Extra-abdominal fibromatosis: Can aggressive management be avoided in a subgroup of patients?

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 10077-10077
Author(s):  
S. Bonvalot ◽  
H. Eldweny ◽  
V. Haddad ◽  
G. Missenard ◽  
D. Vanel ◽  
...  
Keyword(s):  
Systemic Treatment ◽  
Univariate Analysis ◽  
Tumor Evolution ◽  
Surgical Removal ◽  
Tumor Site ◽  
Event Free Survival ◽  
Free Survival ◽  
Quality Of Surgery ◽  
The Impact

10077 Background: The objective was to evaluate the impact of surgery as first-line treatment on event -free survival (EFS) of primary aggressive fibromatosis. Methods: Treatments were categorized into: surgery with or without radiotherapy and non surgical strategies with systemic treatment alone or wait and see policy. Event-free survival curves were estimated using the Kaplan-Meier method. Results: The sex ratio was 39 males/73 females. The median age at the time of the first diagnosis was 30 years. The median size of the primary was 60 mm. Eighty-nine patients (79.5%) had initial resection of their primary tumor followed by postoperative radiotherapy in 13 cases. Twenty-three patients (20.5%) did not undergo surgery but received systemic treatment or watch and wait policy. Median follow-up was 76 months. Three years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to that of patients in the non-surgery group (65% vs 68% for 3-yr EFS respectively). Gender, age, tumor size, treatment period and strategy (surgery versus no surgery) were not statistically significant. Quality of resection according to margins and the tumor site were the only prognostic factors. There was a significant correlation between the tumor site and the quality of surgery (p= 0.0002). Tumor site was the only prognostic factor that remained in the multivariate analysis. Conclusions: Surgical removal as initial treatment was not found to influence the outcome. A subset of patients could be managed with a non aggressive policy. When surgery is finally necessary, it should be performed with the aim of achieving negative margins. Predictive biological factors influencing tumor evolution have to be defined No significant financial relationships to disclose.

Growth Differentation Factor 15 Is a Survival Factor for multiple myeloma cells and its plasma Concentration In Patients Is Related to Reduced Survival

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 614-614
Author(s):  
Jill Corre ◽  
Elodie Labat ◽  
Nicolas Espagnolle ◽  
Hervé Avet-Loiseau ◽  
Murielle Roussel ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Concentration ◽  
Cell Line ◽  
Healthy Subjects ◽  
Univariate Analysis ◽  
Culture Conditions ◽  
Newly Diagnosed ◽  
Event Free Survival ◽  
Free Survival ◽  
The Impact

Abstract Abstract 614 We previously reported that bone marrow mesenchymal stem cells (BMMSCs) from newly diagnosed multiple myeloma (MM) patients were abnormal. In particular, we showed that Growth Differentiation 15 (GDF15) expression was higher in MM BMMSCs than in normal BMMSCs. GDF15 is a divergent member of the human TGFβ superfamily. GDF15 overexpression has been described in numerous malignancies and its concentration is increased in the serum of patients with glioma, prostate, colorectal or pancreatic cancers. Contradictory data prevent to understand clearly GDF15 implication in pathophysiology of tumors. Furthermore, GDF15 has never been studied in haematological malignancies. Our first objective was thus to determine the effect of human recombinant GDF15 (rGDF15) on MOLP-6 stroma-dependent cell line and MM1.S stroma-independent cell line under serum free culture conditions. rGDF15 could significantly increase cell survival in MOLP-6 but not in MM1.S. Interestingly, rGDF15 was able to induce Akt phosphorylation on Threonine 308 in MOLP-6 and primary MM cells but not in MM1.S cells. Furthermore, pre-treatment of MOLP-6 with Akt pharmacologic inhibitor abrogated the prosurvival effect of GDF15, suggesting an Akt-dependent mechanism. In the same culture conditions, we observed that rGDF15 could abrogate toxicity of drugs classically used in MM treatment (melphalan, bortezomid and lenalidomide) for both cell lines MOLP-6 and MM1.S, suggesting that this cytoprotective effect may be Akt-independent. Because of the in vitro effects of GDF15, our second objective was to determine whether the plasma concentration of GDF15 (pGDF15) in patients with MM may be indicative of the seriousness of the disease or correlate with the response to the treatment. Thus, we investigated the pGDF15 in 131 patients with newly diagnosed MM and 13 healthy subjects. We first found that it was significantly higher for patients with MM (0.90±1.10 ng/mL) than for healthy subjects (0.25±0.08 ng/mL) (P< .001). In patients with MM, pGDF15 correlated with the main prognostic factor of the disease (i.e. International Staging System, b2 microglobulin level, presence or absence of deletion of chromosome 13, and bone status). For the 81 patients with high pGDF15 level (≥ 0.50 ng/mL), the probabilities of event-free and overall survival 30 months after diagnosis were 50% and 75%. For the 50 patients with low pGDF15 level (< 0.5 ng/mL), the probabilities were 80% and 97% (P< .0045 and P< .013, respectively). However, we did not find a clear relationship between pGDF15 and response to treatment. We analysed the impact of prognostic factors on event-free survival for the 131 patients with MM. On univariate analysis, event-free survival was significantly related to age (P= .003), b2-microglobulin level (P= .02) and pGDF-15 level (P= .003). On multivariate analysis, event-free survival was significantly related to age (P= .001) and pGDF15 level (P= .04). Our study demonstrates that GDF15 is a survival and cytoprotective factor for MM cells and that pGDF15 is related to initial parameters of the disease and survival, which specifically implicates the MM microenvironment in the pathophysiology and the prognosis of the disease. Disclosures: No relevant conflicts of interest to declare.

