The treatment results in patients with Ewing Sarcoma: The Polish Sarcoma Group Experience.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e23503-e23503
Author(s):  
Iwona Lugowska ◽  
Paulina Jagodzinska-Mucha ◽  
Anna Raciborska ◽  
Katarzyna Bilska ◽  
Hanna Kosela-Paterczyk ◽  
...  

e23503 Background: Ewing Sarcoma is a rare type of aggressive tumor that occurs in bones or in the soft tissue around the bones. The multidisciplinary approach chemotherapy (CHT), radiotherapy (RTH) and surgery (S) is the standard of care. The aim of our study was to analyze prognostic factors and treatment results in patients with Ewing Sarcoma. Methods: 504 patient with Ewing sarcoma treated between 1998 and 2019, F:M 1:1.34, with metastases at presentation was 195 patients (39%), axial localization was in 277 patients (55%). All patient were treated with multimodal treatment based on chemotherapy, radiotherapy and surgery. Kaplan-Meier estimator, log rank test and multivariate Cox model were used for statistical analysis. Results: Five year overall survival (5y-OS) was 54% (CI:49-60%), prognostic factors were: gender (5y-OS for females and males equal to 63% and 48%, respectively), age (5y-OS stratified patients between < 10y; 10-15y; 15-25 and 25+ was 65%, 70%, 41% and 55%;p < 0.001), metastases (5y-OS in group with M1 was 31% and in M0 - 70%; p < 0.001), type of local treatment (5y-OS in group CHT/S/RTH was 61%, CHT/S 59%, only RTH 50% and palliative treatment without local treatment equals 12%; p < 0.001). Multivariate Cox analysis revealed death hazard dependence on gender (male vs. female; HR = 1.80; p < 0.01), metastatic status (M1 vs. M0; HR = 2.91; p < 0.001), bone/bone marrow involvement (present vs. absent; HR = 2.10; p < 0.01) and chemotherapy regime (other vs. VIDE/VAI; HR = 0.69; p = 0.049). Conclusions: The treatment results in Ewing sarcoma are significantly better in children than in adults, which can be related to the more intensive chemotherapy regiments applied. The treatment should be provided in referral centers and based on up-to-dated guideline.

2021 ◽  
Vol 10 (16) ◽  
pp. 3627
Author(s):  
Paulina Jagodzińska-Mucha ◽  
Anna Raciborska ◽  
Hanna Koseła-Paterczyk ◽  
Katarzyna Kozak ◽  
Katarzyna Bilska ◽  
...  

Ewing sarcoma (ES) is a rare and aggressive disease that requires multidisciplinary treatment with the use of chemotherapy, radiotherapy, and surgery. Our retrospective study aimed to analyze the prognostic factors and treatment results in different age groups of patients. Between 1998 and 2018, 569 patients with ES were treated in two referral centers. The patients were divided into four age groups (≤10 years; 11–18 years; 19–25, and >25). The treatment results and prognostic factors were assessed for each group. For statistical analyses, we used the Chi2 test, the Kaplan–Meier estimator with a log-rank test, and the multivariate Cox model. Five-year overall survival (OS) rate was 56%. In the age subgroups: ≤10 years, 11–18 years, 19–25 years, and >25 years, the 5-year OS rates were 75%, 58%, 41%, and 52%, respectively. Favorable prognostic factors: female gender (p = 0.024), non-axial localization (p = 0.005), VIDE regimen (p < 0.001), and surgery as a local treatment (p < 0.001) dominated in the group ≤10 years. In multivariate analysis, male (HR = 1.53), axial localization (HR = 1.46), M1 status at presentation (HR = 2.64), and age > 10 years (HR = 2.29) were associated with shorter OS. The treatment results in ES are significantly better in children aged ≤10 years; the challenge is to provide therapy for adolescents and young adults. The diagnostics and treatment of ES patients must be provided in referral centers.


2020 ◽  
Author(s):  
Yongfeng Li ◽  
Daobao Chen ◽  
Haojun Xuan ◽  
Mihnea P. Dragomir ◽  
George A. Calin ◽  
...  

