scholarly journals Age Dependency of the Prognostic Impact of Tumor Genomics in Localized Resectable MYCN-Nonamplified Neuroblastomas. Report From the SIOPEN Biology Group on the LNESG Trials and a COG Validation Group

2020 ◽  
Vol 38 (31) ◽  
pp. 3685-3697 ◽  
Author(s):  
Inge M. Ambros ◽  
Gian-Paolo Tonini ◽  
Ulrike Pötschger ◽  
Nicole Gross ◽  
Véronique Mosseri ◽  
...  
Keyword(s):  
Age Groups ◽  
Staging System ◽  
Mycn Amplification ◽  
Prognostic Impact ◽  
Excellent Outcome ◽  
Chromosome 1P ◽  
Group I ◽  
Group 5 ◽  
Stage 1 ◽  
Biology Group

PURPOSE For localized, resectable neuroblastoma without MYCN amplification, surgery only is recommended even if incomplete. However, it is not known whether the genomic background of these tumors may influence outcome. PATIENTS AND METHODS Diagnostic samples were obtained from 317 tumors, International Neuroblastoma Staging System stages 1/2A/2B, from 3 cohorts: Localized Neuroblastoma European Study Group I/II and Children’s Oncology Group. Genomic data were analyzed using multi- and pangenomic techniques and fluorescence in-situ hybridization in 2 age groups (cutoff age, 18 months) and were quality controlled by the International Society of Pediatric Oncology European Neuroblastoma (SIOPEN) Biology Group. RESULTS Patients with stage 1 tumors had an excellent outcome (5-year event-free survival [EFS] ± standard deviation [SD], 95% ± 2%; 5-year overall survival [OS], 99% ± 1%). In contrast, patients with stage 2 tumors had a reduced EFS in both age groups (5-year EFS ± SD, 84% ± 3% in patients < 18 months of age and 75% ± 7% in patients ≥ 18 months of age). However, OS was significantly decreased only in the latter group (5-year OS ± SD in < 18months and ≥ 18months, 96% ± 2% and 81% ± 7%, respectively; P = .001). In < 18months, relapses occurred independent of segmental chromosome aberrations (SCAs); only 1p loss decreased EFS (5-year EFS ± SD in patients 1p loss and no 1p loss, 62% ± 13% and 87% ± 3%, respectively; P = .019) but not OS (5-year OS ± SD, 92% ± 8% and 97% ± 2%, respectively). In patients ≥ 18 months, only SCAs led to relapse and death, with 11q loss as the strongest marker (11q loss and no 11q loss: 5-year EFS ± SD, 48% ± 16% and 85% ± 7%, P = .033; 5-year OS ± SD, 46% ± 22% and 92% ± 6%, P = .038). CONCLUSION Genomic aberrations of resectable non– MYCN-amplified stage 2 neuroblastomas have a distinct age-dependent prognostic impact. Chromosome 1p loss is a risk factor for relapse but not for diminished OS in patients < 18 months, SCAs (especially 11q loss) are risk factors for reduced EFS and OS in those > 18months. In older patients with SCA, a randomized trial of postoperative chemotherapy compared with observation alone may be indicated.

The Presence Of Spine Bone Plasmacytomas At The Time Of Multiple Myeloma (MM) Diagnosis Confers Poor Prognosis

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5366-5366
Author(s):  
Marie-Christine Kyrtsonis ◽  
Eftychia Nikolaou ◽  
Dimitrios Maltezas ◽  
Katerina Sarris ◽  
Efstathios Koulieris ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Adverse Outcome ◽  
Staging System ◽  
New Drugs ◽  
High Dose ◽  
Prognostic Impact ◽  
Distinct Subgroup ◽  
The Difference ◽  
Stage 1

