollment were estimated using the Kaplan–Meier method. Hypotheses of associations between risks for event-free survival, death, and postrelapse death with race and ethnicity were assessed using log-rank tests. Results Among 758 patients, 25.6% were household-poverty and 28.5% neighborhood-poverty exposed. Of the patients, 21% of children identified as Hispanic, 15.4% non-Hispanic Black, 5.3% non-Hispanic Other, and 54.0% non-Hispanic White. Neither household or neighborhood poverty nor race and ethnicity were statistically significantly associated with risks for event-free survival or death. Postrelapse risk for death differed statistically significantly across race and ethnicity with non-Hispanic Black patients at greatest risk (4-year postrelapse survival 35.7% Hispanic vs 13.0% non-Hispanic Black vs 43.8% non-Hispanic Other vs 38.9% non-Hispanic White; P = .0046). Conclusions Neither proxied poverty exposures or race and ethnicity were associated with event-free survival or overall survival, suggesting equitable outcomes following frontline osteosarcoma trial-delivered therapy. Non-Hispanic Black children experienced statistically significant inferior postrelapse survival. Investigation of mechanisms underlying postrelapse disparities are paramount.
Monoclonal antibodies directed against insulin-like growth factor-1 receptor (IGF-1R) have shown activity in patients with relapsed Ewing sarcoma. The primary objective of Children's Oncology Group ...trial AEWS1221 was to determine if the addition of the IGF-1R monoclonal antibody ganitumab to interval-compressed chemotherapy improves event-free survival (EFS) in patients with newly diagnosed metastatic Ewing sarcoma.
Patients were randomly assigned 1:1 at enrollment to standard arm (interval-compressed vincristine/doxorubicin/cyclophosphamide alternating once every 2 weeks with ifosfamide/etoposide = VDC/IE) or to experimental arm (VDC/IE with ganitumab at cycle starts and as monotherapy once every 3 weeks for 6 months after conventional therapy). A planned sample size of 300 patients was projected to provide 81% power to detect an EFS hazard ratio of 0.67 or smaller for the experimental arm compared with the standard arm with a one-sided α of .025.
Two hundred ninety-eight eligible patients enrolled (148 in standard arm; 150 in experimental arm). The 3-year EFS estimates were 37.4% (95% CI, 29.3 to 45.5) for the standard arm and 39.1% (95% CI, 31.3 to 46.7) for the experimental arm (stratified EFS-event hazard ratio for experimental arm 1.00; 95% CI, 0.76 to 1.33; 1-sided,
= .50). The 3-year overall survival estimates were 59.5% (95% CI, 50.8 to 67.3) for the standard arm and 56.7% (95% CI, 48.3 to 64.2) for the experimental arm. More cases of pneumonitis after radiation involving thoracic fields and nominally higher rates of febrile neutropenia and ALT elevation were reported on the experimental arm.
Ganitumab added to interval-compressed chemotherapy did not significantly reduce the risk of EFS event in patients with newly diagnosed metastatic Ewing sarcoma, with outcomes similar to prior trials without IGF-1R inhibition or interval compression. The addition of ganitumab may be associated with increased toxicity.
In this special edition update on soft tissue sarcomas (STS), we cover classifications, emerging technologies, prognostic tools, radiation schemas, and treatment disparities in extremity and truncal ...STS. We discuss the importance of enhancing local control and reducing complications, including the role of innovative imaging, surgical guidance, and hypofractionated radiation. We review advancements in systemic and immunotherapeutic treatments and introduce disparities seen in this vulnerable population that must be considered to improve overall patient care.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Abstract Background Multimodal risk-adapted treatment is used in paediatric protocols for synovial sarcoma (SS). Retrospective analyses suggest that low-risk SS patients can be safely treated with ...surgery alone, but no prospective studies have confirmed the safety of this approach. This analysis pooled data from the two prospective clinical trials to assess outcomes in SS patients treated with a surgery-only approach and to identify predictors of treatment failure. Methods Patients with localised SS enrolled on the European paediatric Soft tissue sarcoma Study Group (EpSSG) NRSTS2005 and on the Children Oncology Group (COG) ARST0332 trials, treated with surgery alone were eligible for this analysis. Patients must have undergone initial complete resection with histologically free margins, with a grade 2 tumour of any size or a grade 3 tumour ≤5 cm. Results Sixty patients under 21 years of age were eligible for the analysis; 36 enrolled in the COG (from 2007 to 2012) and 24 in the EpSSG study (from 2005 to 2012). The 3-year event-free survival was 90% (median follow-up 5.2 years, range 1.9–9.1). All eight events were local tumour recurrence, whereas no metastatic recurrences were seen. All patients with recurrence were effectively salvaged, resulting in 100% overall survival. Conclusion This joint prospective analysis showed that patients with adequately resected ≤5 cm SS, regardless of grade, can be safely treated with a surgery-only approach. Avoiding the use of adjuvant chemotherapy and radiotherapy in this low-risk patient population may decrease both short- and long-term morbidity and mortality.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
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