Quantification of VEGF Isoforms and VEGFR Transcripts by qRT-PCR and Their Significance in Acute Myeloid Leukemia

2009 ◽  
Vol 24 (1) ◽  
pp. 22-31 ◽  
Author(s):  
Samia Mourah ◽  
Raphaël Porcher ◽  
Géraldine Lescaille ◽  
Philippe Rousselot ◽  
Marie-Pierre Podgorniak ◽  
...  
Keyword(s):  
Overall Survival ◽  
Acute Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Univariate Analysis ◽  
Prognostic Impact ◽  
Mrna Levels ◽  
Event Free Survival ◽  
Free Survival ◽  
The Impact ◽  
Acute Myeloid

Vascular endothelial growth factor (VEGF) and its receptors are known to play an important role in normal and pathological hematopoiesis but the prognostic impact of VEGF isoform transcripts in acute myeloid leukemia (AML) has not been addressed. We conducted a single-institution prospective study to analyze the impact of these angiogenic factors and the expression of their receptors on the survival of adult patients newly diagnosed with AML. We investigated the levels of VEGF transcript isoforms VEGF121, -145, -165, -189 and -206 and their receptors, VEGFR-1 and VEGFR-2, using quantitative reverse transcriptase polymerase chain reaction assays in peripheral blood mononuclear cells (PBMCs) of 67 consecutive AML patients at diagnosis. VEGF total protein was measured for comparison with mRNA levels in PBMCs. The VEGF121 splice variant transcript in AML PBMCs was significantly higher than in the normal controls. VEGF transcripts were quantified in all samples while its protein was detected in 42/67 (63%) of AML samples. High levels of VEGF121, VEGF165 transcripts and VEGF protein in AML were significantly related to a worse prognosis when analyzing overall survival (p<0.0001, p=0.019 and p=0.012, respectively) or event-free survival (p<0.0001, p=0.010 and p=0.047) using univariate analysis. In multivariable analysis only VEGF121 expression remained an independent prognostic factor for either event-free survival or overall survival [aHR=8.83 (3.48–22.4), p<0.0001, and aHR=9.52 (3.41–26.6), p<0.0001]. No prognostic value was observed for the other isoforms and the two receptors. Our findings show that the level of VEGF121 mRNA in circulating cells from AML patients is a strong independent prognostic parameter, which could be useful in the management of unselected AML patients.

