Cumulative survival analysis of patients with spinal myxopapillary ependymomas in the first 2 decades of life

2014 ◽  
Vol 13 (4) ◽  
pp. 400-407 ◽  
Author(s):  
Sunil Kukreja ◽  
Sudheer Ambekar ◽  
Anthony Hunkyun Sin ◽  
Anil Nanda

Object Reports of myxopapillary ependymomas (MPEs) of the spinal cord in pediatric patients are scarce. In the literature, various authors have shared their experiences with small groups of patients, which makes it difficult to create a consensus regarding the treatment approach for spinal MPEs in young patients. The aim of this study was to perform a survival analysis of patients in the first 2 decades of life whose cases were selected from the published studies, and to examine the influence of various factors on outcomes. Methods A comprehensive search of studies published in English was performed on PubMed. Patients whose age was ≤ 20 years were included for integrative analysis. Information about age, treatment characteristics, critical events (progression, recurrence, and death), time to critical events, and follow-up duration was recorded. The degree of association of the various factors with the survival outcome was calculated by using Kaplan-Meier estimator and Cox proportional hazard model techniques. Results A total of 95 patients were included in the analysis. The overall rate of recurrence (RR) was 34.7% (n = 33), with a median time to recurrence of 36 months (range 2–100 months). Progression-free survival (PFS) and overall survival rates at 5 years were 73.7% and 98.9%, respectively. Addition of radiotherapy (RT) following resection significantly improved PFS (log-rank test, p = 0.008). In patients who underwent subtotal resection (STR), administering RT (STR + RT) improved outcome with the lowest failure rates (10.3%), superior to patients who underwent gross-total resection (GTR) alone (RR 43.1%; log-rank test, p < 0.001). Addition of RT to patients who underwent GTR was not beneficial (log-rank test, p = 0.628). In patients who had disseminated tumor at presentation, adjuvant RT controlled the disease effectively. High-dose RT (≥ 50 Gy) did not change PFS (log-rank test, p = 0.710). Conclusions Routine inclusion of RT in the treatment protocol for spinal MPEs in young patients should be considered. Complete resection is always the goal of tumor resection. However, when complete resection does not seem to be possible in complex lesions, RT should be used as an adjunct to avoid aggressive resection and to minimize inadvertent injury to the surrounding neural tissues. High-dose RT (≥ 50 Gy) did not provide additional survival benefits, although this association needs to be evaluated by prospective studies.

2010 ◽  
Vol 28 (4) ◽  
pp. E5 ◽  
Author(s):  
Isaac Yang ◽  
Michael E. Sughrue ◽  
Martin J. Rutkowski ◽  
Rajwant Kaur ◽  
Michael E. Ivan ◽  
...  

Object Craniopharyngiomas have a propensity to recur after resection, potentially causing death through their aggressive local behavior in their critical site of origin. Recent data suggest that subtotal resection (STR) followed by adjuvant radiotherapy (XRT) may be an appealing substitute for gross-total resection (GTR), providing similar rates of tumor control without the morbidity associated with aggressive resection. Here, the authors summarize the published literature regarding rates of tumor control with various treatment modalities for craniopharyngiomas. Methods The authors performed a comprehensive search of the English language literature to identify studies publishing outcome data on patients undergoing surgery for craniopharyngioma. Rates of progression-free survival (PFS) and overall survival (OS) were determined through Kaplan-Meier analysis. Results There were 442 patients who underwent tumor resection. Among these patients, GTR was achieved in 256 cases (58%), STR in 101 cases (23%), and STR+XRT in 85 cases (19%). The 2- and 5-year PFS rates for the GTR group versus the STR+XRT group were 88 versus 91%, and 67 versus 69%, respectively. The 5- and 10-year OS rates for the GTR group versus the STR+XRT group were 98 versus 99%, and 98 versus 95%, respectively. There was no significant difference in PFS (log-rank test) or OS with GTR (log-rank test). Conclusions Given the relative rarity of craniopharyngioma, this study provides estimates of outcome for a variety of treatment combinations, as not all treatments are an option for all patients with these tumors.