Abstract The negative prognostic consequences of extramedullary plasmacytomas in the context of symptomatic MM is well known. On the contrary, the clinical impact of bone plasmacytomas (BP) has not been extensively studied, although their presence is one of Duries’ major MM diagnostic criteria. The aim of the present study was to study eventual differences between MM patients presenting at diagnosis with or without BP. Patients and Methods Two hundred and twelve symptomatic MM patients were studied from diagnosis to last follow-up. Their median age was 66 years and 60% were men. Twenty-three percent, 27% and 50% of patients were in ISS stage 1, 2 and 3 respectively; MM type was IgG in 59% , IgA in 26%, light chain only in 12%, IgD in 1,5% and non- or oligo-secretory in 1,5%. All patients required treatment and BP ones were additionally given radiotherapy. The median follow-up time of the whole cohort was 46,4 months. Results Forty patients (19%) had a BP at the time of their diagnosis. BP was located in the spine (BP-S) in 19 out of 40 and in other sites (BP-O: skull, pelvis, long bones, and ribs) in 21 patients. BP patients had equivalent demographics, stage and MM type as the others. Non-BP patients tended to have a better survival than BP ones but the difference was not statistically significant (p=0.1). However, BP-S patients had a significantly worse outcome than BP-O patients (24±6 versus 48±11 months, p<0.00001), and indeed worse than non-BP patients. It is worthwhile to outline that 17 out of 19 BP-S patients had received new drugs (thalidomide, lenalidomide, Bortezomib) during their course while 8 of them underwent high dose treatment with autologous stem-cell transplantation. In addition, the ISS staging has absolutely no prognostic impact in these patients (p=0.5), on the contrary to the whole cohort (p=0.03). In conclusion, MM patients presenting spine plasmacytoma at diagnosis constitute a distinct subgroup with adverse outcome that is difficultly predictable, given that the current staging system does not separate them. Further confirmation of this finding in larger cohorts is needed, as well as exploration of the biologic background. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Significance of MYCN Amplification in International Neuroblastoma Staging System Stage 1 and 2 Neuroblastoma: A Report From the International Neuroblastoma Risk Group Database

2009 ◽  
Vol 27 (3) ◽  
pp. 365-370 ◽  
Author(s):  
Rochelle Bagatell ◽  
Maja Beck-Popovic ◽  
Wendy B. London ◽  
Yang Zhang ◽  
Andrew D.J. Pearson ◽  
...  
Keyword(s):  
Risk Group ◽  
Prognostic Significance ◽  
Staging System ◽  
Moderate Intensity ◽  
Mycn Amplification ◽  
Localized Neuroblastoma ◽  
International Neuroblastoma Staging System ◽  
Stage 1 ◽  
Additional Therapy

Purpose Treatment of patients with localized neuroblastoma with unfavorable biologic features is controversial. To evaluate the outcome of children with low-stage MYCN-amplified neuroblastoma and develop a rational treatment strategy, data from the International Neuroblastoma Risk Group (INRG) database were analyzed. Patients and Methods The database is comprised of 8,800 patients. Of these, 2,660 patients (30%) had low-stage (International Neuroblastoma Staging System stages 1 and 2) neuroblastoma, known MYCN status, and available follow-up data. Eighty-seven of these patients (3%) had MYCN amplified tumors. Results Patients with MYCN-amplified, low-stage tumors had less favorable event-free survival (EFS) and overall survival (OS) than did patients with nonamplified tumors (53% ± 8% and 72% ± 7% v 90% ± 1% and 98% ± 1%, respectively). EFS and OS were statistically significantly higher for patients whose tumors were hyperdiploid rather than diploid (EFS, 82% ± 20% v 37% ± 21%; P = .0069; OS, 94% ± 11% v 54% ± 15%; P = .0056, respectively). No other variable had prognostic significance. Initial treatment consisted of surgery alone for 29 (33%) of 87 patients. Details of additional therapy were unknown for 14 patients. Twenty-two patients (25%) underwent surgery and moderate-intensity chemotherapy; another 22 underwent surgery, intensive chemotherapy, and radiation therapy. Nine of the latter 22 underwent stem cell transplantation. Survival in patients who received transplantation did not differ from survival in those who did not receive transplantation. Conclusion Among patients with low-stage, MYCN-amplified neuroblastoma, outcomes of patients with hyperdiploid tumors were statistically, significantly better than those with diploid tumors. The data suggest that tumor cell ploidy could potentially be used to identify candidates for reductions in therapy. Further study of MYCN-amplified, low-stage neuroblastoma is warranted.

