How Eye-Preserving Therapy Affects Long-Term Overall Survival in Heritable Retinoblastoma Survivors

2016 ◽  
Vol 34 (26) ◽  
pp. 3183-3188 ◽  
Author(s):  
Petra Temming ◽  
Marina Arendt ◽  
Anja Viehmann ◽  
Lewin Eisele ◽  
Claudia H.D. Le Guin ◽  
...  

Purpose Intraocular retinoblastoma is curable, but survivors with a heritable predisposition are at high risk for second malignancies. Because second malignancies are associated with high mortality, prognostic factors for second malignancy influence long-term overall survival. This study investigates the impact of all types of eye-preserving therapies on long-term survival in the complete German cohort of patients with heritable retinoblastoma. Patients and Methods Overall survival, disease staging using international scales, time period of diagnosis, and treatment type were analyzed in the 633 German children treated at the national reference center for heritable retinoblastoma. Results The 5-year overall survival of children diagnosed in Germany with heritable retinoblastoma between 1940 and 2008 was 93.2% (95% CI, 91.2% to 95.1%), but long-term mortality was increased compared with patients with nonheritable disease. Overall survival correlated with tumor staging, and 92% of patients were diagnosed with a favorable tumor stage (International Retinoblastoma Staging System stage 0 or I). Despite a 5-year overall survival of 97.4% (95% CI, 96.0% to 98.8%) in patients with stage 0 or I, only 79.5% (95% CI, 74.2% to 84.8%) of these patients survived 40 years after diagnosis. Long-term overall survival was reduced in children treated with eye-preserving radiotherapy compared with enucleation alone, and adding chemotherapy aggravated this effect. Conclusion The benefits of preserving vision must be balanced with the impact of eye-preserving treatments on long-term survival in heritable retinoblastoma, and the genetic background of the patient influences choice of eye-preserving treatment. Germline RB1 genetic analysis is important to identify heritable retinoblastoma among unilateral retinoblastoma cases. Eye-preserving radiotherapy should be carefully considered in patients with germline RB1 mutations. Life-long oncologic follow-up is crucial for all retinoblastoma survivors, and less detrimental eye-preserving therapies must be developed.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3614-3614 ◽  
Author(s):  
Sylvain Choquet ◽  
Stefan Oertel ◽  
Ioannis Anagnostopoulos ◽  
Hanno Riess ◽  
Madalina Uzunov ◽  
...  

Abstract Background: PTLD is a rare and severe complication of solid organ and hematopoetic stem cell transplantation and CNS localizations are well known to be associated with an unfavourable outcome. Published data on PTLD with CNS involvement (CNS-PTLDs) are nearly inexistent and the impact of rituximab is unknown. Methods: We performed a retrospective analysis on CNS-PTLDs in two centres, the Pitié salpêtrière university hospital in Paris, France, and the Charité university hospital in Berlin, Germany, in order to have an homogeneous way to handle these diseases and to avoid biases of large national registers. PTLDs with extra-CNS localization were excluded. While attitudes for diagnosis, staging and initial immunosuppression diminution were identical, one centre largely used intravenous (iv) rituximab and radiotherapy while the other preferred high dose chemotherapy. The Pitié Salpêtrière series of 72 PTLD patients without CNS involvement served as a control population to identify specific disease characteristics of primary CNS-PTLD. Results: 24 patients with CNS-PTLD (median age 55y) have been analyzed and compared to the non-CNS PTLD group (table I). The mean follow-up of patients alive is 5 years. Primary CNS-PTLD are clearly of late onset (mean 1366 days after transplantation) with only 3/24 patients diagnosed within the first year after transplantation. There was a significant overrepresentation of renal allografts in the CNS-PTLD group as compared to PTLDs without CNS involvement, (75% vs 29%). Primary CNS-PTLDs were always of B-cell phenotype and tumors were EBV positive in 88% of cases. Treatment of primary CNS-PTLDs consisted of chemotherapy (CT) alone with high dose (HD) Mtx and/or HD AraC in 8 cases, intrathecal (it) Mtx only in 1 case and it single agent rituximab in 1 case. Rituximab has been used in combination with CT in 2 cases. Radiotherapy (RX) was used at a mean dose of 30 Gy in combination with CT in 6 patients, and in combination with rituximab in 6 patients. The overall survival of patients suffering from primary CNS-PTLD was 180 days, but some patients obtained sustained complete remissions (CR) and 11 survived more than one year [395d – 3965d]. Eight patients are alive at the time of analysis, 9 died of PTLD progression and 2 by early sepsis. The mean DFS is 1456 days. Among the 13 patients obtaining a CR, only one relapsed 6 years after his first PTLD diagnosis in an extra CNS form. Five patients died, 3 by sudden death (d60, d408, d671), one by cerebral toxoplasmosis (d703) and one by sepsis (d91). Among patients with long term survival, 5 have been treated with CT alone, 3 by RX +/− R and 3 with combined CT-RX. The role of rituximab in primary CNS-PTLD is still unclear, as only 4/9 patients treated with rituximab achieved survival, all the more so since it as been always used but once in association. Concusion: Primary CNS-PTLD is a specific entity inside the PTLD family, with a high representation of kidney grafts and EBV positive tumors. As in immunocompetent patients, long survival is possible, especially with HD CT with or without RX. The impact of rituximab seems to be reduced. CNS-PTLD Non CNS PTLD n 24 72 Age (years) 55 47 Sex ratio (M/F) 12/12 49/72 Delay from transplantation 1366 days 830 Kidney transplantation 75% (18/24) 29% Monomorphic/polymorphic 86% (19/22) 68% B phenotype 100% (24/24) 90% EBV positive (tumor) 88% (21/24) 71% ECOG > 2 33% (7/21) 14% (18/70) Overall survival 180 days 372 days Table1: comparison between primary CNS-PTLD and non CNS-PTLD


