scholarly journals The impact of incomplete registration on survival rate of children with very rare tumors

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jelena Rascon ◽  
Lukas Salasevicius ◽  
Giedre Rutkauskiene ◽  
Ewa Bien ◽  
Ieva Vincerzevskiene

AbstractPediatric very rare tumors (VRTs) represent a heterogeneous subset of childhood cancers, with reliable survival estimates depending dramatically on each (un)registered case. The current study aimed to evaluate the number of VRTs among Lithuanian children, to assess the impact of the registration status on survival rates and to track changes in treatment outcomes over the 16-year study period. We performed a population-based retrospective study across children below 18 years old diagnosed with VRTs in Lithuania between the years 2000 and 2015. The identified cases were cross-checked with the Lithuanian Cancer Registry—a population-based epidemiology cancer registry—for the fact of registration and survival status. The overall survival was calculated in relation to the registration status and treatment period. Thirty-seven children with VRTs were identified within the defined time frame. Six of them (16.2%) were not reported to the Lithuanian Cancer Registry at diagnosis. The probability of overall survival at 5 years (OS5y) differed significantly between the registered (n = 31) and unregistered (n = 6) cohorts: 51.6% versus 100%, respectively (p = 0.049). A 5-year survival estimate for children diagnosed with a VRT at the age of 0–14 years differed by 10 percentage points according to the registration completeness: 52.1% calculated for the entire cohort versus 42.1% for registered patients only. The OS5y has not improved over the analyzed period: 61.1% in 2000–2007 versus 57.9% in 2008–2015 (p = 0.805). The survival continued to decline beyond 5 years post-diagnosis due to late cancer-related adverse events: 59.5% of patients were alive at 5 years as compared to 44.3% at 10 years. The OS5y of children affected by VRT was lower than in more common childhood cancers. The survival rate of the unregistered patients may lead to misinterpretation of treatment outcomes. Meticulous registration of VRTs is crucial for correct evaluation of treatment outcomes, especially across small countries with few cases.

2020 ◽  
Author(s):  
Jelena Rascon ◽  
Lukas Salasevicius ◽  
Giedre Rutkauskiene ◽  
Ieva Vincerzevskiene

Abstract Background. Pediatric very rare tumors (VRTs) represent a heterogeneous subset of childhood cancers, with reliable survival estimates depending dramatically on each (un)registered case. The current study aimed to evaluate the number of VRTs among Lithuanian children, to assess the impact of the registration status on survival rate and to track changes in treatment outcome over the 16-year study period. Methods. We performed a population-based retrospective study across children below 18 years old diagnosed with a VRT in Lithuania between the years 2000 and 2015. The identified cases were cross-checked with the Lithuanian Cancer Registry – a population-based epidemiology cancer registry – for the fact of registration and survival status. The overall survival was calculated according to the registration status and treatment period. Results. Thirty-seven children with VRTs were identified within the defined time frame. Six of them (16.2%) were not reported to the Lithuanian Cancer Registry at diagnosis. The probability of overall survival at 5 years (OS5y) differed significantly between the registered (n = 31) and unregistered (n = 6) cohorts: 51.6% vs 100%, respectively (p = 0.049). A 5-year survival estimate for children diagnosed with a VRT at the age of 0-14 years differed by 10% according to the registration completeness: 52.1% calculated for the entire cohort vs 42.1% for registered patients only. The OS5y did not improve over the analyzed period: 61.1% in 2000-2007 vs 57.9% in 2008-2015 (p = 0.805). The survival continued to decline beyond 5 years due to late cancer-related adverse events: 59.5% of patients were alive at 5 years as compared to 44.3% at 10 years. Conclusions. The OS5y of children affected by VRT was lower than in more common childhood cancers. The survival rate of the unregistered patients was superior that mislead interpretation of treatment outcome. Meticulous registration of VRTs is crucial for correct evaluation of treatment outcome, especially across small countries with few cases.


2019 ◽  
Author(s):  
Lukas Salasevicius ◽  
Giedre Rutkauskiene ◽  
Ieva Vincerzevskiene ◽  
Jelena Rascon