Recursive partitioning for new classification of patients with esophageal cancer treated by chemoradiotherapy.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e14500-e14500
Author(s):  
Motoo Nomura ◽  
Kohei Shitara ◽  
Takeshi Kodaira ◽  
Chihiro Kondoh ◽  
Daisuke Takahari ◽  
...  
Keyword(s):  
Esophageal Cancer ◽  
Lymph Node ◽  
Recursive Partitioning ◽  
Staging System ◽  
Prognostic Impact ◽  
New Classification ◽  
Group I ◽  
Lymph Node Size ◽  
Staging Classification ◽  
Node Size

e14500 Background: The 7th edition of the American Joint Committee on Cancerstaging system does not include lymph node size in the guidelines for staging patients with esophageal cancer. The objectives of this study were to determine the prognostic impact of the largest lymph node diameter (ND) on survival and to develop and validate a new staging system for patients with esophageal squamous cell cancer who were treated with definitive chemoradiotherapy (CRT). Methods: Information on 402 patients with esophageal cancer undergoing CRT at 2 institutions was reviewed. Univariate and multivariate analyses of data from 1 institution were used to assess the impact of clinical factors on survival, and recursive partitioning analysis was performed to develop the new staging classification. To assess its clinical utility, the new classification was validated using data from the second institution. Results: According to RPA, ND stages were best when classified as ND0 (the absence of lymph node metastases), ND1 (< 2.8 cm), and ND2 (≥ 2.8 cm). By multivariate analysis, gender, T, N, and ND stages were independently and significantly associated with survival (p<0.05). The resulting new staging classification showed the following: T1-2ND0 as Group I; T3-4ND0 or T1-2ND1 as Group II; T3-4ND1 as Group III; and TanyND2 as Group IV. The 4 new stages led to good separation of survival curves in both the developmental and validation datasets (p<0.05). Conclusions: Our results showed that lymph node size is a strong independent prognostic factor and that the new staging system, which incorporated lymph node size, provided good prognostic power and discriminated effectively for patients with esophageal cancer undergoing CRT.

Reliability of the Mangled Extremity Severity Score in the Management of Peripheral Vascular Injuries in Children: A Retrospective Review

2020 ◽  
Author(s):  
Ahmed Mousa ◽  
Ossama M. Zakaria ◽  
Mai A. Elkalla ◽  
Lotfy A. Abdelsattar ◽  
Hamad Al-Game'a
Keyword(s):  
Severity Score ◽  
Vein Graft ◽  
Bypass Surgery ◽  
Age Groups ◽  
Vascular Injuries ◽  
Peripheral Vascular ◽  
Group Iii ◽  
Mangled Extremity ◽  
Group I ◽  
End To End

AbstractThis study was aimed to evaluate different management modalities for peripheral vascular trauma in children, with the aid of the Mangled Extremity Severity Score (MESS). A single-center retrospective analysis took place between 2010 and 2017 at University Hospitals, having emergencies and critical care centers. Different types of vascular repair were adopted by skillful vascular experts and highly trained pediatric surgeons. Patients were divided into three different age groups. Group I included those children between 5 and 10 years; group II involved pediatrics between 11 and 15 years; while children between 16 and 21 years participated in group III. We recruited 183 children with peripheral vascular injuries. They were 87% males and 13% females, with the mean age of 14.72 ± 04. Arteriorrhaphy was performed in 32%; end-to-end anastomosis and natural vein graft were adopted in 40.5 and 49%, respectively. On the other hand, 10.5% underwent bypass surgery. The age groups I and II are highly susceptible to penetrating trauma (p = 0.001), while patients with an extreme age (i.e., group III) are more susceptible to blunt injury (p = 0.001). The MESS has a significant correlation to both age groups I and II (p = 0.001). Vein patch angioplasty and end-to-end primary repair should be adopted as the main treatment options for the repair of extremity vascular injuries in children. Moreover, other treatment modalities, such as repair with autologous vein graft/bypass surgery, may be adopted whenever possible. They are cost-effective, reliable, and simple techniques with fewer postoperative complication, especially in poor/limited resources.