2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 169-169
Author(s):  
Martin Snajdauf ◽  
Tomas Harustiak ◽  
Alexandr Pazdro ◽  
Robert Lischke

Abstract Background Esophagectomy with 2–3 field lymph node dissection is one of the most invasive surgical treatment for malignancy and is still associated with a high mortality and morbidity despite improvements in surgical techniques and postoperative management. The impact of postoperative complications on perioperative morbidity is widely accepted. But the impact of postoperative complications on long-term survival remains controversial. Methods A retrospective analysis was performed on patient who underwent transthoracic esophagectomy with intrathoracic anastomosis for esophageal cancer between January 2005 and December 2012 in our department (415 patients). We excluded non-radical resections (R1, R2 – 27 patients, 6.5%) and patients who died within 90 day after operation (20 patients, 4.8%). Data on gender, BMI, histologic diagnosis, tumor staging, neoadjuvant treatment, comorbidities, technical complications and postoperative medical complications were reviewed. Considered postoperative complications were anastomotic leak, empyema, chyle leak, pneumonia, ARDS, cardiac arrhythmia, wound infection and urinary tract infection. We analysed separately extrapolated serious complications Clavien Dindo 3–4 and their possible impact on overall survival. Prognostic factors were assessed by multivariate analysis. Results Total number of analysed patients was 363. The median follow up was 8.5 years. From the baseline characteristics, the presence of atrial fibrillation (P = 0.0157, HR 2.376) and hypertension (P = 0.0093, HR 1.488), higher staging pT3–4 (0.0146, HR 1.437) and presence of lymph node metastasis pN + (P < 0.001, HR 2.263) had a negative impact on overall survival. Among the postoperative complications, only chyle leak (P = 0.0327, HR 4.023) had a negative prognostic factor on overall survival. Conclusion In this single institution series, among the postoperative complications only chylothorax affect negatively the overall survival. Accurate ligation of resected thoracic duct stumps to minimize chyle leak is important to improve outcomes. The influence of others postoperative complications wasn’t significant. We assume important to exclude postoperative mortality from analysis to prevent bias. Disclosure All authors have declared no conflicts of interest.


2021 ◽  
Vol 11 ◽  
Author(s):  
Hua-Yang Pang ◽  
Lin-Yong Zhao ◽  
Hui Wang ◽  
Xiao-Long Chen ◽  
Kai Liu ◽  
...  