Abstract Background Metaplastic breast cancer (MBC) is a rare type of breast cancer with an increasing incidence, we aim to develop clinical nomograms to predict the overall survival and cancer-specific survival for patients with MBC.MethodsPatients data were collected from the SEER database between 1973 and 2015. All included patients were randomly assigned into the training and validation sets. Univariate and multivariate Cox analysis were performed to identify independent prognostic factors of MBC. These essential prognostic variables were combined to construct nomogram models to predict overall survival (OS) and cancer-specific survival (CSS) in patients with MBC. Model performance was evaluated by concordance index (C-index) and calibration plots.ResultsA total of 1835 patients were collected and divided into the training (1223) and validation (612) groups. The multivariate Cox model identified age, TNM stage, T stage, and N stage, chemotherapy and radiotherapy as independent covariates associated with OS, while these variables except for age and chemotherapy were independent prognostic factors of CSS. The nomogram constructed based on these covariates demonstrated excellent accuracy in estimating 3-, and 5-year OS and CSS, with a C-index of 0.759 (95% CI, 0.746-0.772) for OS and 0.766 (95% CI, 0.751-0.781) for CSS in the training cohort. In the validation cohort, the nomogram-predicted C-index was 0.754 for OS (95%CI, 0.734-0.774) and 0.752 (95%CI, 0.728-0.776) for CSS. All calibration curves exhibited good consistency between predicted and actual survival.ConclusionsThese nomogram models established in this study can help to enhance the accuracy of prognostic prediction, which may thereby improve individualized assessment of survival risks and facilitate to provide constructive therapeutic suggestions.


2020 ◽  
Author(s):  
Yongfeng Li ◽  
Daobao Chen ◽  
Haojun Xuan ◽  
Mihnea P. Dragomir ◽  
George A. Calin ◽  
...  

Abstract Background Metaplastic breast cancer (MBC) is a rare type of breast cancer with an increasing incidence, we aim to develop clinical nomograms to predict the overall survival and cancer-specific survival for patients with MBC.MethodsPatients data were collected from the SEER database between 1973 and 2015. All included patients were randomly assigned into the training and validation sets. Univariate and multivariate Cox analysis were performed to identify independent prognostic factors of MBC. These essential prognostic variables were combined to construct nomogram models to predict overall survival (OS) and cancer-specific survival (CSS) in patients with MBC. Model performance was evaluated by concordance index (C-index) and calibration plots.ResultsA total of 1129 patients were collected and divided into the training (753) and validation (376) groups. The multivariate Cox model identified age, stage_ajcc, T stage, chemotherapy and radiotherapy as independent covariates associated with OS, while age, race, stage_ajcc, T stage, and radiotherapy were independent prognostic factors of CSS. The nomogram constructed based on these covariates demonstrated excellent accuracy in estimating 3-, and 5-year OS and CSS, with a C-index of 0.744 (95% CI, 0.701-0.787) for OS and 0.746 (95% CI, 0.695-0.797) for CSS in the training cohort. In the validation cohort, the nomogram-predicted C-index was in OS and 0.818 for OS (95% CI, 0.775-0.861) and 0.800 (95% CI, 0.747-0.853) for CSS. All calibration curves exhibited good consistency between predicted and actual survival.ConclusionsThese nomogram models established in this study can help to enhance the accuracy of prognostic prediction, which may thereby improve individualized assessment of survival risks and facilitate to provide constructive therapeutic suggestions.


2020 ◽  
Author(s):  
Yongfeng li ◽  
Daobao chen ◽  
Haojun xuan ◽  
Mihnea P. Dragomir ◽  
George A. Calin ◽  
...  