Abstract Background: Pediatric very rare tumors (VRTs) represent a heterogeneous subset of childhood malignancies, with reliable survival rate estimations depending dramatically on each (un)registered case. The current study aimed to evaluate the number of VRTs among Lithuanian children and the change in treatment outcome over the 16 year study period as well as to assess the impact of the registration status on survival estimation. Methods . We performed a population-based retrospective analysis across children below 18 years old diagnosed with VRTs in Lithuania between the years 2000 to 2015. The identified cases were then crosschecked with the Lithuanian Cancer Registry (a population-based epidemiology cancer registry) for the registration and survival status. A five year overall survival (OS 5y ) was calculated using Kaplan-Meier estimation method. Results . Forty-four children affected by VRTs were identified within the defined time frame. Nine of them (20.5%) were not reported to the Lithuanian Cancer Registry at the time of diagnosis. The OS 5y of the entire cohort was 55.8%. The cure rate did not improve over the analyzed time periods – 54.2% in 2000-2007 vs 49.4% in 2008-2015. The OS 5y differed significantly between registered (n=35) and unregistered (n=9) cohorts: 45.1% vs 100%, respectively (p=0.016). The tumor progression was responsible for treatment failure in 95% of cases. Conclusions. The OS 5y of all analyzed children affected by VRT was lower as compared to the other childhood cancers. The survival rate of the unregistered patients was significantly superior that mislead interpretation of treatment outcome. Meticulous registration of VRTs is crucial for correct evaluation of treatment outcome, especially across small countries with fewer numbers of cases.


2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Fang Lai ◽  
Gengbiao Zhou ◽  
Shutao Mai ◽  
Xiaolian Qin ◽  
Wenting Liu ◽  
...  

Background. Sini Decoction (SND) is composed of Aconitum carmichaelii Debeaux, Zingiber officinale Roscoe, and Glycyrrhiza uralensis Fisch, having been used in China for centuries for collapsing phrase of disease. Studies reported that SND could alleviate inflammatory response, ameliorate microcirculatory disturbances, and improve shock reversal and adrenal gland glucocorticoid stress response during sepsis shock, yet the underlying mechanism is still elusive. Toll-like receptor (TLR) 4 is demonstrated to be crucially correlated with the corticosterone secretion and the impaired adrenal glucocorticoid responses in sepsis. Materials and Methods. SND at dose of 10 g/kg (in low-dose SND group, LD-SND) and 20 g/kg (in high-dose SND group, HD-SND) was administered to CLP rats. Four days later, overall survival rates of rats were calculated; rat serum and adrenal glands were collected. Basic serum corticosterone levels were determined, and the increase of corticosterone after 0.8 ug/kg ACTH injection was checked to detect the adrenocortical sensitivity to ACTH. The protein and mRNA expression of TLR4 in adrenal glands were measured to study the impact of SND on TLR4 expression. mRNA levels of IL-10 and TNF-a in adrenal glands and IL-10 and TNF-a levels in serum were also determined to study the cytokines profile. Results. SND improved the cumulative survival rate of CLP rats up to 4 days (P < 0.05 with HD-SND) and adrenocortical sensitivity to 0.8 ug/kg ACTH stimulation (P < 0.05 at 60 mins, 31.02 ± 19.23 ng/ml in LD-SND group and 32.18 ± 14.88 ng/ml in HD-SND group versus 5.03 ± 13.34 ng/ml in CLP group), with a significant decrease of protein (P < 0.05, 29.6% in LD-SND group and 27.8% in HD-SND group), mRNA expression of TLR4 (P < 0.05, 32.9% in LD-SND group and 36.1% in HD-SND group), mRNA expression of IL-10 (P < 0.05, 32.0% in LD-SND group and 29.6% in HD-SND group), TNF-a in adrenal glands (P < 0.05, 26.0% in LD-SND group and 25.3% in HD-SND group), and TNF-a level in serum (P < 0.05, 100.20 ± 19.41 pg/ml in LD-SND group and 92.40 ± 11.66 pg/ml in HD-SND group versus 134.40 ± 27.87 pg/ml in CLP group). Conclusion. SND increased overall survival rate within 4 days and attenuated adrenal insufficiency in septic rats by downregulating TLR4 mRNA and protein expression in adrenal tissue, inhibiting adrenal production of TNF-α and IL-10, and improving adrenal responsiveness. Our results suggest that SND is able to ameliorate adrenal stress responses in a local immune-adrenal crosstalk way involving downregulated expression of TLR4 in adrenal tissue. SND might be a promising treatment for adrenal insufficiency prevention in prolonged sepsis.


2019 ◽  
Vol 56 (6) ◽  
pp. 1104-1109 ◽  
Author(s):  
Jan Viehof ◽  
Elisabeth Livingstone ◽  
Elena Loscha ◽  
Paul Stockhammer ◽  
Agnes Bankfalvi ◽  
...  