BackgroundThis study aimed to evaluate the impact of postoperative complication and its etiology on long-term survival for gastric cancer (GC) patients with curative resection.MethodsFrom January 2009 to December 2014, a total of 1,667 GC patients who had undergone curative gastrectomy were analyzed. Patients with severe complications (SCs) (Clavien–Dindo grade III or higher complications or those causing a hospital stay of 15 days or longer) were separated into a “complication group.” Univariate and multivariate analyses were performed to reveal the relationship between postoperative complications and long-term survival. A 2:1 propensity score matching (PSM) was used to balance baseline parameters between the two groups.ResultsSCs were diagnosed in 168 (10.08%) patients, including different etiology: infectious complications (ICs) in 111 (6.66%) and non-infectious complications (NICs) in 71 (4.26%) patients. Multivariate analysis showed that presence of SCs (P=0.001) was an independent prognostic factor for overall survival, and further analysis by complication type demonstrated that the deteriorated overall survival was mainly caused by ICs (P=0.004) rather than NICs (P=0.068). After PSM, patients with SCs (p=0.002) still had a significantly decreased overall survival, and the presence of ICs (P=0.002) rather than NICs (P=0.067) showed a negative impact on long-term survival.ConclusionSerious complications, particularly of an infectious type, may have a negative impact on overall survival of GC patients. However, additional multicenter prospective studies with larger sample size are required to verify this issue.


2019 ◽  
Vol 32 (Supplement_2) ◽  
Author(s):  
J H Saunders ◽  
F Yanni ◽  
M S Dorrington ◽  
C R Bowman ◽  
R S Vohra ◽  
...  

Abstract Aim Postoperative complications following the resection of oesophago-gastric carcinoma can result in considerable early morbidity and mortality, however the long-term effects are less clear. Literature reports are mixed, so it remains unclear if complications reduce survival, as has been demonstrated in colorectal cancer. Background & Methods Some 1100 patients who underwent oesophago-gastric resection between 2006-16 were stratified by complication severity to determine the effect of leak and severe non-leak related complications on overall survival, recurrence and disease free survival. Results The median age was 69 years, 48% had stage III disease, with cancer recurrence in 39%. Clavien-Dindo (CD) complications ≥ III occurred in 22.2% of patients. The most common complications were pulmonary (30%), with a 13% incidence of pneumonia, 10% atrial dysrhythmia and 9.6% anastomotic leak. In comparison to CD 0-I complication free patients, those with CD III-IV leak did not suffer a significantly reduced survival. However patients with CD III-IV non-leak related complications were associated with a significant reduction in median overall survival (19.7 vs. 42.7 months) and disease free survival (18.4 vs. 36.4 months). Cox regression revealed age, stage, resection margin, and CD III-IV non-leak complications as independently associated with poor overall and disease free survival. Conclusion This cohort demonstrates that whilst leak does not affect long-term survival, other severe postoperative complications do significantly reduce overall survival and disease recurrence. A reduction in these complications, such as pneumonia, seen with adoption of hybrid / minimally invasive surgery may help change this pattern of disease recurrence and reduced survival.


Author(s):  
Anantha Madhavan ◽  
Sivesh K Kamarajah ◽  
Maziar Navidi ◽  
S Wahed ◽  
Arul Immanuel ◽  
...  

Summary To compare long-term and short-term outcomes in patients &lt;70 years old with those ≥ 70 years old, who underwent transthoracic esophagectomy for carcinoma. With an ageing population more patients, with increasing co-morbidities are being diagnosed with potentially curable esophageal cancer. Concerns exist regarding offering older patients esophagectomy, conversely undue prejudice may exists that may prevent surgery being offered. Consecutive patients from a single unit between January 2000 and July 2016 that underwent trans-thoracic esophagectomy with or without neoadjuvant treatment for carcinoma were included. Short-term outcomes including morbidity, mortality, length of stay and long-term survival were compared between those &lt;70 and those ≥ 70. This study identified 992 patients who underwent esophagectomy during the study period, of which 302 (30%) ≥ 70 years old. Greater proportion ≥ 70 years old had SCC (squamous cell carcinoma) (23%) than &lt;70 (18%) (p = 0.07). Patients ≥ 70 years old were noted to have higher ASA Grade 3 (34% vs 25%, p = 0.004) and were less likely to receive neoadjuvant treatment (64% vs 45% p&lt;0.001). Length of stay was longer in ≥ 70 (14 vs 17 days p&lt;0.001), and there were more complications (63% vs 75% p&lt;0.001). In hospital mortality was higher in ≥ 70 (2% vs 5% p = 0.026). Overall survival was 50 months in &lt;70 vs 36 months in ≥ 70 (p = &lt;0.001). In &lt;70s with adenocarcinoma, overall survival was 52 months vs 35 months in the ≥ 70 (p&lt;0.001). No significant difference in survival in patients with SCC, 49 months in &lt;70 vs 54 months in ≥ 70 (p = 0.711). Increased peri-operative morbidity and mortality combined with the reduction in the long term survival in the over 70s cohort should be addressed when counselling patients undergoing curative resection for oesophageal cancer.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 7584-7584
Author(s):  
A. Kilic ◽  
M. J. Schuchert ◽  
J. R. Landreneau ◽  
J. P. Landreneau ◽  
A. Oostdyk ◽  
...  