2017 ◽  
Vol 19 (2) ◽  
pp. 239-248 ◽  
Author(s):  
Subhas K. Konar ◽  
Shyamal C. Bir ◽  
Tanmoy K. Maiti ◽  
Anil Nanda

OBJECTIVE The incidence of primary spinal cord glioblastoma multiforme (GBM) in the pediatric age group is very rare. Only a few case series and case reports have been published in the literature; therefore, overall survival (OS) outcome and the as-yet poorly defined management options are not discussed in detail. The authors performed a cumulative survival analysis of all reported cases of pediatric spinal cord GBM to identify the predictive factors related to final survival outcome. METHODS A comprehensive search for relevant articles was performed on PubMed's electronic database MEDLINE for the period from 1950 to 2015 using the search words “malignant spinal cord tumor” and “spinal glioblastoma multiforme.” This study was limited to patients younger than 18 years of age. Survival rates for children with various tumor locations and treatments were collected from the published articles and analyzed. RESULTS After an extensive literature search, 29 articles met the study inclusion criteria. From the detailed information in these articles, the authors found 53 children eligible for the survival analysis. The majority (45%) of the children were more than 12 years old. Thirty-four percent of the cases were between 7 and 12 years of age, and 21% were younger than 7 years. In the Kaplan-Meier survival analysis, children younger than 7 years of age had better survival (13 months) than the children older than 7 years (7–12 years: 10 months, > 12 years: 9 months; p = 0.01, log-rank test). Fifty-five percent of the children were female and 45% were male. A cervical tumor location (32%) was the most common, followed by thoracic (28.3%). Cervicothoracic (18.9%) and conus (18.8%) tumor locations shared the same percentage of cases. Cervical tumors had a worse outcome than tumors in other locations (p = 0.003, log-rank test). The most common presenting symptom was limb weakness (53%), followed by sensory disturbances (25%). Median OS was 10 months. The addition of adjuvant therapy (radiotherapy [RT] and/or chemotherapy [CT]) after surgery significantly improved OS (p = 0.01, log-rank test). Children who underwent gross-total resection and RT had better outcomes than those who underwent subtotal resection and RT (p = 0.04, log-rank test). Cerebrospinal fluid spread, hydrocephalus, brain metastasis, and spinal metastasis were not correlated with OS in primary spinal GBM. CONCLUSIONS Adjuvant therapy after surgery had a beneficial effect on overall outcome of spinal GBM in the pediatric age group. Gross-total resection followed by RT produced a better outcome than subtotal resection with RT. Further large-scale prospective study is required to establish the genetic and molecular factors related to OS in primary GBM of the spinal cord in pediatric patients.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 417-417
Author(s):  
Stefan O Schonland ◽  
Ute Hegenbart ◽  
Simona Iacobelli ◽  
Jennifer Hoek ◽  
M Rovira ◽  
...  