scholarly journals A CBCT Based Three-Dimensional Assessment of Mandibular Posterior Region for Evaluating the Possibility of Bypassing the Inferior Alveolar Nerve While Placing Dental Implants

Diagnostics ◽  
2020 ◽  
Vol 10 (6) ◽  
pp. 406 ◽  
Author(s):  
Mohammed G. Sghaireen ◽  
Kumar Chandan Srivastava ◽  
Deepti Shrivastava ◽  
Kiran Kumar Ganji ◽  
Santosh R. Patil ◽  
...  
Keyword(s):  
Nerve Injury ◽  
Age Groups ◽  
Inferior Alveolar Nerve ◽  
Three Dimensions ◽  
High Rate ◽  
Cortical Plate ◽  
Computed Tomography Images ◽  
Group I ◽  
Linear Distance ◽  
The Right

A high rate of nerve injury and related consequences are seen during implant placement in the posterior mandibular arch. An approach has been proposed to avoid nerve injury by dodging the inferior alveolar nerve (IAN) while placing an implant. A prospective study with a total of 240 CBCT (cone beam computed tomography) images of patients with three dentate statuses, namely, edentulous (group I), partially edentulous (group II) and dentate (group III) were included in the study. The nerve path tracing was done on CBCT images with On-demand 3D software. The three dimensions, i.e., the linear distance from the outer buccal cortical plate to the inferior alveolar nerve (BCPN), linear distance from the outer lingual cortical plate to the inferior alveolar nerve (LCPN) and linear distance from the midpoint of the alveolar crest to the inferior alveolar nerve (ACN) were assessed. The data were presented and analyzed between variables using one-way ANOVA and independent t-test in SPSS version 21.LCPN of the right 1st premolar region (p < 0.05) was significantly different among the groups with edentulous subjects recorded with the minimum value (6.50 ± 1.20 mm). Females were found to have significantly (p < 0.05) less available bone (6.03 ± 1.46 mm) on the right side of the mandibular jaw compared to males in edentulous group of patients. On comparing age groups for partially edentulous subjects, LCPN of the right 1st premolar region had significantly (p < 0.05) less available bone (6.03 ± 0.38 mm) in subjects with age ≥54 years. The IAN follows a lingual course in the molar region and later flips to the buccal side in the premolar region. The LCPN dimension in the 1st and 2nd premolar region was found to be more than 6 mm irrespective of age, gender and side of the jaw. Thus, it can be considered as a suitable site for placing implants while bypassing the IAN with CBCT assessment remaining as the mainstay in the pre-surgical phase.

Novel staging classification of primary mitral regurgitation based on the presence of cardiac damage

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A.L Van Wijngaarden ◽  
Y.L Hiemstra ◽  
P Van Der Bijl ◽  
V Delgado ◽  
N Ajmone Marsan ◽  
...  
Keyword(s):  
Mitral Regurgitation ◽  
Kidney Function ◽  
Left Ventricular ◽  
Prognostic Impact ◽  
Cardiac Damage ◽  
Stage 4 ◽  
All Cause Mortality ◽  
Primary Mitral Regurgitation ◽  
Stage 1 ◽  
Staging Classification