Systematic literature review of clinical outcomes in adults with metastatic or advanced synovial sarcoma

2020 ◽  
Vol 16 (35) ◽  
pp. 2997-3013
Author(s):  
Kentaro Kogushi ◽  
Michael LoPresti ◽  
Shunya Ikeda
Keyword(s):  
Synovial Sarcoma ◽  
High Frequency ◽  
Clinical Evidence ◽  
Systemic Treatment ◽  
Progression Free Survival ◽  
Treatment Modalities ◽  
Free Survival ◽  
New Treatment ◽  
Poor Outcomes

Background: Synovial sarcoma (SS) is a rare, aggressive soft tissue sarcoma with a poor prognosis after metastasis. The objective of this study was to conduct a systematic review of the clinical evidence for therapeutic options for adults with metastatic or advanced SS. Materials & methods: Relevant databases were searched with predefined keywords. Results: Thirty-nine publications reported clinical data for systemic treatment and other interventions. Data on survival outcomes varied but were generally poor (progression-free survival: 1.0–7.7 months; overall survival: 6.7–29.2 months) for adults with metastatic and advanced SS. A high frequency of neutropenia with systemic treatment and low quality of life post-progression were reported. Conclusion: Reported evidence suggests poor outcomes in adults with metastatic and advanced SS and the need for the development of new treatment modalities.

scholarly journals Chemotherapy Delays Are Associated with Inferior Outcome in Acute Lymphoblastic Leukemia: A Retrospective Study from a Tertiary Cancer Center in South India

2021 ◽  
Vol 42 (01) ◽  
pp. 051-060
Author(s):  
Vineet Agrawal ◽  
Smita Kayal ◽  
Prasanth Ganesan ◽  
Biswajit Dubashi
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
Lymphoblastic Leukemia ◽  
Univariate Analysis ◽  
Cancer Center ◽  
Survival Outcomes ◽  
Separate Analysis ◽  
Term Survival ◽  
Free Survival ◽  
Intensive Phase ◽  
The Impact

Abstract Background Treatment protocols for acute lymphoblastic leukemia (ALL) have evolved over time to give excellent cure rates in children and moderate outcomes in adults; however, little is known how delays in chemotherapy affect long-term survival. Objectives To find the association of delays during different treatment phases on the survival outcomes. Materials and Methods Data from 149 ALL cases treated between 2009 and 2015 were retrospectively analyzed. Treatment course in commonly used protocols was divided into three phases—induction, consolidation (postremission), maintenance, and also a combined intensive phase (induction plus consolidation) for the purpose of analysis, and delay in each phase was defined based on clinically acceptable breaks. Analysis was done to find the impact of treatment delay in each phase on the survival outcomes. Results The median age was 12 years (range, 1–57). Multi-center Protocol-841 (MCP-841) was used for 72%, German Multicenter Study Group for Adult ALL (GMALL) for 19%, and Berlin, Frankfurt, Muenster, 95 protocol (BFM-95) for 9% of patients. Delay in induction was seen in 52%, consolidation in 66%, and during maintenance in 42% of patients. The median follow-up was 41 months, and 3-year survival outcomes for the entire cohort were event-free survival (EFS)—60%, relapse-free survival (RFS)—72%, and overall survival (OS)—68%. On univariate analysis, delay in induction adversely affected EFS (hazard ratio [HR] = 1.78, p = 0.04), while delay in intensive phase had significantly worse EFS and RFS (HR = 2.41 [p = 0.03] and HR = 2.57 [p = 0.03], respectively). On separate analysis of MCP-841 cohort, delay in intensive phase affected both EFS (HR = 3.85, p = 0.02) and RFS (HR = 3.42, p = 0.04), whereas delay in consolidation significantly affected OS with (HR = 4.74, p = 0.04) independently. Conclusion Treatment delays mostly in intensive phase are associated with worse survival in ALL; attempts should be made to maintain protocol-defined treatment intensity while adequately managing toxicities.

scholarly journals The impact of bronchial artery embolisation on the quality of life of patients with haemoptysis: a prospective observational study

2021 ◽  
Author(s):  
Naoki Omachi ◽  
Hideo Ishikawa ◽  
Masahiko Hara ◽  
Takashi Nishihara ◽  
Yu Yamaguchi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Survival Rate ◽  
Bronchial Artery ◽  
Recurrence Free Survival ◽  
Free Survival ◽  
Kaplan Meier ◽  
Procedural Success ◽  
Bronchial Artery Embolisation ◽  
The Impact

