Revised Neuroblastoma Risk Classification System: A Report From the Children's Oncology Group

2021 ◽  
pp. JCO.21.00278
Author(s):  
Meredith S. Irwin ◽  
Arlene Naranjo ◽  
Fan F. Zhang ◽  
Susan L. Cohn ◽  
Wendy B. London ◽  
...  
Keyword(s):  
Overall Survival ◽  
High Risk ◽  
Risk Group ◽  
Staging System ◽  
Risk Classification ◽  
Intermediate Risk ◽  
Oncology Group ◽  
Event Free Survival ◽  
Free Survival ◽  
Pathology Classification

PURPOSE Treatment planning for children with neuroblastoma requires accurate assessment of prognosis. The most recent Children's Oncology Group (COG) risk classification system used tumor stage as defined by the International Neuroblastoma Staging System. Here, we validate a revised classifier using the International Neuroblastoma Risk Group Staging System (INRGSS) and incorporate segmental chromosome aberrations (SCA) as an additional genomic biomarker. METHODS Newly diagnosed patients enrolled on the COG neuroblastoma biology study ANBL00B1 between 2007 and 2017 with known age, International Neuroblastoma Staging System, and INRGSS stage were identified (N = 4,832). Tumor MYCN status, ploidy, SCA status (1p and 11q), and International Neuroblastoma Pathology Classification histology were determined centrally. Survival analyses were performed for combinations of prognostic factors used in COG risk classification according to the prior version 1, and to validate a revised algorithm (version 2). RESULTS Most patients with locoregional tumors had excellent outcomes except for those with image-defined risk factors (INRGSS L2) with MYCN amplification (5-year event-free survival and overall survival: 76.3% ± 5.8% and 79.9% ± 5.5%, respectively) or patients age ≥ 18 months with L2 MYCN nonamplified tumors with unfavorable International Neuroblastoma Pathology Classification histology (72.7% ± 5.4% and 82.4% ± 4.6%), which includes the majority of L2 patients with SCA. For patients with stage M (metastatic) and MS (metastatic, special) disease, genomic biomarkers affected risk group assignment for those < 12 months ( MYCN) or 12-18 months ( MYCN, histology, ploidy, and SCA) of age. In a retrospective analysis of patient outcome, the 5-year event-free survival and overall survival using COG version 1 were low-risk: 89.4% ± 1.1% and 97.9% ± 0.5%; intermediate-risk: 86.1% ± 1.3% and 94.9% ± 0.8%; high-risk: 50.8% ± 1.4% and 61.9% ± 1.3%; and using COG version 2 were low-risk: 90.7% ± 1.1% and 97.9% ± 0.5%; intermediate-risk: 85.1% ± 1.4% and 95.8% ± 0.8%; high-risk: 51.2% ± 1.4% and 62.5% ± 1.3%, respectively. CONCLUSION A revised 2021 COG neuroblastoma risk classifier (version 2) that uses the INRGSS and incorporates SCAs has been adopted to prospectively define COG clinical trial eligibility and treatment assignment.

A Novel Risk Classification Model Predicts Overall Survival and Locoregional Surgery Benefit in Colorectal Patients with Distant Metastasis at the Initial Diagnosis

2020 ◽  
Author(s):  
Mo Chen ◽  
Tian-en Li ◽  
Pei-zhun Du ◽  
Junjie Pan ◽  
Zheng Wang ◽  
...  
Keyword(s):  
Overall Survival ◽  
High Risk ◽  
Distant Metastasis ◽  
Cox Regression ◽  
Risk Group ◽  
Risk Groups ◽  
Risk Classification ◽  
Low Risk ◽  
Classification Model ◽  
Intermediate Risk