AbstractOBJECTIVESPulmonary metastasectomy for malignant melanoma requires an individualized therapeutic decision. Due to recently developed novel treatment options, the prognosis of patients with melanoma has improved significantly. Validated prognostic factors that identify patients who are most likely to benefit from metastasectomy are urgently needed.METHODSWe retrospectively reviewed all consecutive patients with melanoma undergoing complete pulmonary metastasectomy between January 2010 and December 2016. The impact of age, sex, extrapulmonary metastases, preoperative systemic therapy, number of metastases, laterality and largest diameter of metastasis on survival after metastasectomy was analysed.RESULTSA total of 29 male and 32 female patients were included in the study. The median follow-up time was 25.6 months. The mean number of resected metastases was 1.7 ± 1.1 (range 1–5). Ten patients had repetitive pulmonary metastasectomies. The median survival time was 31.3 months with a 2-year survival rate of 54%. Bilateral metastases or multiple nodules were not associated with a significantly decreased overall survival rate after metastasectomy. Shorter overall survival times were observed in male patients [hazard ratio (HR) 2.9, 95% confidence interval (CI) 1.42–5.92; P = 0.0035] and in patients with nodules larger than 2 cm (HR 3.18, 95% CI 1.45–6.98; P = 0.004). In multivariable analysis, both gender and tumour size remained significant independent prognostic factors.CONCLUSIONSExcellent overall survival rates after pulmonary metastasectomy for melanoma metastases were observed in patients with a metastatic diameter less than 2 cm and in female patients. In view of improved long-term outcome due to novel treatment options, the selection of patients for pulmonary metastasectomy based on prognostic factors will become increasingly important.


PLoS ONE ◽  
2013 ◽  
Vol 8 (10) ◽  
pp. e76052 ◽  
Author(s):  
Jianfeng Luo ◽  
Linhai Xiao ◽  
Chunxiao Wu ◽  
Ying Zheng ◽  
Naiqing Zhao

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4650-4650
Author(s):  
Alessia Bari ◽  
Raffaella Marcheselli ◽  
Ivan Rashid ◽  
Goretta Bonacorsi ◽  
Orsola Bonanno ◽  
...  

Abstract Background Because in the past Chronic Myeloproliferative Disorders (CMPD) were not considered to be malignant conditions, cancer registries rarely recorded data on these diseases. Thus, information on incidence and outcome of CMPD in the population is limited. The aim of the present study was to better define epidemiological data of CMPD by examining all cases identified by the Modena Cancer Registry (MCR). Materials and methods We considered all cases of CMPD diagnosed in the Province of Modena (population 633.993 at 2001 Census). Cases, except Chronic Myeloid Leukemia, diagnosed from 1997 to 2005, were identified using the MCR database and the archival files of the centralized hemolymphopathological laboratory at Modena Cancer Centre according to ICD-O-3 codes 9950, 9960–64. Death certificate, cytology and histology report, both local and national reports of hospital admission, ICD-9 code reported in medical records were used as sources for identifying new CMPD cases and their outcome. All cases were checked and validated by a hematologist (A.B.) and a pathologist (G.B.) by a review of the original pathology report. Uniform diagnostic criteria were adopted, because the large majority of bone marrow aspirate and biopsy were examined by the same pathologist (G.B.). Clinical and follow-up data were retrieved by active search of discharge letters, review of hospital records and interview of general practitioners. Information on vital status was achieved from official population registries. Age standardized rates (ASR) were calculated according to the World Standard population. The dates of diagnosis and death or the closing date of study (December 2006) were used to estimate survival. Observed survival and relative survival were calculated according to Kaplan-Meier method and the Hakulinen approach, respectively. Results According to the 2001 World Health Organization (WHO) classification, a total of 380 cases of CMPD were identified. There were 155 Essential Thrombocythemia (ET) (41% of all CMPD), 114 Policythemia Vera (PV) (30%), 75 Idiopathic Myelofibrosis (20%), 2 Hypereosinophilic Syndrome/Chronic Eosinophilic Leukaemia (0.5%), 1 Chronic Neutrophilic Leukemia (0.3%) and 31 CMPD not otherwise specified (8%). The ASR of CMPD was 3.2/100,000 varying slightly (from 2.5 to 4.1/100,000) during the study period (p = 0.11); the crude incidence rate was 6.6/100,000. Median age at diagnosis was 69 years. No statistically significant differences were observed between sex regarding incidence and age at diagnosis. Overall relative survival was 97%, 89% and 88% at 1, 3 and 5 years after diagnosis, respectively. Analyzing CMPD, we observed a better survival for ET and PV in comparison with other subtypes (p = 0.01). Conclusions To our knowledge, this study is the first in Italy providing information on the incidence and outcome of CMPD using population-based data. Our results confirm that the risk of developing CMPD increases with age. The incidence of CMPD was substantially stable during the study period. Overall survival patterns reflect the well known chronic course of these diseases. As expected, we observed important differences in overall survival by WHO subtypes. We believe that the availability of precise epidemiological data, in particular those regarding outcome could help clinicians in choosing the most appropriate cost-effective treatments.


