Neoadjuvant Chemotherapy for Peripheral Malignant Neuroectodermal Tumor of Bone: Recent Experience at the Istituto Rizzoli

2000 ◽  
Vol 18 (4) ◽  
pp. 885-885 ◽  
Author(s):  
Gaetano Bacci ◽  
Stefano Ferrari ◽  
Franco Bertoni ◽  
Davide Donati ◽  
Patrizia Bacchini ◽  
...  

PURPOSE: The results achieved in 44 patients with nonmetastatic peripheral neuroectodermal tumor (PNET) of bone treated with neoadjuvant chemotherapy are reported. PATIENTS AND METHODS: A six-drug regimen of chemotherapy (vincristine, doxorubicin, dactinomycin, cyclophosphamide, ifosfamide, and etoposide) was administered to all patients. Local treatment consisted of surgery in 20 patients, surgery followed by radiotherapy in 13, and radiotherapy only in 11. RESULTS: At a mean follow-up of 4.5 years (range, 2 to 7 years), 23 patients (52%) remain event-free, 20 have relapsed (45%), and one has died of chemotherapy-related toxicity. The 5-year event-free survival and overall survival were 54.2% and 62.7%, respectively. To assess the prognostic significance of neural differentiation in the family of Ewing’s sarcoma, these results have been compared with the outcomes of 138 concomitant patients with typical Ewing’s sarcoma (TES) who were treated according to the same protocol. Of these, 103 (75%) remained continuously event-free, 34 (24%) relapsed, and one died of chemotherapy-related toxicity. It follows that PNET patients treated with this chemotherapy regimen have a significantly worse prognosis than typical ES patients (5-year event-free survival, 54.2% v 70.6%, P < .012; 5-year overall survival, 62.7% v 78.3%, P < .002). CONCLUSION: The authors conclude that studies into new adjuvant therapy for Ewing’s sarcoma modulated according to risk of relapse should also consider neural differentiation as a risk factor.

2020 ◽  
Author(s):  
Jie Jiang ◽  
Chong Liu ◽  
Guoyong Xu ◽  
Tuo Liang ◽  
Chaojie Yu ◽  
...  

Abstract Background: Ewing's sarcoma (ES) is the second most prevalent malignancy among bone tissue tumors, and there is no adequate prognosis biomarker. The protein encoded by CCT6A is a molecular chaperone. Early studies have suggested that CCT6A is involved in the development of many cancers, however, there is no clear evidence of a role for CCT6A in ES.Methods: In this study, we performed a bioinformatics analysis of 32 Ewing sarcoma specimens from the GSE17618 dataset for differences in gene expression and overall survival, event-free survival, and gene expression in different subgroups. Results: After three screenings, we identified CCT6A as highly correlated with Ewing's sarcoma prognosis. Survival analysis showed low overall survival (OS) for CCT6A high expression (P=0.024). On the other hand, Cox regression analysis showed that CCT6A expression, event-free survival (EFS), and age were strongly associated with the prognosis of Ewing sarcoma, identified as independent poor prognostic biomarkers. (CCT6A: P=0.015; Age: P-value=0.026; EFS: P-value=0.001). Conclusion: The expression level of CCT6A is strongly associated with the prognosis of Ewing's sarcoma. High expression of the CCT6A gene may serve as a biomarker for poor prognosis in patients with Ewing's sarcoma.


2020 ◽  
Author(s):  
Jie Jiang ◽  
Chong Liu ◽  
Guoyong Xu ◽  
Tuo Liang ◽  
Chaojie Yu ◽  
...  

Abstract Background Ewing's sarcoma (ES) is the second most prevalent malignancy among bone tissue tumors, and there is no adequate prognosis biomarker. The protein encoded by CCT6A is a molecular chaperone. Early studies have suggested that CCT6A is involved in the development of many cancers, however, there is no clear evidence of a role for CCT6A in ES. Methods In this study, we performed a bioinformatics analysis of 32 Ewing sarcoma specimens from the GSE17618 dataset for differences in gene expression and overall survival, event-free survival, and gene expression in different subgroups. Results After three screenings, we identified CCT6A as highly correlated with Ewing's sarcoma prognosis. Survival analysis showed low overall survival (OS) for CCT6A high expression (P = 0.024). On the other hand, Cox regression analysis showed that CCT6A expression, event-free survival (EFS), and age were strongly associated with the prognosis of Ewing sarcoma, identified as independent poor prognostic biomarkers. (CCT6A: P = 0.015; Age: P-value = 0.026; EFS: P-value = 0.001). Conclusion The expression level of CCT6A is strongly associated with the prognosis of Ewing's sarcoma. High expression of the CCT6A gene may serve as a biomarker for poor prognosis in patients with Ewing's sarcoma.


2004 ◽  
Author(s):  
Gaetano Bacci ◽  
Stefano Ferrari ◽  
Alessandra Longhi ◽  
Davide Donati ◽  
Enza Barbieri ◽  
...  

2019 ◽  
Vol 58 (9) ◽  
pp. 1335-1339 ◽  
Author(s):  
Ayako Takigami ◽  
Hideaki Yamasawa ◽  
Ayako Kurosaki ◽  
Noritaka Sakamoto ◽  
Tsugitoshi Onuki ◽  
...  