Abstract Background and aims: In this research, we aimed to construct a risk classification model to predict overall survival (OS) and locoregional surgery benefit in colorectal cancer (CRC) patients with distant metastasis.Methods: We selected a cohort consisting of 12741 CRC patients diagnosed with distant metastasis between 2010 and 2014, from the Surveillance, Epidemiology and End Results (SEER) database. Patients were randomly assigned into training group and validation group at the ratio of 2:1. Univariable and multivariable Cox regression models were applied to screen independent prognostic factors. A nomogram was constructed and assessed by the Harrell’s concordance index (C-index) and calibration plots. A novel risk classification model was further established based on the nomogram.Results: Ultimately 12 independent risk factors including race, age, marriage, tumor site, tumor size, grade, T stage, N stage, bone metastasis, brain metastasis, lung metastasis and liver metastasis were identified and adopted in the nomogram. The C-indexes of training and validation groups were 0.77 (95% confidence interval [CI] 0.73-0.81) and 0.75 (95% CI 0.72-0.78), respectively. The risk classification model stratified patients into three risk groups (low-, intermediate- and high-risk) with divergent median OS (low-risk: 36.0 months, 95% CI 34.1-37.9; intermediate-risk: 18.0 months, 95% CI 17.4-18.6; high-risk: 6.0 months, 95% CI 5.3-6.7). Locoregional therapies including surgery and radiotherapy could prognostically benefit patients in the low-risk group (surgery: hazard ratio [HR] 0.59, 95% CI 0.50-0.71; radiotherapy: HR 0.84, 95% CI 0.72-0.98) and intermediate risk group (surgery: HR 0.61, 95% CI 0.54-0.68; radiotherapy: HR 0.86, 95% CI 0.77-0.95), but not in the high-risk group (surgery: HR 1.03, 95% CI 0.82-1.29; radiotherapy: HR 1.03, 95% CI 0.81-1.31). And all risk groups could benefit from systemic therapy (low-risk: HR 0.68, 95% CI 0.58-0.80; intermediate-risk: HR 0.50, 95% CI 0.47-0.54; high-risk: HR 0.46, 95% CI 0.40-0.53).Conclusion: A novel risk classification model predicting prognosis and locoregional surgery benefit of CRC patients with distant metastasis was established and validated. This predictive model could be further utilized by physicians and be of great significance for medical practice.

Chronic Lymphocytic Leukemia: Evaluation of Cytogenetic Markers as Prognostic Factors

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4860-4860
Author(s):  
Jose Carda ◽  
Patricia Sousa ◽  
Patricia Olim ◽  
Emília Magalhães ◽  
Luis Rito ◽  
...  
Keyword(s):  
Overall Survival ◽  
Prognostic Factors ◽  
Retrospective Study ◽  
High Risk ◽  
Chronic Lymphocytic Leukemia ◽  
Progressive Disease ◽  
Risk Group ◽  
Staging System ◽  
Lymphocytic Leukemia ◽  
Intermediate Risk

Abstract Abstract 4860 Backgroud: Chronic lymphocytic leukemia (CLL) is one of the most frequent chronic lymphoproliferative disorders in Europe. It is characterized by persistent monoclonal lymphocitosis with localized or generalized lymphadenopathy. Despite the initial clinical presentation, it has a heterogeneous natural history, with the majority of patients living 10–12 years, but with some patients dying rapidly, within 2–3 years of diagnosis. Beside clinical prognostic factors, novel cytogenetic markers are recognized to be useful in predicting disease free and overall survival in CLL. AIMS: In a retrospective study throughout 10 years (1999-2009), we analyzed the clinical and biological presentation and compared the evolution and survival of patients with B-CLL using different cytogenetic markers. METHODS: We identified 112 cases (63 males and 49 females) of B-CLL with cytogenetic study by fluorescence in situ hybridization (FISH). RESULTS: Amongst 112 patients, the male to female (M/F) ratio was 1.3:1 and the median age was 70 (43-96) years. At diagnosis, the median lymphocyte count was 15.5 G/L (5.4-173). Fifty five patients (49%) had lymphadenopathies and seventeen (15%) had splenomegaly and/or hepatomegaly at presentation. By the revised Rai staging system seventy (63%) patients were included in low risk group, thirty (27%) in intermediate risk group and twelve (10%) in high risk group. The expression of ZAP-70 and CD38 by flow citometry was performed in 75 patients and revealed 13 (17%) patients CD38+ and 12 (16%) ZAP70+. The study of chromosomal aberrations with FISH showed thirty six patients (32%) with no abnormality, thirty six (32%) with isolated 13q deletion, fifteen (14%) with 12 trisomy, twelve (11%) with 11q deletion and thirteen (11%) with 17p deletion. Forty (36%) patients showed progressive disease in a median time of sixteen months (0-120), thirteen with 13qdel, seven with 17pdel and five with 12 trisomy. After treatment two patients showed progressive disease, six maintain a stable disease and thirty two obtain a remission, nine in complete remission. The Overall Survival (OS) at ten years was 70%. By the revised Rai staging system the OS at ten years was 80% for low risk, 70% for intermediate risk and all the high risk patients died during follow up. The OS at five years for the del13q-, 12 trisomy, del11q- and del17p- was 90%, 88%, 58% and 60%, respectively. SUMMARY: Chronic lymphocytic leukemia is currently considered a chronic disorder with a favourable outcome, but with a variable evolution to progressive disease. This retrospective study allowed the characterization of patient with CLL in our department and the acknowledgement that our results are quite similar to the published data. Disclosures: No relevant conflicts of interest to declare.