Abstract Introduction High-dose chemotherapy and autologous stem cell transplantation (ASCT) is a treatment option for eligible patients with systemic light chain (AL) amyloidosis. Compared to patients with multiple myeloma (MM), the risk for complications and transplant-related mortality is increased. However, in this fragile patient group it is often not possible to distinguish between treatment- and amyloidosis-related deaths in the post-transplant period. The CIBMTR reported a one year survival (1-yr OS) of 66% of patients transplanted between 1995 and 2001. Another multicenter analysis from Great Britain reported a one year survival of 75% (Goodman et al., BJH, 2006); interestingly, they could show a significant reduction of day 100 all-cause mortality from 32% to 13% after 1998. In recent single center studies 1-yr OS was better ranging from 80% to 90% (reviewed by Schönland et al., BMT, 2011). The amyloidosis groups of Mayo Clinic and Boston Medical School could also show a survival improvement over time (Tsai et al., Blood, 2012 and Gertz et al., BMT, 2010). Specific Aim The aim of this retrospective study was to analyze the 1-yr OS after ASCT for patients with AL amyloidosis in Europe. Of special interest were calendar year of transplants and center experience. Methodology Patient-, disease-, and transplant-related variables were collected according to the data entries in the EBMT database. Inclusion criteria were as follows: first autologous transplant with peripheral blood stem cells performed between 1997 and 2010. Center experience was measured for each patient by the number of previous MM ASCT done in the center until the year of AL transplant. Results 1315 patients from 259 centers fulfilled the entry criteria and were included in the analysis (for patient characteristics see table). The conditioning regimen was high-dose melphalan in most cases. Median follow up was 47 months. 1-yr OS after ASCT was 80.7% (CI 78.5 – 82.9). In univariate analysis age, gender, time from diagnosis to ASCT had no influence on 1-yr OS. Bad performance status (57% (50-65) vs. 90% (87-92); p<0.001) and progression/relapse as status at conditioning (61% (53-69) vs. 85% (83-87); p<0.001) significantly reduced 1-yr OS. A strong and significant influence of the transplant period (see figure 1, log-rank test, p<0.001) and higher center experience (see figure 2; log-rank test, p<0.001) could also be demonstrated. Interestingly, the proportion of patients with bad performance status decreased from 28% to 13% in most recent years (p=0.001). These results hold in multivariate analysis. Bad performance status (HR 4.3; p<0.001), progression/relapse as status at conditioning (HR 1.96; p<0.001) and earlier transplant period (HR 1.1; p<0.001) retained their highly significant negative influence on 1-yr OS. In an alternative multivariate model replacing transplant period with center experience, the latter has also a beneficial effect (HR 0.99 for 10 additional previous MM transplants; p=0.015) and all other prognostic factors retained the estimated effects. Conclusion This is the first report from the EBMT about the results of ASCT in AL amyloidosis from 259 European centers and the largest retrospective analysis for this rare entity. It clearly shows that short term survival has been improved over time probably due to better patient selection and increase of center experience. Of note, in the most recent cohort (2009 to 2010) the 1-yr OS was 91% (CI 87-96) supporting the further use of ASCT in eligible AL amyloidosis patients. Disclosures: Leblond: Roche : Consultancy, Honoraria, Membership on an entity’s Board of Directors or advisory committees, Speakers Bureau; Janssen: Honoraria, Membership on an entity’s Board of Directors or advisory committees; Mundipharma: Honoraria, Membership on an entity’s Board of Directors or advisory committees, Speakers Bureau.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 8075-8075
Author(s):  
H. Yiu ◽  
R. K. Ngan ◽  
H. Cheng ◽  
K. Wong ◽  
M. Cheung ◽  
...  

8075 Background: Nasal NK/T lymphoma is uncommon but prognosis is universally poor even in early stage disease. Chemotherapy is often added to radiation for improving outcome although its role has not been well defined. Methods: Since 1998, an intensive protocol has been introduced for pts with stage I or II disease, which consists of 3 cycles of anthracycline-based (CHOP or ProMACE-CytaBOM) chemotherapy to be followed by cCRT (50–54Gy radiotherapy with weekly cisplatin at 40mg/m2) for pts with adequate renal function, and then consolidation HDCT. Cyclophosphamide, BCNU & Etoposide HDCT was offered to pts <60 years with adequate organ functions. Otherwise, 3 more cycles of conventional chemotherapy were given. Results: Out of the 27 pts recruited into the intensive protocol up till 2005, 23 completed cCRT & 14 received HDCT. Six pts had failed either locally or systemically, among whom 2 local failures were salvaged by further local radiotherapy. Eight patients had died with 4 related to lymphoma progression, 3 due to treatment complications and 1 died of prevailing medical illness. A preceding cohort of 35 pts intended to be treated with combined sequential conventional chemotherapy and radiotherapy only (sCRT: n=35) from 1986 to 1997 was also analyzed and compared with the current cohort (cCRT: n=27). Pts in both groups had similar characteristics. The type of intended treatment was a statistically significant factor for 6-year failure-free survival (sCRT 22.9%, cCRT 65.9%; p=0.0049, log-rank test) and overall survival (sCRT 36.4%, cCRT 69.8%; p=0.05, log-rank test), so are B symptoms (present Vs absent) and stage (I Vs II). Conclusions: The intensive protocol with sequential brief induction conventional chemotherapy, cCRT, and consolidation HDCT / conventional chemotherapy in appropriate pts was feasible, leading to better outcome than the combination of sequential conventional chemotherapy and radiation alone. The relative contributions of cCRT and HDCT to the improved outcome should be further explored. No significant financial relationships to disclose.