Abstract Background Metaplastic breast cancer (BMC) is a rare type of breast cancer with an increasing incidence, we aim to develop clinical nomograms to predict the overall survival and cancer-specific survival for patients with BMC.MethodsPatients data were collected from the SEER database between 1973 and 2015. All included patients were randomly assigned into the training and validation sets. Univariate and multivariate Cox analysis were performed to identify independent prognostic factors of MBC. These essential prognostic variables were combined to construct nomogram models to predict overall survival (OS) and cancer-specific survival (CSS) in patients with MBC. Model performance was evaluated by concordance index (C-index) and calibration plots.ResultsA total of 1835 patients were collected and divided into the training (1223) and validation (612) groups. The multivariate Cox model identified age, TNM stage, T stage, and N stage, chemotherapy and radiotherapy as independent covariates associated with OS, while these variables except for age and chemotherapy were independent prognostic factors of CSS. The nomogram constructed based on these covariates demonstrated excellent accuracy in estimating 3-, and 5-year OS and CSS, with a C-index of 0.759(95% CI, 0.746-0.772) for OS and 0.766 (95% CI, 0.751-0.781) for CSS in the training cohort. In the validation cohort, the nomogram-predicted C-index was 0.754 for OS (95%CI, 0.734-0.774) and 0.752 (95%CI, 0.728-0.776) for CSS. All calibration curves exhibited good consistency between predicted and actual survival.ConclusionsThese nomogram models established in this study can help enhance the accuracy of prognostic prediction, which may thereby improve individualized assessment of survival risks and facilitate to provide constructive therapeutic suggestions.


2009 ◽  
Vol 54 (1) ◽  
pp. 19-24 ◽  
Author(s):  
Neriman Sarı ◽  
Giray Toğral ◽  
M. Faik Çetindağ ◽  
B. Şafak Güngör ◽  
İnci Ergürhan İlhan

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 2568-2568
Author(s):  
M. Bonneterre ◽  
N. Penel ◽  
M. Vanseymortier ◽  
E. Dansin ◽  
S. Clisant ◽  
...  

2568 Background: For investigators, the selection of patients to be considered for phase I clinical trials is difficult, because of the lack of objective criteria for a rational decision-making process. From October 1997 to October 2002, we retrospectively assessed prognostic factors for cancer patients considered for Phase 1 trials. Methods: 148 consecutive patients who had been screened for inclusion in 6 different phase I trials were included in the present study. 70 out of them actually received the phase I treatment. Univariate (Log-Rank test) and multivariate analysis (Cox proportional hazard ratio model) were performed to determine the prognostic factors related to overall survival (OS) after screening. Results: The study comprised 63 men and 85 women, with a median age of 54 (range 23–79). The most frequent primary cancer sites were: breast (38 cases), head and neck (28 cases), lung (18 cases) and colorectal (17 cases). 91 out of them had a performance status PS = 0. The median OS of the 148 patients was 5.7 months (173 days, range 1–2,421). Univariate analysis identified PS = 1, Body Mass Index < 20, liver and visceral metastasis, serum albumin < 38 g/L, lymphocytes count < 0.7 x 109/L and granulocytes count > 7.5 x 109/L as poor prognostic factors. The Cox model identified serum albumin < 38 g/L (HR 2.51 [1.51–4.18], p=0.0001) and lymphocyte count < 0.7 x 109/L (HR 2.27 [1.13–4.62], p=0.024) as independent prognostic variables for OS. All patients presenting with both prognostic factors died within 90 days. Conclusion: We propose a simple model, easily obtained at the patient bedside, which can discriminate patients who have a life expectancy of over 3 months and thus could be enrolled in phase-I anti-cancer trials. No significant financial relationships to disclose.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10082-10082
Author(s):  
David Lorente ◽  
Robert Diaz ◽  
Barbara Torres ◽  
Adela Cañete ◽  
Jorge Aparicio ◽  
...  