Validation of Proposed European Leukemia Net Recommendations for Standardized Reporting and Classification of Cytogenetic Risk in AML Patients Treated with Stem Cell Transplantation

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4894-4894
Author(s):  
Claudia Ulrike Walter ◽  
Fahed Almhareb ◽  
Naeem A. Chaudhri ◽  
Wahiba Chebbo ◽  
Shad Ahmed ◽  
...  
Keyword(s):  
Overall Survival ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Risk Stratification ◽  
Risk Group ◽  
Intermediate Risk ◽  
Event Free Survival ◽  
Free Survival ◽  
Significant Difference ◽  
First Remission

Abstract Abstract 4894 Background: Pretreatment karyotypes of leukemic cells provide the core for risk-stratification schemes in acute myeloid leukemia (AML) and cytogenetic abnormalities are well established as the strongest prognostic factor for response to therapy and for survival in AML patients. Several cytogenetic risk classifications have been built to stratify AML patients. Only the recent European Leukemia Net (ELN) recommendations by an international panel proposed a new standardized cytogenetic reporting system for AML. More importantly, few studies attempted so far to validate the ELN requirements for risk assessment in AML patients treated with allogeneic hematopoietic stem cell transplantation (HSCT). We investigated whether our series of adult AML patients treated with HSCT could validate the ELN classification criteria. We also assessed the prognostic value of this new risk stratification scheme. Methods: ELN cytogenetic reporting criteria were applied to a series of 110 adult AML patients from a single institution in the Middle East for whom complete cytogenetic data from pretreatment leukemic marrow were available and patients were assigned to the proposed cytogenetic risk subgroups, accordingly. We compared outcome for the different prognostic subgroups. Parameters analyzed were overall survival (OS) and event-free survival (EFS). Our AML group included 62 (56%) patients treated in their first remission (CR1), while most non-CR1 AML patients were treated with HSCT in CR2. The median age of all patients was 25 years (range: 14–57). Results: The patient group included 32 (29%) patients with favorable risk, 60 (55%) in the intermediate-risk group, and 18 (16%) in the adverse-risk group. When all the three groups were considered, there was significant difference in overall survival (OS) (P=0.007) and event-free survival (EFS) (P=0.007). However, there was no significant difference between our favorable-risk group and the intermediate-risk group. This is most likely due to selection bias in the favorable group (e.g. CD56 expression and other unfavorable markers). The adverse-risk group had significantly (P=0.002) shorter survival with median survival of 7.5 months vs. the intermediate-risk group where median survival has not been reached with an average follow-up of 46 months. When we attempted to consider only patients treated with HSCT in the first remission, there were too few patients in the intermediate-risk group (7 patients) for statistical evaluation. Conclusions: The ELN criteria proofed valuable in risk-stratifying our set of adult AML patients who were treated with HSCT and represent a Middle Eastern population. Our series clearly validated the ELN classification. The ELN classification needs further validation in patients treated with HSCT in first remission. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Escalating intravenous methotrexate improves event-free survival in children with standard-risk acute lymphoblastic leukemia: a report from the Children's Oncology Group