Abstract Background The indication for surgery in patients with severe primary mitral regurgitation (MR) is currently based on the presence of symptoms, left ventricular (LV) dilatation and dysfunction, atrial fibrillation and pulmonary hypertension. The aim of this study was to evaluate the prognostic impact of a new staging classification based on cardiac damage including the known risk factors but also including global longitudinal strain (GLS), severe left atrial (LA) dilatation and right ventricular (RV) dysfunction. Methods In total 614 patients who underwent surgery for severe primary MR with available baseline transthoracic echocardiograms were included. Patients were classified according to the extent of cardiac damage (Figure): Stage 0-no cardiac damage, Stage 1-LV damage, Stage 2-LA damage, Stage 3-pulmonary vasculature or tricuspid valve damage and Stage 4-RV damage. Patients were followed for all-cause mortality. Results Based on the proposed classification, 172 (28%) patients were classified as Stage 0, 102 (17%) as Stage 1, 134 (21%) as Stage 2, 135 (22%) as Stage 3 and 71 (11%) as Stage 4. The more advanced the stage, the older the patients were with worse kidney function, more symptoms and higher EuroScore. Kaplan-Meier curve analysis revealed that patients with more advanced stages of cardiac damage had a significantly worse survival (log-rank chi-square 35.2; p&lt;0.001) (Figure). On multivariable analysis, age, male, chronic obstructive pulmonary disease, kidney function, and stage of cardiac damage were independently associated with all-cause mortality. For each stage increase, a 22% higher risk for all-cause mortality was observed (95% CI: 1.064–1.395; p=0.004). Conclusion In patients with severe primary MR, a novel staging classification based on the extent of cardiac damage, may help refining risk stratification, particularly including also GLS, LA dilatation and RV dysfunction in the assessment. Funding Acknowledgement Type of funding source: None

scholarly journals Tumor size may influence the prognosis of solitary hepatocellular carcinoma patients with cirrhosis and without macrovascular invasion after hepatectomy

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Bin-yong Liang ◽  
Jin Gu ◽  
Min Xiong ◽  
Er-lei Zhang ◽  
Zun-yi Zhang ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Tumor Size ◽  
Staging System ◽  
Prognostic Impact ◽  
Term Survival ◽  
Long Term Survival ◽  
Before And After ◽  
Cirrhotic Patients ◽  
Curative Hepatectomy

AbstractHepatocellular carcinoma (HCC) is usually associated with varying degrees of cirrhosis. Among cirrhotic patients with solitary HCC in the absence of macro-vascular invasion, whether tumor size drives prognosis or not after hepatectomy remains unknown. This study aimed to investigate the prognostic impact of tumor size on long-term outcomes after hepatectomy for solitary HCC patients with cirrhosis and without macrovascular invasion. A total of 813 cirrhotic patients who underwent curative hepatectomy for solitary HCC and without macrovascular invasion between 2001 and 2014 were retrospectively studied. We set 5 cm as the tumor cut-off value. Propensity score matching (PSM) was performed to minimize the influence of potential confounders including cirrhotic severity that was histologically assessed according to the Laennec staging system. Recurrence-free survival (RFS) and overall survival (OS) were compared between the two groups before and after PSM. Overall, 464 patients had tumor size ≤ 5 cm, and 349 had tumor size > 5 cm. The 5-year RFS and OS rates were 38.3% and 61.5% in the  ≤ 5 cm group, compared with 25.1% and 59.9% in the > 5 cm group. Long-term survival outcomes were significantly worse as tumor size increased. Multivariate analysis indicated that tumor size > 5 cm was an independent risk factor for tumor recurrence and long-term survival. These results were further confirmed in the PSM cohort of 235 pairs of patients. In cirrhotic patients with solitary HCC and without macrovascular invasion, tumor size may significantly affect the prognosis after curative hepatectomy.

scholarly journals Family history of early onset acute lymphoblastic leukemia is suggesting genetic associations

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Xinjun Li ◽  
Kristina Sundquist ◽  
Jan Sundquist ◽  
Asta Försti ◽  
Kari Hemminki
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
Lymphoblastic Leukemia ◽  
Statistical Significance ◽  
Age Groups ◽  
Age Group ◽  
Genetic Associations ◽  
Sequencing Data ◽  
Cancer Data ◽  
Group 5 ◽  
Prostate Cancers