2021 ◽  
Vol 11 ◽  
Author(s):  
Silin Chen ◽  
Ning Li ◽  
Yuan Tang ◽  
Bo Chen ◽  
Hui Fang ◽  
...  

PurposeTo create a prognostic prediction radiomics model for soft tissue sarcoma (STS) of the extremities and trunk treated with neoadjuvant radiotherapy.MethodsThis study included 62 patients with STS of the extremities and trunk who underwent magnetic resonance imaging (MRI) before neoadjuvant radiotherapy. After tumour segmentation and preprocessing, 851 radiomics features were extracted. The radiomics score was constructed according to the least absolute shrinkage and selection operator (LASSO) method. Survival analysis (disease-free survival; DFS) was performed using the log-rank test and Cox’s proportional hazards regression model. The nomogram model was established based on the log-rank test and Cox regression model. Harrell’s concordance index (C-index), calibration curve and receiver operating characteristic (ROC) curve analysis were used to evaluate the prognostic factors. The clinical utility of the model was assessed by decision curve analysis (DCA).ResultsThe univariate survival analysis showed that tumour location (p = 0.032), clinical stage (p = 0.022), tumour size (p = 0.005) and the radiomics score were correlated with DFS (p &lt; 0.05). The multivariate analysis showed that tumour location, tumour size, and the radiomics score were independent prognostic factors for DFS (p &lt; 0.05). The combined clinical-radiomics model based on the multivariate analysis showed the best predictive ability for DFS (C-index: 0.781; Area Under Curve: 0.791). DCA revealed that the use of the radiomics score-based nomogram was associated with better benefit gains relative to the prediction of 2-year DFS events than other models in the threshold probability range between 0.12 and 0.38.ConclusionThe radiomics score from pretreatment MRI is an independent prognostic factor for DFS in patients with STS of the extremities and trunk. The radiomics score-based nomogram could improve prognostic stratification ability and thus contribute to individualized therapy for STS patients.


2020 ◽  
Author(s):  
Xingchen Li ◽  
Xinyu Bi ◽  
Jianjun Zhao ◽  
Zhiyu Li ◽  
Jianguo Zhou ◽  
...  

Abstract Background Only few studies have been evaluated the clinical characteristics and prognosis of hepatocellular carcinoma in young patients. The purpose of this study is to identify prognostic factors and develop an efficient and practical nomogram to predict cancer-specific survival in young patients with hepatocellular carcinoma.Methods Four-hundred-and-forty-one young patients with hepatocellular carcinoma who had undergone surgery from 2004-2015 were selected from the Surveillance, Epidemiology, and End Results database. The competing risk model, Lasso and Cox regression were used to screen prognostic factors for cancer-specific survival, and a prognostic nomogram was established using these factors. Thirty-nine young patients with hepatocellular carcinoma from the National Cancer Center, Cancer Hospital, Chinese Academy of Medical Science were used to validate our model. To further evaluate the predictive performance of our model, the concordance index was calculated and the calibration curves were drawn. The clinical usefulness was evaluated by decision curve analysis(DCA). Finally, all patients were grouped by our nomogram. The survival of different risk groups was analyzed using the Kaplan-Meier method, and the differences among survival curves were compared by the log-rank test.Results The median survival times of the Surveillance, Epidemiology, and End Results training group and the external National Cancer Center validation group were 41 and 52 months, respectively. Histological grade, tumor size, Alpha-fetoprotein, T stage, and M stage were selected as independent factors for cancer-specific survival, and a prognostic nomogram was established. The concordance indices of the training and external validation groups were 0.76 (95% CI, 0.72 to 0.80) and 0.92 (se=0.085), respectively. The calibration plots showed good agreement. Decision curve analysis revealed that our nomogram resulted in a better clinical net benefit than the AJCC 7th edition and Barcelona Clinic Liver Cancer staging systems. Patients were divided into two risk groups according to the cut-off value of 125 of the total points from our nomogram. Kaplan-Meier plots for cancer-specific survival were performed using the log-rank test, the p-value of which was <0.001.Conclusions The practical nomogram resulted in a more-accurate prognostic prediction for young hepatocellular carcinoma patients after curative liver resection.