Blood ◽  
2011 ◽  
Vol 118 (2) ◽  
pp. 243-251 ◽  
Author(s):  
Yousif Matloub ◽  
Bruce C. Bostrom ◽  
Stephen P. Hunger ◽  
Linda C. Stork ◽  
Anne Angiolillo ◽  
...  
Keyword(s):  
Overall Survival ◽  
Acute Lymphoblastic Leukemia ◽  
High Risk ◽  
Lymphoblastic Leukemia ◽  
Oncology Group ◽  
Event Free Survival ◽  
Free Survival ◽  
Cancer Group ◽  
Leucovorin Rescue ◽  
Standard Risk

Abstract Children's Cancer Group-1991 selected 2 components from the Children's Cancer Group studies shown to be effective in high-risk acute lymphoblastic leukemia and examined them in children with National Cancer Institute standard-risk acute B-precursor lymphoblastic leukemia. These were (1) vincristine and escalating IV methotrexate (MTX) without leucovorin rescue during the interim maintenance (IM) phases and (2) addition of a second delayed intensification (DI) phase. Eligible patients (n = 2078) were randomly assigned to regimens containing either oral (PO) MTX, PO mercaptopurine, dexamethasone, and vincristine or IV MTX during IM phases, and regimens with either single DI or double DI. Five-year event-free survival (EFS) and overall survival for patients on the PO MTX arms were 88.7% ± 1.4% and 96% ± 0.9% versus 92.6% ± 1.2% and 96.5% ± 0.8% for those on the IV MTX arms (P = .009, P = .66). Five-year EFS and overall survival for patients who received single DI were 90.9% ± 1.3% and 97.1% ± 0.8% versus 90.5% ± 1.3% and 95.4% ± 3.8% for those who received double DI (P = .71, P = .12). No advantage was found for a second DI; however, replacement of PO MTX, PO mercaptopurine, vincristine, and dexamethasone during IM with vincristine and escalating IV MTX improved EFS.

Genome-Wide Genotype-Based Risk Model for Survival in Core Binding Factor Acute Myeloid Leukemia Patients

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1623-1623
Author(s):  
Silvia Park ◽  
Choi Hangseok ◽  
Hee-Je Kim ◽  
Jae-Sook Ahn ◽  
Hyeoung Joon Kim ◽  
...  
Keyword(s):  
Overall Survival ◽  
High Risk ◽  
Risk Model ◽  
Risk Group ◽  
Snp Array ◽  
High Risk Group ◽  
Event Free Survival ◽  
Free Survival ◽  
Genome Wide ◽  
Binding Factor

Abstract Introduction: The present study attempted to build a single nucleotide polymorphism (SNP)-based risk model for predicting overall survival (OS) and event free survival (EFS) in patients with core binding factor acute myeloid leukemia (CBF-AML). Methods: Adopting genome-wide SNP array using Affymetrix SNP array 6.0, we analyzed 868,157 SNPs with respect to OS and EFS in 104 patients with CBF-AML. Significant SNPs were identified from single SNP analysis. The risk model was constructed with incorporation of six SNPs and three clinical factors (age, c-kit exon 17 mutation, and LDH) for OS, and six SNPs and three clinical factors (age, WBC, and LDH) for EFS. The model was further defined into low and high risk groups based on risk scores. Results: The median age was 39 years, and the subgroup of t(8;21) and inv(16) or t(16;16) was assessed in 68 (65.4%) and 36 patients (34.6%). Finally 6 SNPs per each OS (rs4353685, rs4908185, rs7709207, rs12034, rs1554844, and rs17241868) and EFS (rs13385610, rs11210617, rs11169282, rs7709207, rs4438401 and rs16894846) were incorporated into the risk model. OS was significantly different in favor of the low risk group (80.4%) compared to the high risk group (22.0% at 3 years; p= 8.75 x 10-13; HR 8.67). For EFS, there was also a significant difference between the low (75.0%) versus high risk group (17.1% at 3 years; p=5.95 x 10-13; HR 7.67). Conclusion: A genome-wide SNP based risk model can stratify CBF-AML patients according to their OS and EFS in 104 patients. Figure 1 Overall survival and event free survival by risk model composed of SNPs and clinical risk factors Figure 1. Overall survival and event free survival by risk model composed of SNPs and clinical risk factors Figure 2 Figure 2. Disclosures No relevant conflicts of interest to declare.