10082 Background: Treatment of Ewing sarcoma pts. usually follows pediatric protocols, both in children and in adults. However, older patients fare poorly in most series. We analyze our experience with the 2001 protocol of the Spanish Society of Pediatric Oncology. Methods: Retrospective analysis. Schema: 6 cycles (cy) of VIDE chemotherapy (CT: vincristine, ifosfamide, etoposide, doxorrubicin). If no progression, local treatment (surgery or RT) and consolidation adjusted to risk: VACx8 (vincristine, dactinomycin, ciclophosphamyde) in standard-risk pts; if increased risk (axial, complete response in lung metastases or non-pulmonary metastases) VACx1, high-dose CT (busulphan-melphalan) and autologous transplant (ATSP). Analysis: induction CT toxicity, pathological response rates, consolidation treatment, disease-free (DFS) and overall survival (OS) (Kaplan- Meier). Log-rank and Cox regression analysis of prognostic factors in OS. Results: 35 patients (01.2003-05.2011). 60% male. Median age 16 y (r 7-57). Axial (43%), extremities (34%), extra-osseous (18%) and ribs (9%). Metastases: 54% (lung 58%, bone 26%, others 12%). > 1 location: 29%. Induction CT: 83% received 6 cy. 6% early progressions and 3% toxic deaths. 196 cycles of CT. Dose reduction (etoposide) in 60%. Grade 3-4 toxicity: neutropenia 13%, anemia 14%, neutropenic fever 13%, diarrhoea-stomatitis 7%.Local treatment: surgery (49%), radiotherapy (29%), none (22%). In 17 resections, > 90% necrosis in 53%. Consolidation: VACx8 29%; VACx1-ATSP in 34%; 37% other treatments (progression). No ATSP-related mortality. Median follow-up: 36 m ( 5-101 m). Median DFS 25 m (16-34 m). Median OS 28 m (15-41 m), 3-year OS 40%. Median time to progression 7 m (0.4-15 m). Median OS from progression 7 m (0.4-15 m). Age < 15 years, a non-axial primary and no extra-pulmonary metastases were favourable prognostic factors in the univariate analysis. Conclusions: Induction CT with the VIDE regimen is feasible in most patients, with a low risk of early progression. Hematological toxicity is substantial but manageable. Adults patients have a worse prognosis compared to pediatric patients. Unfortunately, survival after progression is dismal.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. 11529-11529
Author(s):  
Leo Mascarenhas ◽  
Allen Buxton ◽  
Steven G. DuBois ◽  
Dian Wang ◽  
Nadia N. Laack ◽  
...  

11529 Background: Maximum tumor dimension > 8 cm. and large tumor volume have been reported to be adverse prognostic factors in patients with ES but have not been prospectively evaluated in the context of a phase 3 clinical trial with interval compressed chemotherapy. Methods: COG AEWS1031 (NCT01231906) was a randomized phase 3 clinical trial comparing interval compressed chemotherapy regimens in patients with newly diagnosed localized ES of bone and soft tissue. A correlative objective of AEWS1031 was to evaluate tumor size and volume as prognostic factors. Institution-reported dimensions of the primary tumor from baseline imaging were prospectively collected. For inclusion in this analysis, patients had to have at least 1 tumor dimension reported for tumor size analyses and dimensions in 3 axes for tumor volume analyses. Maximum dimension was dichotomized as less than vs. > / = 8cm. Tumor volume was dichotomized as less than vs. > / = 200 mL. Event-free (EFS) and overall survival (OS) from enrollment were calculated using Kaplan-Meier methods and compared between groups using a two-sided log-rank test. Hazard ratios (HR) and confidence intervals (CI) were calculated using the Cox model. Results: The 5-year EFS and OS of the 629 eligible patients was 78% (95% CI: 75-81%) and 87% (95% CI: 84-90%) respectively and there was no significant difference in both EFS and OS between the randomized interval compressed chemotherapy arms of AEWS1031. 590 of 629 (94%) patients were evaluable for maximum tumor dimension and 307 (52%) had tumors > / = 8 cm. Patients with tumors > / = 8 cm were at significantly increased risk for EFS events (p = 0.016) with estimated 5-year EFS of 73.7% (95% CI: 68.1 vs.78.4%) vs. 82.9% (95% CI 77.7-87.1%) for patients with tumors < 8 cm [HR: 1.53 (1.08-2.17)]. For tumor volume, 586 of 629 patients (93%) were evaluable and 180 (31%) had tumors > / = 200 mL. Patients with tumor volume > / = 200 mL were at significantly increased risk for EFS events (p = 0.003) with estimated 5-year EFS of 70% (95% CI: 62.3-76.4%) vs. 81.6% (95% CI: 77.2-85.2%) for patients with tumors < 200 mL [HR: 1.69 (1.2-2.39)]. Conclusions: Maximum tumor dimension and tumor volume as defined are both prognostic in patients with newly diagnosed localized ES treated with interval compressed chemotherapy. Clinical trial information: NCT01231906 .