2019 ◽  
Vol 37 (4_suppl) ◽  
pp. 288-288
Author(s):  
Phani Keerthi Surapaneni ◽  
Zhuo Li ◽  
Lalitha Padmanabha Vemireddy ◽  
Pashtoon Murtaza Kasi ◽  
Jason Scott Starr ◽  
...  

288 Background: Obesity is a risk factor for developing cholangiocarcinoma (CCA). However, the effect of obesity on survival of CCA is unclear. The primary aim of this study was to analyze the impact of obesity upon overall survival of CCA patients. Secondary aims were to analyze impact of obesity upon other disease characteristics such as tumor site, stage, age, sex, BMI and Ca 19-9. Methods: A total of 411 unique pts diagnosed with CCA at Mayo Clinic Florida between 2000 and 2018 were retrieved from our collective SDMS database. Variables evaluated included:demographics, Body Mass Index (BMI), AJCC stage, tumor location and Ca 19-9.A total of 185 pts had all data available pertaining to these variables. We further restricted the analysis to pts with intrahepatic CCA classified BMI as per CDC criteria normal (18.5-25kg/m2), overweight (25-29.9kg/m2) and obese (≥30 kg/m2), thus leaving a total of 152 pts. Continuous and categorical variables were compared across BMI groups using Chi-squared or Fisher’s exact test. Overall survival rates after diagnosis at 1, 2 and 3 years were estimated using Kaplan-Meier method. Results: Among 152 pts included in the study, 28% were normal weight, 40% were overweight and 32% were obese. The overall survival rate at 1, 2 and 3 years for normal weight pts with all stages combined was 54.1%, 35%, and 30.7%, respectively. The overall survival rate at 1, 2 and 3 years for overweight pts with all stages combined was 59.7 %, 32.6%, and 25.4%, respectively. The overall survival rate at 1, 2 and 3 years for obese pts with all stages combined was 63.9%, 37.6%, and 26.7%, respectively(p = 0.8766). Multivariate analysis demonstrated is no significant difference in overall survival for obese pts compared to normal or overweight pts.(Table to be shown) However it showed, gender and Ca19-9 were statistically significant predictors of overall survival, with males and pts with Ca19-9≥100 doing worse (HR1.65 (CI = 1.05, 2.61, p = 0.031) and HR 2.31 (CI = 1.49, 3.59, p = < 0.01), respectively). Conclusions: BMI did not make a significant impact on the overall survival, though there may be a trend toward worse OS for ptswith higher BMI. A larger, stage focused evaluation is warranted for further exploration of this trend.


Cancer ◽  
2009 ◽  
Vol 115 (4) ◽  
pp. 776-783 ◽  
Author(s):  
Daniel Y. C. Heng ◽  
Kim N Chi ◽  
Nevin Murray ◽  
Tao Jin ◽  
Jorge A Garcia ◽  
...  

2019 ◽  
Author(s):  
Gaozan Zheng ◽  
Jinqiang Liu ◽  
Yinghao Guo ◽  
Fei Wang ◽  
Shushang Liu ◽  
...  

Abstract Background It remains controversial whether prophylactic No.10 lymph node clearance is necessary for gastric cancer. Thus, the present study aims to investigate the impact of prophylactic No.10 lymph node clearance on the perioperative complications and prognosis of upper and middle third gastric cancer.Methods A network meta-analysis to identify both direct and indirect evidence with respect to the comparison of gastrectomy alone (G-A), gastrectomy combination with splenectomy (G+S) and gastrectomy combination with spleen-preserving splenic hilar dissection (G+SPSHD) was conducted. We searched Medline, Embase, and the Cochrane Central Register of Controlled Trials (CENTRAL) for studies published before September 2018. Perioperative complications and overall survival were analyzed. Hazard ratios (HR) were extracted from the publications on the basis of reported values or were extracted from survival curves by established methods.Results Ten retrospective studies involving 2565 patients were included. In the direct comparison analyses, G-A showed comparable 5-year overall survival rate (HR: 1.1, 95%CI: 0.97-1.3) but lower total complication rate (OR: 0.37, 95%CI: 0.17-0.77) compared with G+S. Similarly, the 5-year overall survival rate between G+SPSHD and G+S was comparable (HR: 1.1, 95%CI: 0.92-1.4), while the total complication rate of G+SPSHD was lower than that of G+S (OR: 0.50, 95%CI: 0.28-0.88). In the indirect comparison analyses, both the 5-year overall survival rate (HR: 1.0, 95%CI: 0.78-1.3) and total complication rate (OR: 0.75, 95%CI: 0.29-1.9) were comparable between G-A and G+SPSHD.Conclusion Prophylactic No.10 lymph node clearance was not recommended for treatment of upper and middle third gastric cancer.


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