Survival and prognostic factors in a series of adults with medulloblastomas

2009 ◽  
Vol 111 (3) ◽  
pp. 478-487 ◽  
Author(s):  
Laurent Riffaud ◽  
Stephan Saikali ◽  
Emmanuelle Leray ◽  
Abderrahmane Hamlat ◽  
Claire Haegelen ◽  
...  
Keyword(s):  
Overall Survival ◽  
Prognostic Factors ◽  
High Risk ◽  
Survival Time ◽  
Risk Group ◽  
Survival Rates ◽  
Event Free Survival ◽  
Term Survival ◽  
Free Survival ◽  
Standard Risk

Object In this article, the authors report their experience in the management of adult patients with medulloblastoma at their institution to identify prognostic factors important for survival and disease control. Methods Between 1977 and 2005, 27 patients who were ≥ 16 years old and had medulloblastoma were treated consecutively. There were 16 women and 11 men with a median age of 21 years (range 16–54 years). Gross-total resection was performed in 21 patients, subtotal (≥ 90%) in 2, incomplete in 1, and biopsy in 3 patients. Six patients had the desmoplastic variant, and 21 patients presented with classic medulloblastoma. Staging according to the Chang classification showed 4 patients with tumors invading the brainstem (2 with Stage T3b and 2 with Stage T4), 3 patients with metastases (2 with Stage M2 and 1 with Stage M3), and 1 patient in whom the stage was unknown (Stage MX) who died 10 days postoperatively. Twenty patients were assigned to the standard-risk group and 7 to the high-risk group. All patients except the one whose status was classified as Stage MX underwent craniospinal radiotherapy at our institution. Seven patients received chemotherapy before radiotherapy. Results The 5- and 10-year overall survival rates for the present study were 81 and 62%, respectively. The median overall survival time was 17.7 years. The 5- and 10-year event-free survival rates were 72 and 57%, respectively. The median event-free survival time was 17.9 years. Univariate analysis showed that survival was significantly correlated with sex (women had a better prognosis than men) and M stage (patients without metastases had a better outcome). Patient age, duration of symptoms, Karnofsky Performance Scale score at presentation, hydrocephalus, tumor location, brainstem invasion, extent of resection, histological subtype, preradiotherapy chemotherapy, risk group, and period of presentation were not significant variables. Multivariate analysis identified sex and M stage as well as the period of presentation as independent prognostic factors for overall and event-free survival times. Eleven patients suffered tumor recurrence within a median time of 4.2 years. The posterior fossa was not the most common site of recurrence, and delayed recurrence was not rare. All patients in whom the tumor recurred have died despite aggressive treatments. The median survival time after diagnosis of recurrence was 2.5 years. Questionnaires on quality of life and cognition showed high scores in favor of limited negative effects in the perception of mental and physical health after treatment. The authors observed 1 supposed second malignancy (thyroid carcinoma) and no evidence of pituitary dysfunction. Conclusions Long-term survival is possible in adults treated for medulloblastoma. Although rare, metastasis seeding at presentation is a poor prognostic factor. The possibility of delayed recurrence necessitates close follow-up of all patients. Tumor recurrences should be treated with aggressive therapies as some patients may have sustained response. Adjuvant chemotherapy should be given to high-risk patients, but its role in reducing recurrences, particularly distant ones, remains unclear in the standard-risk group.

scholarly journals Age, Diagnostic Category, Tumor Grade, and Mitosis-Karyorrhexis Index Are Independently Prognostic in Neuroblastoma: An INRG Project