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e17022-e17022
Author(s):  
Jana Obertova ◽  
Patrik Palacka ◽  
Dalibor Gallik ◽  
Jan Slopovsky ◽  
Michal Chovanec ◽  
...  

e17022 Background: Systemic immune-inflammation index (SII) is a prognostic factor in patients with metastatic urothelial cancer (MUC). The objective of this prospective study was to evaluate the prognostic value of the SII at baseline before neoadjuvant cisplatin-based chemotherapy start in patients with muscle-infiltrating urothelial cancer (MIUC). Methods: Seventy-two patients (49 men) with MIUC (71 bladder, 1 upper tract) were treated with cisplatin-based neoadjuvant chemotherapy (NACT). Thitrty-nine patients (pts.) underwent radical cystectomy (RC), 18 pts. external radiotherapy (EXRT) with concomitant cisplatin chemotherapy, and 15 pts. had no local treatment (NLT) afterwards. SII was defined as PxN/L, based on platelets (P), neutrophils (N) and lymphocytes (L) counts. This study population was dichotomized by median SII into low and high groups. Disease-free survival (DFS) and overall survival (OS) were estimated by Kaplan-Meier method and compared with logrank test. Results: At median follow-up of 13 months (range 2-40), 26 pts. relapsed and 24 of them died. Pts. with low SII at baseline had better DFS and OS compared to those with high SII (NR vs. 19 months, HR = 0.37, 95% CI 0.17-0.88, P = 0.0185 for DFS, 31 months vs. 20 months, HR = 0.43, 95% CI 0.19-0.98, P = 0.052 for OS, respectively). Subgroup analysis showed significant benefit for EXRT regarding of SII, however no differences were observed in both, RC and NL. Conclusions: The level of SII at baseline before neoadjuvant cisplatin-based chemotherapy initiation predicted survival in MIUC pts. Based on the level of SII, stratification of pts. into clinical trials could be possible. Pts. with high level of SII might be the candidates for the different therapeutic approaches. Key Words: Muscle-Infiltrating Urothelial Carcinoma. Systemic Immune-Inflammation Index. Neoadjuvant Chemotherapy. Disease-Free Survival. Overall Survival.


2016 ◽  
Vol 97 (3) ◽  
pp. 365-368 ◽  
Author(s):  
Yinghui Ding ◽  
Zhenlin Huang ◽  
Yafei Ding ◽  
Zhankui Jia ◽  
Chaohui Gu ◽  
...  

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 180-181
Author(s):  
L. Chatzis ◽  
V. Pezoulas ◽  
A. Goules ◽  
I. Stergiou ◽  
C. Mavragani ◽  
...  

Background:Sjögren’s Syndrome (SS) is a chronic systemic autoimmune disease of unknown etiology, carrying the highest lymphoma risk among autoimmune diseases, with significant impact on mortality and morbidity of patients.Objectives:To describe: i) the clinical phenotype of SS, ii) the histologic type, stage, treatment options regarding lymphomas and iii) the prognosis of patients with SS related lymphoproliferative disorders.Methods:Eight hundred and fifteen consecutive SS patients’ records from a single center fulfilling the 2016 ACR/EULAR were reviewed retrospectively for the purpose of this study. One hundred twenty-one patients with a diagnosis of non-Hodgkin Lymphoma (NHL) were identified and enrolled in the study population. Cumulative clinical, laboratory and histologic data were recorded and overall survival as well as event free survival curves were constructed using the Kaplan-Meier method. An event was defined as a disease progression, lymphoma relapse, treatment failure, histologic transformation, development of a 2nd lymphoma or death from any cause.Results:From 121 pSS patients with lymphoma the most common histologic type encountered was MALT lymphoma (92/121, 76,0%) followed by DLBCL (11/121, 9.0%) and NMZL (8/119, 6.6%). The remaining 10 patients had various lymphomas of B (follicular, lymphoplasmacytic, chronic lymphocytic leukemia} and T cell origin (peripheral T cell lymphoma not otherwise specified, primary cutaneous T cell lymphoma, angioimmunoblastic t-cell lymphoma). Permanent salivary gland enlargement (66.1%, 80/121), palpable purpura (34,7% 42/121), peripheral nervous involvement (9,9%, 12/121), interstitial lung disease (8,2%, 10/121) presence of serum cryoglobulins (38,7%, 43/111) and C4 hypocomplementemia (69,8% 81/116) present at least 1 year before the development of lymphoma were the main pSS related features. The median age at lymphoma diagnosis was 58 years old (range 29-82) while MALT lymphomas developed earlier compared to DLBCL from pSS diagnosis (8 vs 3 OR= 3.84, 95%CI: 0.29 to 10.46; p=0.0266). The commonest biopsy proven extranodal sites included the labial minor salivary (43,8% patients) and parotid glands (30,5%) while 11% of patients had more than 1 extranodal sites affected. Bone marrow involvement was evident in 24,3% of patients (29/119) while nodal involvement in 35,5% (42/118). The majority of patients (65%) had limited disease (stage I or II). A watch and wait therapeutic policy was chosen in 40 patients while the rest received rituximab with or without chemotherapy. The 10-year survival and event free rates were 79% and 45,5% for MALT lymphomas, 40,9% and 24,2% for DLBCL and 46% and 31% for NMZL respectively (Figure 1). The Mantel-Cox log-rank comparison of the overall survival curves revealed a statistically significant difference (p=0.0016) among lymphoma subtypes.Figure 1.Overall and event free survival of SS-associated lymphoma patients. A. Kaplan-Meier overall survival analysis. B. A Kaplan-Meier event free survival analysis.Conclusion:This is the largest single center series of SS- associated lymphoma patients, providing a detailed description of SS and lymphoma related features, combined with a 10-year survival and event free curves for the first time in the literature.Disclosure of Interests:None declared.


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