7584 Background: The aim of this study was to evaluate the impact of length of hospital stay (LOS) following surgical resection of stage I non-small cell lung cancer (NSCLC) on long-term survival. Methods: We reviewed the records of patients undergoing surgical resection for stage I NSCLC at our institution between 1990–2003. Patients not surviving hospitalization related to their surgery were excluded from analysis. Multivariate analysis was utilized to evaluate the impact of age, gender, tumor histology, tumor stage, LOS, and type of operation (lobar or sublobar) on long-term (>5 year) survival. As a secondary analysis, Kaplan-Meier survival curves of patients stratified according to LOS were compared using the log-rank test. Two-tailed p-values less than 0.05 were considered statistically significant. Results: A total of 730 patients underwent lobectomy (n=518) or sublobar resection during the study time period. There were 18 (2.5%) operative or in-hospital mortalities. Median LOS was 6 (range 1–81) and 7 (range 1–46) days in the lobar and sublobar cohorts, respectively. Patients with a longer hospital stay (≥14 days) had significantly worse 5- and 10-year overall survival rates as compared to those with a shorter hospitalization (lobectomy: 5-year- 60.3% vs 33.8%; 10-year-27.3% vs 8.4%; p<0.001; sublobar: 5-year-44.3% vs 11.7%; 10-year-9.9% vs 0%; p=0.006). There were 171 patients with extended clinical follow-up who had survived at least 5 years (mean follow-up = 88.1 ± 2.0 months). Multivariate analysis demonstrated that LOS predicted long-term survival independent of patient age, gender, tumor histology, tumor stage, and type of operation (p=0.013). Conclusions: LOS following surgical resection of stage I NSCLC is an independent predictor of long-term survival. These survival differences related to hospital stay may be related to underlying medical co-morbidities important to the decision making regarding therapy of patients with otherwise resectable stage I lung cancer. Prospective assessment of medical co-morbidities may be an important initiative for future treatment planning of early stage lung cancer patients. No significant financial relationships to disclose.


2021 ◽  
Vol 28 ◽  
pp. 107327482199743
Author(s):  
Ke Chen ◽  
Xiao Wang ◽  
Liu Yang ◽  
Zheling Chen

Background: Treatment options for advanced gastric esophageal cancer are quite limited. Chemotherapy is unavoidable at certain stages, and research on targeted therapies has mostly failed. The advent of immunotherapy has brought hope for the treatment of advanced gastric esophageal cancer. The aim of the study was to analyze the safety of anti-PD-1/PD-L1 immunotherapy and the long-term survival of patients who were diagnosed as gastric esophageal cancer and received anti-PD-1/PD-L1 immunotherapy. Method: Studies on anti-PD-1/PD-L1 immunotherapy of advanced gastric esophageal cancer published before February 1, 2020 were searched online. The survival (e.g. 6-month overall survival, 12-month overall survival (OS), progression-free survival (PFS), objective response rates (ORR)) and adverse effects of immunotherapy were compared to that of control therapy (physician’s choice of therapy). Results: After screening 185 studies, 4 comparative cohort studies which reported the long-term survival of patients receiving immunotherapy were included. Compared to control group, the 12-month survival (OR = 1.67, 95% CI: 1.31 to 2.12, P < 0.0001) and 18-month survival (OR = 1.98, 95% CI: 1.39 to 2.81, P = 0.0001) were significantly longer in immunotherapy group. The 3-month survival rate (OR = 1.05, 95% CI: 0.36 to 3.06, P = 0.92) and 18-month survival rate (OR = 1.44, 95% CI: 0.98 to 2.12, P = 0.07) were not significantly different between immunotherapy group and control group. The ORR were not significantly different between immunotherapy group and control group (OR = 1.54, 95% CI: 0.65 to 3.66, P = 0.01). Meta-analysis pointed out that in the PD-L1 CPS ≥10 sub group population, the immunotherapy could obviously benefit the patients in tumor response rates (OR = 3.80, 95% CI: 1.89 to 7.61, P = 0.0002). Conclusion: For the treatment of advanced gastric esophageal cancer, the therapeutic efficacy of anti-PD-1/PD-L1 immunotherapy was superior to that of chemotherapy or palliative care.


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