2020 ◽  
Vol 38 (17) ◽  
pp. 1906-1918 ◽  
Author(s):  
Elizabeth Sokol ◽  
Ami V. Desai ◽  
Mark A. Applebaum ◽  
Dominique Valteau-Couanet ◽  
Julie R. Park ◽  
...  
Keyword(s):  
Risk Group ◽  
Recursive Partitioning ◽  
Diagnostic Category ◽  
Staging System ◽  
Tumor Grade ◽  
Risk Classification ◽  
Event Free Survival ◽  
Cox Models ◽  
Free Survival ◽  
The Individual

PURPOSE The Children’s Oncology Group (COG) stratifies the treatment of patients with neuroblastoma on the basis of a combination of biomarkers that include age and tumor histology classified by age-linked International Neuroblastoma Pathology Classification (INPC) criteria. By definition, this leads to a duplication of the prognostic contribution of age. The individual histologic features underlying the INPC have prognostic strength and are incorporated in the International Neuroblastoma Risk Group classification schema. Here, we analyzed data in the International Neuroblastoma Risk Group Data Commons to validate the prognostic strength of the underlying INPC criteria and to determine whether a risk classification devoid of the confounding of age and INPC criteria will identify new prognostic subgroups. PATIENTS AND METHODS Event-free survival of patients diagnosed between 1990 and 2002 (cohort 1; n = 10,104) and between 2003 and 2016 (cohort 2; n = 8,761) was analyzed. Recursive partitioning with univariate Cox models of event-free survival (“survival tree regression”) was performed using (1) individual INPC criteria (age at diagnosis, histologic category, mitosis-karyorrhexis index (MKI), grade of differentiation) and (2) factors in (1) plus other COG-risk biomarkers (International Neuroblastoma Staging System [INSS] stage, MYCN status, ploidy). RESULTS The independent prognostic ability of age, histologic category, MKI, and grade were validated. Four histologic prognostic groups were identified (< 18 months with low v high MKI, and ≥ 18 months with differentiating v undifferentiated/poorly differentiating tumors). Compared with survival trees generated with established COG risk criteria, an additional prognostic subgroup was identified and validated when individual histologic features were analyzed in lieu of INPC. CONCLUSION Replacing INPC with individual histologic features in the COG risk classification will eliminate confounding, facilitate international harmonization of risk classification, and provide a schema for more precise prognostication and refined therapeutic approaches.

Chronic Myeloid Leukemia Patients In Tunisia: Epidemiology and Outcome In The Imatinib Era (A Multicentric Experience)

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4037-4037
Author(s):  
Raihane Ben Lakhal ◽  
Hela Ghedira ◽  
Hatem Bellaaj ◽  
Yosra Ben Youssef ◽  
Samia Menif ◽  
...  
Keyword(s):  
Overall Survival ◽  
Developing Countries ◽  
High Risk ◽  
Myeloid Leukemia ◽  
Risk Group ◽  
Progression Free Survival ◽  
Molecular Response ◽  
Event Free Survival ◽  
Free Survival ◽  
Optimal Response