Author(s):  
Janet L. Peacock ◽  
Sally M. Kerry

Chapter 11 covers survival analysis, and includes Kaplan–Meier estimates, the log rank test, Cox regression, and further reading.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 923-923
Author(s):  
David Cary Seldin ◽  
Jennifer J. Anderson ◽  
Martha Skinner ◽  
Karim Malek ◽  
Daniel G. Wright ◽  
...  

Abstract Over the past 10 years, HDM/SCT has been employed for treatment of patients with AL amyloidosis. Although peri-transplant mortality is greater than for other hematologic diseases, HDM/SCT leads to durable hematologic complete responses (CR), improvements in organ function, and extended survival in a substantial proportion of patients (Skinner et al., Ann. Int. Med. 2004). In that study, the proportion of patients eligible for HDM/SCT was lower for patients >65 years of age than for younger patients (34.3% vs. 68.4%), but the hematologic complete response (CR) rate and survival appeared to be no different. Here, we update these results and present a detailed analysis of outcomes in patients >65 vs. those <65. Data were collected with IRB approval. Through 07/01/04, 349 patients began G-CSF mobilization, of which 66 (19%) were >65. The median age of the 66 patients was 68, range 65–80; 73% male; 18% ? and 82% ?, while for the 283 patients <65, the median age was 55, range 29–64; 57% male; 16% ? and 84% ?. The median number of involved organs was no different in the two groups, nor was the type of organ involvement or performance status. There was also no difference in the rate of serious complications during stem cell collection, as a similar fraction of patients in both groups did not proceed to transplant, 12.1% of patients >65 versus 10.3% of patients <65 (p=.659). Based on treatment protocol, only 19% of patients >65 received an IV dose of melphalan of 200 mg/m2 (the remainder being treated with 140 mg/m2 or less), vs. 64% of those <65 (p <.001). There was no difference in early transplant-related mortality (10.3% in patients >65 vs. 13.4% in patients <65, p=.665). There was also no difference in one year mortality (22.4% for those >65 vs. 20.9% for those <65, p=.859). There was a non-significant trend towards a lower rate of hematologic CR in the older patients (32% vs. 44%, p=0.155). However, the median survival after HDM/SCT was no different (see fig; 4.9 years for patients >65 vs. 4.6 years for those <65, p=0.42 by log-rank test). Thus, while fewer patients >65 meet eligibility requirements for HDM/SCT, for those who do, treatment-related mortality, hematologic CR rate, and survival are no different than for younger patients. These data strongly support the use of HDM/SCT for suitable patients with AL amyloidosis who are >65 years old. Figure. Figure.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2472-2472
Author(s):  
Asim F. Belgaumi ◽  
Amani A. Al-Kofide ◽  
Nicey Joseph ◽  
Yasser Khafaga ◽  
Rubina J. Malik ◽  
...  