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 10011-10011
Author(s):  
P. Leavey ◽  
L. Mascarenhas ◽  
N. Marina ◽  
Z. Chen ◽  
M. Krailo ◽  
...  

10011 Background: The prognosis for patients with recurrent Ewing sarcoma (EWS) is very poor with 5-year survival of 13%. Since 30–40% of patients with newly diagnosed, non-metastatic EWS develop recurrence, the Children's Oncology Group (COG) sought to evaluate prognostic factors for these patients. Data was derived from the phase III, multi-institutional study INT 0091. Methods: Between 1988 and 1994, five hundred and seventy-eight eligible patients with previously untreated EWS or PNET of bone were enrolled on INT 0091. Patients who experienced recurrence or disease progression as their first analytic event were considered. Survival was calculated from date of disease progression to death or last patient contact. Comparisons of the risk for death across groups were accomplished using the log-rank test. Survivor functions were estimated by the method of Kaplan and Meier. Results: Two hundred and sixty-two patients experienced disease progression or recurrence. The median time to first recurrence was 1.3 years (range 0–7.4 years) for all patients, 1.4 years (range 0 to 7.4 years) for patients with initially localized disease and 1 year (range 0 to 6 years) for patients with initially metastatic disease. Time to first recurrence from date of initial diagnosis was a predictor of post-recurrence survival (p<0.0001). Twenty-one percent of patients whose disease recurred or progressed experienced first recurrence 2 or more years from initial diagnosis and had an estimated 5 year post-recurrence survival of 30%. This compares with 7% for those whose first recurrence or progression was earlier. No statistical difference was detected when patients whose disease recurred < 12 months were compared with those whose recurrence was 12–24 months from initial diagnosis. Significant risk factors for death after recurrence included metastatic disease at initial diagnosis, elevated LDH at initial diagnosis and female gender. In patients non-metastatic at initial diagnosis pelvic primary site was also a risk factor for death after recurrence. Conclusions: Patients with longer time to first recurrence represent the subset of patients most likely to survive following recurrence. All patients with recurrent Ewing sarcoma would benefit from collaborative trials to investigate new therapeutic options. No significant financial relationships to disclose.


2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Guang Li ◽  
Yan-Ping Song ◽  
Yao Lv ◽  
Zhen-Zhen Li ◽  
Yan-Hua Zheng

Background. Extramedullary disease (EMD), an infrequent manifestation of multiple myeloma (MM), can present at diagnosis or develop during the disease course. EMD can be clinically divided into bone-related EMD (EMD-B) and soft tissue-related EMD (EMD-S). The purpose of our study is to investigate the clinical characteristics, survival outcomes, and prognostic factors of MM patients with EMD. Methods. A total of 155 MM patients with EMD were ultimately enrolled in our study by retrieving the Surveillance, Epidemiology, and End Results (SEER) database. The Kaplan–Meier survival curves and log-rank test for overall survival (OS) and myeloma-specific survival (MSS) were conducted to compare each potential variable. Variables with a p value <0.1 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors, with a hazard ratio (HR) >1 representing adverse prognostic factors. Results. The median age at diagnosis was 63 years old. EMD-B occurred in 99 patients (63.90%), while EMD-S occurred in 56 cases (36.10%). Patients with EMD-S had a significant survival disadvantage in MSS (HR = 1.844, 95% CI 1.117–3.042, p  = 0.017) and OS (HR = 1.853, 95% CI 1.166–2.942, p  = 0.009) compared to those with EMD-B. Patients with EMD interval ≤24 months were at higher risk of death than those with EMD at diagnosis in MSS (HR = 1.885, 95% CI 1.175–3.346, p  = 0.042) and in OS (HR = 1.33, 95% CI 1.119–2.529, p  = 0.036). Patients with EMD interval >24 months were at a lower risk of death as opposed to those with EMD at diagnosis. Conclusion. Age at MM diagnosis, site of EMD, and time interval from diagnosis to EMD occurrence were independent prognostic factors in MM patients with EMD. EMD-B bore a better prognosis than EMD-S.


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