Abstract Background The introduction of the first targeted tyrosine kinase inhibitor (TKI), imatinib mesylate (IM) revolutionized the therapeutic paradigm and dramatically improved outcomes of chronic myeloid leukemia(CML). Data are limited in developing countries regarding the clinicopathologic features and response to therapy in the era of IM. Aims To report on the clinicoepidemiologic features of CML in Tunisia, to evaluate the long-term outcome of patients in chronic (CP) or accelerated phase (AP) treated with IM 400mg daily as frontline therapy and to determine imatinib’s efficacy and safety. Methods From October 2002 to December 2011, two hundred and ninety two CML patients were treated with IM in 6 Tunisian departments of hematology. Monitoring response was defined as the ELN provided guidelines. Response (Hematologic, cytogenetic and molecular responses), adverse events and outcome (overall survival, event free survival and progression free survival) were evaluated. The factors associated with outcome of IM therapy were also analyzed. Results Two hundred ninety-two patients enrolled with a median follow-up duration of 56 (8 -290) months: The median age was 44 years (3-78 years). One hundred and fifty (51.3%) patients were male, 134 (49%) were asymptomatic at diagnosis. Splenomegaly was present in 237 of 292 (81%). Additional cytogenetic abnormalities were encountred in 24 (8.3%) patients. At diagnosis, 271 (92.8%) patients were in CP, 21 (7.2%) were in AP. The Sokal risk was low in 64 (23%), intermediate in 94 (33.5%), and high in 122 patients (43.5%). The Eutos risk was low in 179 (75%), and high in 60 (25%) patients.The rate of cumulative complete hematologic response (CHR), complete cytogenetic response (CCyR), major molecular response (MMR) and molecular response 4/5 log (CMR) in CP/AP CML patients were 93.8%, 73%, 65% and 33.9%, respectively. According to the 2009 ELN criteria, optimal, suboptimal response and failure were noted in 132 (47%), 68 (24%) and 82 (29%) patients, respectively. Five year event free survival (EFS), progression free survival (PFS) and overall survival (OS) were 78%, 89% and 91%, respectively. By multivariate analyzis, AP, high Eutos risk and baseline WBC ≥ 150G/l remained independent predictive factors of non optimal response to IM. AP was an adverse independent prognostic factor for EFS, PFS and OS. Patients obtained CCyR at 12 months after the initiation of IM treatment were associated with longer PFS (P< 0.0001) and OS (P< 0.0001). ELN response was also significantly associated with EFS. The adverse events (AE) of IM were moderate and tolerable. Only 3 patients discontinued IM for intolerance. IM-related hematologic AE included neutropenia in 6.2%, anemia in 8.9%, and thrombocytopenia in 17.2%. Nonhematologic AE (21%), including mainly edema in 7.1%, digestive disorders in 5.5%, weight gain and skin rash in 3.1%. Conclusion We found that substantial number of patients in our series were in intermediate or high risk group. With the caveats that the monitoring of the disease was not optimal, response rates were similar to those reported in previous studies. It is clear to us that improvements should be made in treatment of AP-CML and high Sokal risk group of CP-CML. The front-line use of 2nd TKI are expected to improve the results of the first line treatment of these high risk Tunisian patients, but cost and accessibility of this therapy remain the problems in developing countries. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Neuroblastoma Among Omani Children

2021 ◽  
Author(s):  
Abeer Al-Battashi ◽  
Ameera Al-Rahbi ◽  
Adbulhakeem Al-Rawahi ◽  
Mohammed Mamdouh ◽  
Ibrahim Al-Ghaithi ◽  
...  
Keyword(s):  
Overall Survival ◽  
High Risk ◽  
Survival Data ◽  
Low Risk ◽  
P Value ◽  
Intermediate Risk ◽  
Event Free Survival ◽  
Female Ratio ◽  
Free Survival ◽  
One Year

Objectives: Neuroblastoma is a common childhood malignancy with limited number of publications from the middle east. This study describes the clinical characteristics and the survival outcome of Omani children with neuroblastoma who are treated at the National Oncology Center from 2010 to 2017. Methods: Data was retrospectively collected for Omani Children aged less than thirteen-years with neuroblastoma from January 2010 to December 2017. The survival data were statistically correlated with known prognostic factors including age, stage of disease, MYCN profile and presence of metastasis. Results: Fifty-six Omani children were included. The male to female ratio was 1:1. The mean age at presentation was one year and ten months. The two most common presenting complaints were body masses (48.2%) and constitutional symptoms (33.9%). About 54.5% were high-risk, 35.7% were intermediate risk and 9.8% were low-risk. High-risk neuroblastoma was mainly in children older than one year (76.6%), with low-risk being mainly observed in less than one year of age (80%). The overall survival of all groups combined was 74% (p value < 0.05); and the event free survival was 67% (p value < 0.05). The five years overall survival for the high-risk, intermediate-risk and low-risk was 60%, 88% and 100% respectively. Moreover, the event free survival was 51%, 79% and 100% respectively. Conclusion: Omani children with neuroblastoma mainly presented with masses or constitutional symptoms. The majority of Omani children with neuroblastoma had an advanced disease at presentation which was associated with inferior survival. The survival outcomes were reasonably similar to published international data. Keywords: Neuroblastoma, Oman, Survival

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