Abstract Objectives Patients with haemoptysis often experience daily physical and mental impairment. Bronchial artery embolisation is among the first-line treatment options used worldwide; however, no evidence exists regarding the health-related quality of life (HRQoL) after bronchial artery embolisation. Therefore, this study aimed to evaluate the effects of bronchial artery embolisation on the HRQoL of patients with haemoptysis. Methods We prospectively enrolled 61 consecutive patients who visited our hospital from July 2017 to August 2018 and received bronchial artery embolisation for haemoptysis. The primary outcome was the HRQoL evaluated using the Short Form Health Survey, which contains physical and mental components, before and after bronchial artery embolisation. The secondary outcomes were procedural success, complications, and recurrence-free survival rate at 6 months. Results The mean age of the patients was 69 years (range, 31–87 years). The procedural success rate was 98%. No major complications occurred. The recurrence-free survival rate estimated using the Kaplan-Meier analysis at 6 months after bronchial artery embolisation was 91.8% (95% confidence interval, 91.1–92.5%). Compared with the pre-treatment scores, the physical and mental scores were significantly improved at 6 months after bronchial artery embolisation (p < 0.05). Conclusion Bronchial artery embolisation improved the HRQoL of patients with haemoptysis. Key Points • Bronchial artery embolisation improved the HRQoL of patients with haemoptysis. • Vessel dilation on computed tomography and systemic artery-pulmonary artery direct shunting on angiography were the most common abnormalities. • The recurrence-free survival rate estimated using the Kaplan-Meier analysis at 6 months after bronchial artery embolisation was 91.8%.

Malignant Sacrococcygeal Germ Cell Tumors in Children: A 30-year Experience from a Single Institution

2013 ◽  
Vol 99 (1) ◽  
pp. 51-56 ◽  
Author(s):  
Münevver Büyükpamukcu ◽  
Ali Varan ◽  
Serhan Küpeli ◽  
Saniye Ekinci ◽  
Sule Yalcin ◽  
...  
Keyword(s):  
Germ Cell ◽  
Univariate Analysis ◽  
Survival Rates ◽  
Germ Cell Tumors ◽  
Patient Characteristics ◽  
Tumor Stage ◽  
Event Free Survival ◽  
Treatment Results ◽  
Free Survival ◽  
Chemotherapy Protocol

Background Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors. Procedure Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded. Results A total of 58 patients (M/F = 20/38) with malignant sacrococcygeal germ cell tumor was included in analysis. With a mean follow-up of 156 months (range, 26 days to 288.8 months) overall and event-free survival rates of the 58 patients were 50.9% and 43.8%, respectively. AFP status of the patients (37% in patients with <10,000 ng/ml, 68.9% in patients with ≥10,000 ng/ml), type of resection (total vs others), coccygeal resection, chemotherapy protocol (PEB vs others) and number of chemotherapy courses had an impact on event-free survival in univariate analysis. In multivariate analysis, AFP status had the greatest effect on prognosis. Conclusions Our treatment results are worse than those reported in the literature. Elevated AFP level at the time of diagnosis had a beneficial effect on prognosis, but year of diagnosis, tumor stage, presence of metastasis, tumor size and histopathological subtype had no impact on survival in patients with malignant sacrococcygeal germ cell tumors.

scholarly journals Primary and Metastatic Intracranial Ewing Sarcoma at Diagnosis: Retrospective International Study and Systematic Review

Cancers ◽  
2020 ◽  
Vol 12 (6) ◽  
pp. 1675
Author(s):  
Lianne M. Haveman ◽  
Andreas Ranft ◽  
Henk van den Berg ◽  
Stephanie Klco-Brosius ◽  
Ruth Ladenstein ◽  
...  
Keyword(s):  
Local Control ◽  
Ewing Sarcoma ◽  
Univariate Analysis ◽  
International Study ◽  
Cerebral Metastases ◽  
The Body ◽  
Event Free Survival ◽  
Free Survival ◽  
Metastatic Lesions ◽  
Chemotherapeutic Treatment