AbstractChildhood acute lymphoblastic leukemia (ALL) has an origin in the fetal period which may distinguish it from ALL diagnosed later in life. We wanted to test whether familial risks differ in ALL diagnosed in the very early childhood from ALL diagnosed later. The Swedish nation-wide family-cancer data were used until year 2016 to calculate standardized incidence ratios (SIRs) for familial risks in ALL in three diagnostic age-groups: 0–4, 5–34 and 35 + years. Among 1335 ALL patients diagnosed before age 5, familial risks were increased for esophageal (4.78), breast (1.42), prostate (1.40) and connective tissue (2.97) cancers and leukemia (2.51, ALL 7.81). In age-group 5–34 years, rectal (1.73) and endometrial (2.40) cancer, myeloma (2.25) and leukemia (2.00, ALL 4.60) reached statistical significance. In the oldest age-group, the only association was with Hodgkin lymphoma (3.42). Diagnostic ages of family members of ALL patients were significantly lower compared to these cancers in the population for breast, prostate and rectal cancers. The patterns of increased familial cancers suggest that BRCA2 mutations could contribute to associations of ALL with breast and prostate cancers, and mismatch gene PMS2 mutations with rectal and endometrial cancers. Future DNA sequencing data will be a test for these familial predictions.

scholarly journals Age-Related Alteration of Risk Profile, Inflammatory Response, and Angiographic Findings in Patients with Acute Coronary Syndrome

2009 ◽  
Vol 3 ◽  
pp. CMC.S2118 ◽  
Author(s):  
Hala Mahfouz Badran ◽  
Mohamed Fahmy Elnoamany ◽  
Tarek Salah Khalil ◽  
Mostafa Mohamed Ezz Eldin
Keyword(s):  
Inflammatory Response ◽  
Age Groups ◽  
Young Patients ◽  
Risk Profile ◽  
High Morbidity ◽  
Cardiac Enzymes ◽  
Coronary Syndrome ◽  
Group I ◽  
Hs Crp ◽  
Angiographic Findings

Background Coronary artery disease (CAD) is a major public health problem which in turn imposes a significant burden on health care systems because of high morbidity and mortality. Although the multifactorial etiology of CAD increases with age, but in recent years, the incidence is increasing among younger age groups. Objectives In this study we aimed to evaluate the effect of age on risk profile, inflammatory response and the angiographic findings in patients with ACS. Patients and Methods The study comprised 253 ACS patients. Seventy six (30%) with UA, 56 (22%) with NSTEMI and 121(48%) with STEMI diagnosis. The value of Hs-CRP, lipid profile, cardiac enzymes, risk factors, EF% and angiographic score were analyzed and compared in different age groups. Results Group 1 (n = 68) with age <45 years, group II (n = 110) with age ≥45-<65 years and group III (n = 75) ≥65 years. Group I had more prevalence of male sex, smoking, family history, hypertriglyceridemia and low levels of HDL (P < 0.01), higher incidence of STEMI (P < 0.01) and lower prevalence of UA (P < 0.01). Diabetes mellitus, hypertension, and female gender were more common in older groups. Hs-CRP was significantly lower in the young age (group I). Group I showed a preponderance of single-vessel disease, lower coronary atherosclerotic score and prevalent left anterior descending artery (LAD) involvement compared with older age groups. Hs-CRP was positively correlated to severity of CAD only in older groups. Stepwise multiple regression analysis showed that age, male gender, cardiac enzymes and EF% were common predictors of multivessel disease. Smoking was independent predictor in young patients <45 years while diabetes and Hs-CRP was the key predictor in older patient groups. Conclusion Young patients with ACS had different clinical, angiographic and biochemical profile. Hs-CRP peak concentration did not correlate with angiographic findings in young patients that could be attributed to different risk profile and discrete underlying mechanism.

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