Abstract Hodgkin Lymphoma (HL) is rarely seen in <5-year olds in developed nations. Even in developing countries, where a tendency towards younger age of presentation has been shown, this represents a minority of cases. Little is known about the biology and behavior of these very young patients with HL as compared to older children. Methods: We retrospectively reviewed HL cases diagnosed and treated at our institution between 1975 and 2003. HL was diagnosed histopathologically and staged clinically. The pediatric age group ranged from 0–14 years. Treatment strategy for these very young children was focused on the elimination of radiation therapy (XRT). Results: 69/368 (18.75%) patients were less than 5 years at diagnosis. When compared to older patients, there was a trend towards male predominance (M:F 4.31 v. 2.65; p=0.2), but no difference in the incidence of B-symptoms (26.1% v. 32.9%; p=1.0) and stage distribution (p=1.0). There was less mediastinal involvement (p=0.025) or bulky disease (p=0.01) in the younger patients. These patients had more mixed cellularity and less nodular sclerosis subtype (p=0.025). Fifty-five were treated with chemotherapy (CTX) alone, 12 with combined modality therapy (CMT) and 2 with XRT only. 35/55 CTX patients were treated with ABVD (20 per standard schedule, 13 modified and 2 unknown), 12 MOPP and 8 with hybrid or combination CTX (4 MOPP/ABVD, 3 COPP/ABVD and 1 unspecified). All CMT patients received ABVD (9 standard and 3 modified) and XRT (1500cGy/IF for 5, 1500cGy/EF for 4 and 2400cGy/EF, 2720cGy/IF and 3060cGy/IF for one each). The two XRT alone patients had stage I cervical disease and received 3900cGy and 3250cGy IFXRT. At ten years the EFS and OS for these patients under 5-years of age was 81.5% and 90.4%, respectively, compared to 75.5% and 90.5% for the children between 5 and 14 years of age (p>0.5 for both comparisons). OS (86.4% v. 100%; p=0.3, Log Rank test) and EFS (81.0% v. 90.9%; p=0.4, Log Rank test) for CTX v. CMT groups were not statistically significantly different. The CTX group had more B-symptoms (29.1% v. 16.1%) and higher stage disease (stage III/IV 47.3% v. 25%; stage IV 12.73% v. 0%). Conclusions: HL patients <5years old do not present with higher risk disease than older children. They can successfully be treated without XRT using CTX alone. XRT can be reserved for treating the few who relapse. This may result in reduction in XRT related toxicity, which can be significant in these very young children.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 59-59 ◽  
Author(s):  
Abderrahman Abdelkefi ◽  
Saloua Ladeb ◽  
Tarek Ben Othman ◽  
Lamia Torjman ◽  
Amel Lakhal ◽  
...  

Abstract Background: Autologous stem cell transplantation (ASCT) is now considered standard therapy in young patients (<65 years) with multiple myeloma (MM). The Intergroupe Francophone du Myelome conducted a randomized trial of the treatment of MM with high-dose chemotherapy followed by either one or two successive ASCTs. The probabilities of event-free-survival and overall survival were doubled with a double transplant. However, no randomized trial has compared tandem transplant up-front with a strategy including planned second ASCT at relapse or progression. Therefore, we performed a multicenter, sequential, randomized trial designed to assess the optimal timing of a second ASCT. Methods: From May 2003 to April 2006, 140 patients with symptomatic MM (de novo) and less than 60 years of age, were randomly assigned to receive either tandem transplantation up-front (within 6 months of the first transplantation) [Arm A, n=69] or one ASCT followed by a consolidation therapy with thalidomide (day +90, 100 mg/per day during 5 months) [Arm B, n=71]. Patients included in the arm B received a second transplant in case of disease progression on consolidation therapy, or in case of relapse in responders. Clinical characteristics of each group were similar. In both arms of the study, ASCT was preceded by first-line therapy with thalidomide-dexamethasone and subsequent collection of peripheral blood stem cells with high-dose cyclophosphamide (4 g/m2) and G-CSF. Data were analyzed on an intent-to-treat basis. Results: With a median follow-up of 23 months (range: 6–34), the 2-year overall survival was 55% in the arm A and 75% in the arm B. Survival curves were not different (P=0.28, log-rank test). The 2-year event-free survival was 41% in the arm A and 60% in the arm B (P=0.4, log-rank-test). In the arm B, relapse-free survival of ≥ 16 months following the first transplantation was an important predictor of overall survival (p< 0.001). Conclusion: Data from the present study suggest that up-front single ASCT followed by a consolidation therapy with thalidomide and a second ASCT after relapse or progression is a safe and effective global strategy to treat MM patients. Longer follow-up is needed before definite conclusions can be given concerning the optimal timing of second autologous transplantations in patients with MM.


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