Intracranial Ewing sarcoma (EwS) is rare and publications on primary or metastatic intracranial EwS are minimal. The aim of this study was to describe incidence, clinical behavior, treatment, and factors associated with outcome in patients with primary intracranial EwS or patients with a primary extracranial EwS and cerebral metastases at diagnosis. We reviewed all patients with primary or with metastatic intracranial EwS at diagnosis registered in the International Clinical Trial Euro-E.W.I.N.G.99 (EE99). In total, 17 of 1435 patients (1.2%) presented with primary intracranial EwS; 3 of them had metastatic disease. Four patients (0.3%) with primary extracranial EwS presented with intracranial metastatic lesions. The 3-year event-free survival (EFS) was 64% and overall survival (OS) was 70% in patients with a primary intracranial EwS. Local control in patients with primary intracranial EwS consisted of surgery (6%), radiotherapy (RT) (18%), or both modalities (76%). Univariate analysis showed that patients < 15 years of age had significantly better outcome (EFS: 72%; OS: 76%) compared to those aged above 15 years (EFS: 13%; OS: 25%). In conclusion, primary intracranial EwS and extracranial EwS with cerebral metastases at diagnosis is rare, yet survival is comparable with local and metastatic EwS elsewhere in the body. Age and stage of disease are important prognostic factors. Besides chemotherapeutic treatment, local control with surgical resection combined with RT is recommended whenever feasible.

Multi-modality management of extraosseous Ewing sarcoma: 10-year experience from a tertiary care centre

2020 ◽  
pp. 1-7
Author(s):  
Arvind Sathyamurthy ◽  
Ashish Singh ◽  
Tarun Jose ◽  
Patricia Sebastian ◽  
Rajesh Balakrishnan ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Univariate Analysis ◽  
Tertiary Care ◽  
Disease Free Survival ◽  
Molecular Techniques ◽  
Adjuvant Radiation ◽  
Free Survival ◽  
The Impact ◽  
Extraosseous Ewing Sarcoma ◽  
Disease Free

Abstract Aim: To analyse the presentation, diagnosis and patterns of care of extraosseous Ewing sarcoma treated at our institution between 2008 and 2018. Methods: Electronic medical records of extraosseous Ewing sarcoma patients treated at our institution between January 2008 and April 2018 were reviewed. Kaplan–Meier curves were plotted to assess the overall and disease-free survival with 95% confidence intervals. A univariate analysis was carried out to assess the impact of variables such as surgical excision, completeness of surgery, completeness of chemotherapy and addition of radiation therapy on the survivorship. Results: The records of 65 patients treated at our institution were available for review. The mean age was 26·4 years. The most frequent sites of extraosseous Ewing tumour were kidney—9/65 (13·8%) and brain—10/65 (15·4%). Sixteen (24·6%) patients presented with inoperable/metastatic disease at diagnosis. The other 49 (75·4%) had localised disease at presentation. The median overall survival of the 49 non-metastatic patients was 46 months, and the disease-free survival was 45 months. Conclusion: Extraosseous Ewing sarcoma is a rare and aggressive tumour diagnosed by molecular techniques. Multi-modality treatment including surgical resection with wide margins, adjuvant radiation when indicated and completion of systemic chemotherapy results in optimum outcomes.

Whole lung irradiation for completely responding pulmonary metastases in pediatric Ewing sarcoma

2020 ◽  
Vol 16 (15) ◽  
pp. 1043-1051
Author(s):  
Hagar Elghazawy ◽  
Azza Nasr ◽  
Iman Zaky ◽  
Manal Zamzam ◽  
Ahmed Elgammal ◽  
...  
Keyword(s):  
Pulmonary Metastases ◽  
Pediatric Population ◽  
Event Free Survival ◽  
Relapse Free Survival ◽  
Free Survival ◽  
Real Value ◽  
Lung Irradiation ◽  
Whole Lung Irradiation ◽  
The Impact

Aim: Scarce data assessing the real value of whole lung irradiation (WLI) in Ewing’s sarcoma (ES) with lung-only metastasis, with published conflicting results. We studied the impact of WLI in a homogenous pediatric population. Materials & methods: Retrospective study evaluating the survival outcomes of WLI in these patients. Results: Out of 163 metastatic ES; 41 patients were eligible for WLI. 30 patients (73.1%) received WLI (+ve) while 11 patients (26.8%) did not receive WLI (-ve). Five-year event-free survival was statistically significant in WLI (+ve). Five-year pulmonary relapse-free survival showed trend for improvement with WLI (+ve), while 5-year overall survival was not statistically significant between the two arms. Conclusion: WLI added significantly to the long term clinical outcome of metastatic ES patients, with no irreversible toxicity.

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